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DefinitionMalignant tumors represent an uncontrolled growth oftissue. Unlike benign neoplasms, they are more locallyinvasive, have a greater degree of anaplasia, and havethe ability to metastasize regionally to lymph nodes ordistantly to other sites. Malignant tumors that arise denovo are termed primary tumors, and those that originatefrom distant primary tumors are referred to as secondaryor metastatic malignancy. Cancers may be caused byviruses, significant radiation exposure, genetic defetts,and exposure to carcinogenic chemicals. For instance,using tobacco is strongly associated with oral carcinoma.

The most convenient method of classification ofcancers is based on histopathology. In this chapter themalignancies that commonly affect the jaws have beendivided into four categories: carcinomas (lesions ofepithelial origin), metastatic lesions from distant sites,sarcomas (lesions of mesenchymal origin), and malig-nancies of the hematopoietic system. Unusual malig-nant tumors have been omitted to concentrate on morecommon lesions that a general practitioner mayencounter.

cause. Most oral cancers occur in men aged 50 yearsand older; however, malignant tumors may occur at anyage in either gender.

Dentists must watch vigilantly for the possibility ofmalignancy in their patients. Because the prevalenceof oral malignancy is low, many general practitionerspractice years without encountering a patient who hasa malignant tumor. This rarity may make a dentist lesslikely to recognize a malignant condition when it doesexist. Lack of attention to this possibility may result indelayed diagnosis, delayed treatment, increased needfor aggressive treatment with added morbidity, and inthe worst case, premature death.

Radiology plays a number of important roles in themanagement of the patient with cancer. First, diagnos-tic images may aid in the establishment of an initialdiagnosis of a tumor. Diagnostic imaging also aids in theappropriate staging of disease from early small cancersto large cancers that have spread. Appropriate radio-logic investigations assist the surgeon or radiationoncologist to determine the anatomic spread of thetumor so that it can be excised or irradiated adequately.Radiologic investigation has the potential to determinethe presence of osseous involvement from soft tissuetumors and allow the practitioner to assess the involve-ment of lymph nodes and treatment outcome. Finally,a thorough radiographic dental examination plays apart in the management of the cancer survivor,who often is rendered xerostomic, neutropenic, andsusceptible to dental caries, periodontal disease, andsystemic infection.

Clinical FeaturesThe following clinical signs and symptoms suggest thata lesion may be malignant: displaced teeth, loosenedteeth over a short time, foul smell, ulceration, pre-sence of an indurated or rolled border, exposure ofunderlying bone, sensory or motor neural deficits,lymphadenopathy, weight loss, dysgeusia, dysphagia,dysphonia, hemorrhage, lack of normal healing, andpain or rapid swelling with no demonstrable dental

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459CHAPTER 22 MALIGNANT DISEASES OF THE JAWS

RADIOGRAPHIC FEATURES

The follQwing features may suggest the presence of amalignant tumor. The absence of visible radiologicsigns as described does not preclude malignancy; it onlyimplies that no visible radiographic signs exist.

so that teeth may appear to be floating in space (Fig.22-1, F).

Occasionally root resorption is present; this is morecommon in sarcomas. Internal trabecular bone isdestroyed, as are cortical boundaries such as the sinusfloor (Fig. 22-1, B), inferior border of the mandible, fol-licular cortices, and the cortex of the inferior alveolarneurovascular canal. Because malignant tumors tend togrow rapidly, they invade by means of the easiest routes,such as through the maxillary antrum or through theperiodontal ligament space around teeth, resulting inirregular widening with destruction of the lamina dura(Fig. 22-1, C); they also may spread through the infe-rior alveolar neurovascular canal, causing similar widen-ing. Where the tumor has destroyed the outer cortex ofbone, usually no periosteal reaction occurs; however,some tumors stimulate unusual periosteal new boneformation (Fig. 22-1, E). Lesions such as osteosarcomaand metastatic prostate lesions, as well as other tumors,can stimulate the formation of thin straight spicules ofbone, giving a "hair-on-end" or sunburst appearance. Ifthere is a secondary inflammatory lesion coexistingwith the malignancy, a periosteal reaction normallyassociated with an inflammatory lesion (e.g., "onionskin-like") may be seen.

LocationPrimary and metastatic malignant tumors may occuranywhere in the oral and maxillofacial region. Primarycarcinomas are .more commonly seen in the tongue,floor of mouth, tonsillar area, lip, soft palate, or gingivaand may invade the jaws from any of these sites. Sarco-mas are more common in the mandible and in poste-rior regions of both jaws. Metastatic tumors are mostcommon in the posterior mandible and maxilla. Somemetastatic lesions grow at the apices of teeth or in thefollicles of developing teeth (Fig. 22-1, D).

Periphery and ShapeThe typical appearance of the periphery (border) of amalignant lesion is an ill-defined border with lack ofcortication and absence of encapsulation (a soft tissueor radiolucent periphery). This infiltrative border hasuneven extensions of bone destruction. Fingerlikeextension of the tumor occurs in many directions; thisextension is followed by osseous destruction producinga region of radiolucency (Fig. 22-1, A). Evidence ofosseous destruction with adjacent soft tissue mass ishighly suggestive of'malignancy (Fig. 22-1, B). Such amass may exhibit a smooth or ulcerated peripheralborder if cast against a radiolucent background. Theshape of a malignant tumor of the jaw is irregular.

Carcinomas

SQUAMOUS CELL CARCINOMA ARISING IN

SOFT TISSUE

SynonymEpidermoid carcinoma

Internal StructureBecause most malignancies do not produce bone orstimulate the formation of reactive bone, their internalaspect is radiolucent in most instances. Occasionally,residual islands of bone are present, resulting in apattern of patchy destruction with some scatteredresidual internal osseous structure. Some tumors, suchas metastatic prostate or breast lesions, can induce boneform~tion, resulting in an abnormal-appearing inter-nal osseous architecture, whereas others, such asosteogenic sarcomas, produce abnormal bone givingthe involved bone a sclerotic (radiopaque) appearance.

DefinitionSquamous cell carcinoma, the most common oralmalignancy, may be defined as a malignant tumororiginating from surface epithelium. It is characterizedinitially by invasion of malignant epithelial cells into theunderlying connective tissue with subsequent spreadinto deeper soft tissues adjacent bone local-regionallymph nodes and ultimately to distant sites such as thelung, liver, and skeleton.

Clinical FeaturesSquamous cell carcinoma appears initially as white orred (sometimes mixed) irregular patchy lesions of theaffected epithelium. With time, these lesions exhibitcentral ulceration; a rolled or indurated border, whichrepresents peripheral invasion of malignant cells; andpalpable infiltration into adjacent muscle or bone. Painmay be variable, and regional lymphadenopathycharacterized by rubbery-hard lymph nodes that maybe tethered to underlying structures may be present.

Effects on Surrounding StructuresMalignancy is destructive, often rapidly so. The effecton surrounding structures mirrors this behavior.Slower-growing benign tumors or cysts may resorb toothroots or displace teeth in a bodily fashion withoutcausing loose teeth. In contrast, rapidly growing malig-nant lesions generally destroy supporting alveolar bone


461MALIGNANT DISEASES OF THE JAWSCHAPTER 22

FIG. 22-1 Radiologic features of oral malignancy. A, 111-defined invasive borders followed by bone destruction.B, Destruction of the cortical boundary (floor of maxillaryantrum) with an adjacent soft tissue mass (arrows). C, Tumorinvasion along the periodontal membrane space, causingirregular thickening of this space. D, Multifocal lesionslocated at root apices and in the papilla of a developingtooth, destroying the crypt cortex and displacing the devel-oping tooth in an occlusal direction (arrow). E, Four types ofeffects on cortical bone and periosteal reaction, from top tothe bottom: cortical bone destruction without periostealreaction, laminat~d periosteal reaction with destruction ofthe cortical bone and the new periosteal bone, destructionof cortical bone with periosteal reaction at the peripheryforming Codman's triangles, and a spiculated or sunray typeof periosteal reaction. F, Bone destruction around existingteeth, producing an appearance of teeth floating in space.

show sharpened thinned bone ends with displacementof segments and an adjacent soft tissue mass. Sclerosisin underlying osseous structures (likely from secondaryinflammatory disease) may be seen in association witherosions from surface carcinomas.

Internal structure. The internal structure of squamouscell carcinoma in jaw lesions is totally radiolucent; theoriginal osseous structure can be completely lost. Occa-sionally small islands of residual normal trabecularbone are visible within this central radiolucency.

Effects on surrounding structures. Evidence of invasionof bone around teeth may first appear as widening ofthe periodontal ligament space with loss of adjacentlamina dura. Teeth may appear to float in a mass ofradiolucent soft tissue bereft of any bony support. Inextensive tumors this soft tissue mass may grow with theteeth in it as "passengers," so teeth are grossly displacedfrom their normal position. Tumors may grow alongthe inferior neurovascular canal and through themental foramen, resulting in an increase in the widthand loss of the cortical boundary of this canal. Destruc-tion of adjacent normal cortical boundaries such as thefloor of the nose, maxillary sinus, or buccal or lingualmandibular plate may occur. The posterior aspect ofthe maxilla may also be effaced. The inferior border ofthe mandible may be thlflried or destroyed. If the tumoris extensive, pathologic fracture may occur.

Other clinical features include a soft tissue mass, pares-thesia, anesthesia, dysesthesia, pain, foul smell, trismus,grossly loosened teeth, or hemorrhage. Large lesionscan obstruct the airway, the opening of the eustachiantube (leading to diminished hearing), or the nasophar-ynx. Patients often report a significant weight loss andfeel unwell. Males are more commonly affected thanfemales. The condition is often fatal if untreated. Mostsquamous cell carcinomas occur in persons older than50 years.

Differential DiagnosisSquamous cell carcinoma is discernible from othermalignancies by its clinical and histologic features.Occasionally it is difficult to differentiate inflammatorylesions such as osteomyelitis from squamous cell carci-noma, especially when oral bacteria secondarily infectthe tumor. Both oosteomyelitis and squamous cell carci-noma are destructive, leaving islands of osseous struc-ture that may appear to be consistent with sequ~stra.Evidence of profound bone destruction or invasivecharacteristics helps to identify the presence of amalignancy when a mixture of inflammatory changesand carcinoma exists. Osteomyelitis usually producessome periosteal reaction, whereas squamous cell carci-noma does not. In cases of osteoradionecrosis, wherethe patient has had prior malignancy, periosteal newbone is absent. If osseous destruction is present, thedifferentiation of this condition from squamous cellcarcinoma requires advanced imaging and biopsy.

Radiographic FeaturesLocation. Squamous cell carcinoma commonlyinvolves the lateral border of the tongue. Therefore acommon site to observe bone invasion is the posteriormandible. Lesions of the lip and floor of the mouthmay invade the anterior mandible. Lesions involvingattached gingiva and underlying alveolar bone maymimic inflammatory disease such as periodontaldisease. This malignancy is also seen on the tonsils, softpalate, and buccal vestibule. It is uncommon on thehard palate.

Periphery and shape. Squamous cell carcinoma mayerode into underlying bone from any direction, pro-ducing a radiolucency that is polymorphous and irreg-ular in outline. Invasion occurs in one half of cases andis characterized most commonly by an ill-defined, non-corticated border (Fig. 22-2). Rarely the border mayappear smooth without a cortex, indicating underlyingerosion rather than invasion. If bone involvement isextensive, the periphery appears to have fingerlikeextensions preceding a zone of impressive osseousdestruction. If pathologic fracture occurs, the borders

ManagementOral squamous cell carcinoma is usually managed usinga combination of surgery and radiation therapy. Thechoice of which modality to use depends on the


,462 PART V RADIOGRAPHIC INTERPRETATION OF PATHOLOGY

FIG. 22-2 A through E, Squamous cell carcinoma (arrows), resulting in irregular destruc-tion of bone. Note the destruction of the right alveolar process and floor of maxillary sinusand the soft tissue mass (arrow) in B. In the occlusal film image (C) the anterior floor ofthe nose has been destroyed (note lack of anterior nasal spine), as well as the anterioralveolar process. D, The supporting alveolar bone has been destroyed from around theteeth. E, Displays destruction of the anterior border of the mandibular ramus by a squa-mous cell carcinoma.


..

CHAPTER 22 463MALIGNANT DISEASES OF THE JAWS

protocol of the treating center and the location andseverity of the tumor. Generally, if an adequate marginof normal tissue can be obtained, surgery is the usualtreatment, followed by radiation treatment. An alter-native is to use radiation as the primary treatmentfollowed by surgical salvage. Currently, there is a trendis to add concomitant chemotherapy as an adjunct toeither radiation or surgical treatment, which requiresthe dental practitioner to be aware of changes in thepatient's circulating blood count prior to any invasivedental treatment. including scaling.

SQUAMOUS CEll CARCINOMA ORIGINATINGIN BONE

SynonymsPrimary intraosseous carcinoma, intraalveolar carci-noma, primary intraalveolar epidermoid carcinoma,primary epithelial tumor of the jaw, central squamouscell carcinoma, primary odontogenic carcinoma, intra-mandibular carcinoma, odontogenic carcinoma, andcentral mandibular carcinoma

FIG. 22-3 This primary intraosseous carcinoma in the leftmandible exhibits no internal structure, a poorly definedperiphery, and thinning of the overlying mandibularbone.

DefinitionPrimary intraosseous carcinoma is a squamous cell car-cinoma arising within the jaw that has no original con-nection with the surface epithelium of the oral mucosa.Primary intraosseous carcinomas are presumed to arisefrom intraosseous remnants of odontogenic epithe-lium. Carcinoma from surface epithelium, odontogeniccysts, or distant sites (metastases) must be excluded.

strates os~eous destruction and varying degrees ofextension at the periphery. The degree of raggednessof the border may reflect the aggressiveness of thelesion. If sufficient in size, pathologic fracture occurs,with its associated step defects, thinned corticalborders, and subsequent soft tissue mass.

Internal structure. The internal structure is wholly radi-olucent with no evidence of bone production and verylittle residual bone left within the center of the lesion.If the lesion is small, overlying buccal or lingual platesmay cast a shadow that may mimic the appearance ofinternal trabecular bone.

Clinical FeaturesThese neoplasms are rare and may remain silent untilthey have reached a fairly large size. Pain, pathologicfracture, and sensory nerve abnormalities such as lipparesthesia and lymphadenopathy may occur with thistumor. It is more common in men and in patients intheir fourth to eighth decade of life. Tbe surface epithe-lium is invariably normal in appearance. Effects on surrounding structures. These lesions are

capable of causing destruction of the antral or nasalfloors, loss of the cortical outline of the mandibularneurovascular canal, and effacement of the laminadura. Root resorption is unusual. Teeth that lose bothlamina dura and supporting bone appear to be floatingin space.

Radiographic FeaturesLocation. The mandible is far more commonlyinvolved than the maxilla, with most cases being presentin the molar region (Fig. 22-3) and less frequently inthe anterior aspect of the jaws. Because the lesion isby definition associated with remnants of the dentallamina, it originates only in tooth-bearing parts of thejaw. Differential Diagnosis

If the lesions are not aggressive and have a smoothborder and radiolucent area, they may be mistaken forperiapical cysts or granulomas. Alternately, if lesions arenot centered about the apex of a tooth, occasionally itis difficult to differentiate this condition from odonto-genic cysts or tumors. If the border is obviously infil-

Periphery and shape. The periphery of the majorityof lesions is ill-defined, although some have beendescribed as well-defined. They are most often roundedor irregular in shape and have a border that demon-


464 !'AK! V KAUIVI.JKAI'HIL INltKI'KtlAllVN VI- I'AIHULU(J'

trative with extensive bone destruction, a metastatIclesion must be excluded, as well as multiple myeloma,fibrosarcoma, and carcinoma arising in a dental cyst.Examination of the oral cavity and especially thesurface epithelium assists in differentiating this condi-tion from surface squamous cell carcinoma.

ManagementGenerally these tumors are excised with their sur-rounding osseous structure in an en bloc resection.Radiation ail.d chemotherapy maybe used as adjunctive

therapies.

I-ill. 22-4 Larclnoma arising in a preexisting dentigerouscyst related to the mandibular left third molar shows absenceof a cyst cortex, invasion into adjacent bone, and ill-definedborders.

SQUAMOUS CEll CARCINOMA ORIGINATING IN

A CYST

SynonymsEpidermoid cell carcinoma and carcinoma ex odonto-

genic cyst

DefinitionSquamous cell carcinoma arising in a preexisting dentalcyst is uncommon and excludes invasion from surfaceepithelial carcinomas, metastatic tumors, and primaryintraosseous carcinoma. This condition may arise frominflammatory periapical, residual, dentigerous, andodontogenic keratocysts. Histologically the lining squa-mous epithelium of the cyst gives rise to the malignant

neoplasm.

Ihe advanced leSIon has an Ill-dehned, mhitrativeperiphery that lacks any cortication. Its shape becomesless "hydraulic" in appearance and more diffuse.

Internal structure. This lesion lacks any ability toproduce bone. It is wholly radiolucent, perhaps moreso than invasive surface carcinoma, owing to priorosteolysis from the cyst.Lllmcal r-eatures

The most common presenting sign or symptomassociated with this condition is pain. The pain may becharacterized as dull and of several months' duration.Swelling is occasionally reported. Pathologic fracturemay occur, as may fistula formation and regionallymphadenopathy. If the upper jaw is involved, sinuspain or swelling may be present.

t"ects on surrounalng structures. ~arcinoma arising indental cysts is capable of thinning and destroying thelamina dura of adjacent teeth or adjacent corticalboundaries such as the inferior border of the jaw orflo<;)r of the nose. It can produce complete destructionof the alveolar process.

Kaolograpnlc t-eaturesLocation. This tumor may occur anywhere an odonto-genic cyst is found, namely, the tooth-bearing portionsof the jaws. Most cases occur in the mandible (Fig. 22-4), with a few cases reported in the anterior maxilla.

umerentlal uiagnosisIf a dental cyst is infected, it may lose its normal corti-cal boundary and appear ragged and identical to amalignant lesion arising in a preexisting cyst. However,inflamed cysts usually show a reactive peripheral scle-rosis because of inflammatory products present in thecyst lumen. This is not normally present in a cyst, whichhas undergone malignant transformation. Neverthe-less, the two may be difficult to differentiate radiologi-cally, and therefore cysts should always be submitted forhistologic examination. Multiple myeloma may appearas a solitary lesion and may be difficult to distinguish,especially if the lesion has a cystic, well-defined shape.Metastatic diseas~ may be similar, although it is com-monly multifocal.

t'erlpnery ana snape. 1 ne raruoiogic pIcture or squa-mous cell carcinoma originating in a cyst mirrors thehistologic findings. Because the lesion arises from acyst, the shape is often round or ovoid. If it is a smalllesion in a cyst wall, the periphery may be mostly welldefined and even corticated. In this case the radi-ographic differentiation from a normal cyst is impossi-ble. As the malignant tissue progressively replaces cystlining, the smooth border is lost or becomes ill-defined.


,465CHAPTER 22 MALIGNANT DISEASES OF THE JAWS

ManagementThe treatment of squamous cell carcinoma originatingin a cyst is identical to that described with primaryintraosseous carcinoma.

CENTRAL MUCOEPIDERMOID CARCINOMA

SynonymMucoepidermoid carcinoma

Definition.Central mucoepidermoid carcinoma is an epithelialtumor arising in bone, likely originating from pluripo-tential odontogenic epithelium or from a cyst lining. Itis histologically indistinguishable from its soft tissuecounterpart. The criteria for diagnosis of a centralmucoepidermoid tumor are the presence of intact cor-tical plates, radiographic evidence of bone destruction,and typical histologic findings consistent with mucoepi-dermoid tumor. Additionally, the practitioner mustexclude the possibility of an invasive overlying mucoepi-dermoid tumor or odontogenic tumor.

FIG. 22-5 The multilocular radiolucency in this radi-ograph has displaced the mandibular canal and destroyedthe superior crest of the alveolar process and the distalsupporting bone of the second molar.

Internal structure. The internal structure has featureslike those of a benign odontogenic tumor such as anameloblastoma. Lesions are often described as beingmultilocular or having either a soap-bubble or honey-comb internal structure, implying the presence ofcompartments separated by thin or thick cortical septa.This bone is not produced by the tumor, but is merelyremodeled residual bone taking the form of septa.

Clinical FeaturesUnlike other malignant tumors of the jaws, the centralmucoepidermoid tumor is more likely to mimic abenign tumor or cyst. The most common complaint isof a painless swelling. The swelling may have beenpresent for months or even years and has been reportedto cause facial asymmetry. Occasionally it may feel as ifteeth have been moved or a denture may no longer fit.Tenderness rather than severe pain may also bepresent. Paresthesia of the inferior alveolar nerve aswell as spreading of the lesion to regional lymph nodeshas been reported. Central mucoepidermoid tumor,unlike other oral malignancies, is more common infemales than males.

Effects on surrounding structures. Mucoepidermoidtumor is capable of causing expansion of adjacentnormaL bony walls. The buccal and lingual corticalplates, inferior border of the mandible, and alveolarcrest are usually intact; however, they may be thinnedand grossly displaced. The mandibular canal maybe depressed or pushed laterally or medially. Thesecharacteristics are more similar to benign tumors thanmalignant tumors. Teeth remain largely unaffected bythis disease, although adjacent lamina dura may be lost.

Radiographic FeaturesLocation. The lesion is twice as common in themandible as the maxilla, usually occurring in the pre-molar and molar region with a few cases reported in theanterior mandible. The lesion most commonly occursabove the mandibular canal, similar to odontogenictumors.

Differential DiagnosisThe differential diagnosis of this lesion reflects its lackof features commonly associated with oral malignancy.The chief differential is ameloblastoma, with which itshares similarities in its peripheral and internal fea-tures. It may not be possible to differentiate the two.Odontogenic myxoma and central giant cell granulomaalso may be confused with mucoepidermoid tumor, asmay other odontogenic cysts or tumors.Periphery and shape. Mucoepidermoid tumor pres-

ents as a unilocular or multilocular expansile mass (Fig.22-5). The border is most often well defined and wellcorticated and often crenated or undulating in nature,which is similar to benign odontogenic tumors. Theperipheral cortication may be impressively thick, whichbelies its malignant nature. Rarely the periphery is notcorticated and has a more malignant appearance.

ManagementMucoepidermoid carcinoma is treated surgically withen bloc resection enc6mpassing a margin of adjacentnormal bone. Neck dissection and postoperative radia-tion therapy may be required to control spread tolymph nodes.


466 PART V RADIOGRAPHIC INTERPRETATION OF PATHOLOGY

maxillary sinus. The mandibular neurovascular canalmay be displaced or eroded.

MALIGNANT AMELOBLASTOMA AND

AMELOBLASTIC CARCINOMA

DefinitionMalignant ameloblastoma is defined as an ameloblas-toma exhibiting the histologic criteria of a malignantneoplasm, such as increased and abnormal mitosis andhyperchromatic, large, pleomorphic nuclei. The histo-logic features may not correlate with the clinical behav-ior. On the other hand, ameloblastic carcinoma is an-ameloblastoma with typical benign histologic featuresthat i~ dee~ed malignant because of its biologic behav-ior, namely, metastasis.

Differential DiagnosisThe differential diagnosis of this lesion should includebenign ameloblastoma, odontogenic keratocyst, odon-togenic myxoma, and central mucoepidermoid tumor,from which malignant ameloblastoma may not bedistinguishable radiologically. If the lesion is locallyinvasive and this is apparent radiologically, a diagnosisof carcinoma arising in a dental cyst should be enter-tained. If the patient is young and the location of thelesion is anterior to the second permanent molar,central giant cell granuloma may mimic some of itsradiologic features. Often the final diagnosis is theresult of histologic evaluation or the detection ofmetastatic lesions.

Clinical FeaturesClinically these lesions may behave as benignameloblastomas, exhibiting a hard expansile massof the jaw with displaced and perhaps loosened teethand normal overlying mucosa. Tenderness of theoverlying soft tissue has been reported. Metastaticspread may be to the cervical lymph nodes, lung, orother viscera, as well as to the skeleton, especially thespine. Local extension may occur into adjacent bones,connective tissue, or salivary glands. These tumorsoccur most commonly between the first and sixthdecades of life and are more common in males thanfemales.

ManagementThese lesions are most often treated with en bloc sur-gical resection. However, many may not be discoveredto be malignant until the time of the first surgery oreven later. Because the histologic appearance of theselesions may mimic benign ameloblastoma, the initialtreatment often is inadequate. In addition, the metasta-tic lesions may not appear for many months or yearsafter treatment of the primary tumor, adding anotherreason for treatment failure.

Radiographic FeaturesLocation. These lesions are more common in themandible than in the maxilla, with most occurring inthe premolar and molar region, where ameloblastomais typically found.

Metastatic Tumors

SynonymSecondary malignancy

Periphery and shape. Similar to ameloblastoma, awell-defined border occurs with cortication, presenceof crenations, or scalloping of the border. Malignantameloblastoma may show some of the signs more com-monly seen in malignant neoplasms, namely, loss of andsubsequent breaching of the cortical boundary, invad-ing into the surrounding soft tissue.

DefinitionMetastatic tumors represent the establishment of newfoci of malignant disease from a distant malignanttumor usually by way of the blood vessels. An interest-ing feature of these lesions is that metastatic lesions inthe jaws usually arise from sites that are anatomicallyinferior to the clavicle. Metastatic lesions of the jawsusually occur when the distant primary lesion is alreadyknown, although on occasion the presence of a metasta-tic tumor may herald the presence of a silent primarylesion. Jaw involvement accounts for less than 1 % ofmetastatic malignancies found elsewhere, with mostaffecting the spine, pelvis, skull, ribs, and humerus.Most frequently the tumor is a type of carcinoma, themost common primary sites being the breast, kidney,lung, colon and rectum, prostate, thyroid, stomach,melanoma, testes, bladder, ovary, and cervix. In chil-dren the tumors include neuroblastoma, retinoblas-toma, and Wilms' tumor. Metastatic carcinoma must be

Internal structure. The lesions are either unilocular or,more commonly, multilocular, giving the appearanceof a honeycomb or soap-bubble pattern as seen inbenign ameloblastomas. Most of the septa are robustand thick.

Effects on surrounding structures. Teeth may be movedbodily by the tumor and may exhibit root resorptionsimilar to a benign tumor. Bony borders may be effacedor breached, and as in benign ameloblastoma, thelesions may erode lamina dura and displace normalanatomic boundaries such as the floor of the nose and



differentiated from the more common locally invadingsquamous carcinoma.

increase in the width of the periodontal ligament space.If the tumor has seeded in the papilla of a developingtooth, the cortices of the crypt may be totally or par-tially destroyed. Teeth may seem to be floating in a softtissue mass and may be in an altered position becauseof loss of bony support. Extraction sockets may fail toheal and may increase in size. Resorption of teeth israre (sometimes associated with multiple myelomaand chondrosarcoma); this is more common in benignlesions. The cortical bone of adjacent structures suchas the neurovascular canal, sinus, and nasal fossa isdestroyed. On occasion the tumor breaches the outercortical plate of the jaws and extends into surroundingsoft tissues or presents as an intraoral mass (Fig.22-6, E).

Clinical FeaturesMetastatic disease is more common in patients in theirfifth to seventh decade of life. Patients may complain ofdental pain, numbness or paresthesia of the thirdbranch of the trigeminal nerve, pathologic fracture ofthe jaw, or hemorrhage from the tumor site.

Radiographic ~eaturesLocation.. The posterior areas of the jaws are morecommonly affected (Fig. 22-6, A), the mandible beingfavored over the maxilla. The maxillary sinus may bethe next most common site, followed by the anteriorhard palate and mandibular condyle. Frequentlymetastatic lesions of the mandible are bilateral (Fig. 22-6, Band G). Also, lesions may be located in the peri-odontalligament space (sometimes at the root apex),mimicking periapical and periodontal inflammatorydisease, or in the papilla of a developing tooth.

Differential DiagnosisIn most cases a known primary malignancy is present,and the diagnosis of metastasis is straightforward. Mul-tiple myeloma may be confused with metastatic tumors;however, the border of multiple myeloma is usuallybetter defined than in metastatic disease. When a lesionstarts within the periodontal ligament space of a tooth,the appearance may be identical to that of a periapicalinflammatory lesion. A point of differentiation is thatthe periodontal ligament space widening from inflam-mation is at its greatest width and centered about theapex of the root. In contrast, the malignant tumorusually causes irregular widening, which may extend upthe side. of the root. An odontogenic cyst, if secondar-ily infected, may have an ill-defined border, giving asimilar appearance to a metastatic lesion. Invasion ofthe jaws by primary tumors of the overlying epitheliumsuch as squamous cell carcinoma may be indistinguish-able from metastatic disease but can be differentiatedby clinical examination.

Periphery and shape. Metastatic lesions may be mod-erately well demarcated but have no cortication orencapsulation at their tumor margins; they may alsohave ill-defined invasive margins (see Fig. 22-6, A). Thelesions are not usually round but polymorphous inshape. Both prostate and breast lesions may stimulatebone formation of the adjacent bone, which will be scle-rotic. The tumor may begin as a few zones of osseousdestruction separated by normal bone. Mter a timethese small areas coalesce into a larger, ill-defined massand the jaw may become enlarged.

Internal structure. Lesions are generally radiolucent, inwhich case the internal structure is a combination ofresidual normal trabecular bone in association withareas of bone lysis. If sclerotic metastases are present(i.e., prostate and breast), the normally ragged radiolu-cent area may appear as an area of patchy sclerosis, theresult of new bone formation. The origin of this newbone is not the tumor but stimulation of surroundingnormal bone. If the tumor is seeded in multiple regionsof the jaw, the result is a multifocal appearance (multi-ple small radiolucent lesions) with normal bonebetween the foci. Significant dissemination of metasta-tic tumor may give the jaws a general radiolucentappearance occasionally mimicking osteopenia.

ManagementThe presence of a metastatic tumor in the jaw indicatesa poor prognosis. If metastatic disease is present, in themajority of cases the patient will usually die within 1 to2 years. If the radiographic appearance is suspicious,an opinion from a dental radiologist should be soughtand tissue submitted for histologic analysis. Nuclearmedicine may be employed to detect other metastaticlesions. Isolated malignant deposits, if symptomatic,may be treated with localized high-dose radiation treat-ment. In the rare occasion that the jaw is the first diag-nosed site of malignant spread, it is imperative that thepatient be referred quickly to an oncologist so that theprimary lesion can be found and anticancer treatmentcan be delivered promptly. This treatment may take theform of chemotherapy, radiation therapy, surgery,immunotherapy, or hormone treatment.

Effects on surrounding structures. Metastatic carcino-mas may stimulate a periosteal reaction that usuallytakes the form of a spiculated pattern (prostate andneuroblastoma). Typical of malignancy, the lesioneffaces the lamina dura and can cause an irregular



B

A

D

c

FIG. 22-6 Metastatic carcinomas. A, Metastatic breast carcinoma surrounding the apicalhalf of the second and third molar roots and extending inferiorly. It has destroyed the infe-rior border of the mandible. B, Bilateral metastatic lesions from the lung destroying themandibular rami. C, Coronal CT image using soft tissue algorithm of the same case.D, Destruction of the left mandibular condyle (arrows) from a thyroid metastatic lesion.E, Axial CT image using soft tissue algorithm of the same case showing invasion into sur-rounding soft tissue (arrows).


4b~MALII..NAN I UI:.tA:.t:. UI- I Nt JAW:''-MAl- I ~K ~~

IS elevated and mamtalns Its osteogemc potential but ISbreached in the center, a Codman's triangle at theedges is formed (see Fig. ,22-1, E). Even more rarely,laminar periosteal new bone may be present. In manycases, extension is even more prominent, and a softtissue mass is visible radiographically.

Sarcomas

OSTEOSARCOMA

SynonymOsteogenic sarcoma

Internal structure. Osteosarcoma may be entirelyradiolucent, mixed radiolucent-radiopaque, or quiteradiopaque. The internal osseous structure may takethe appearance of granular- or sclerotic-appearingbone, cotton balls, wisps, or honeycombed .internalstructures in areas with adjacent destruction of the pre-existing osseous architecture. %atever the resultantinternal structure, the normal trabecular structure ofthe jaws is lost.

uennltlonOsteosarcoma is a malignant neoplasm of bone inwhich osteoid is produced directly by malignant stromaas opposed to a.djacent reactive bon~ formation. Thethree major histologic types are chondroblastic,osteoblastic, and fibroblastic osteosarcoma. The causeof osteosarcoma is unknown, but genetic mutation andviral causes have been suggested. It is also known tooccur in association with Paget's disease and fibrousdysplasia after therapeutic irradiation.

tttects on surrounolng structures. Widening of theperiodontal membrane is associated with osteosarcomabut is also seen in other malignancies (Fig. 22-8). Theantral or nasal wall cortices may be lost in maxillarylesions. Mandibular lesions may destroy the cortex ofthe neurovascular canal and adjacent lamina dura.Alternatively, the neurovascular canal may be symmet-rically widened and enlarged. Effects on the periosteumare discussed under the discussion on periphery.

Lllnlcal t-eaturesOsteosarcoma of the jaws is quite rare and accounts forapproximately 7% of all osteosarcomas. Despite itsrarity, the dentist may be the first health professionalwho observes a tumor involving the jaws. The lesionoccurs in all racial groups worldwide and in males twiceas frequently as females. Jaw lesions typically occur witha peak in the fourth decade, about 10 years later onaverage than long bone lesions. The most commonlyreported symptom or sign is swelling, which may bepresent as long as 6 months before diagnosis; theswelling is usually rapid. Other indicators are pain,tenderness, erythema of overlying mucosa, ulceration,loose teeth, epistaxis, hemorrhage, nasal obstruction,exophthalmos, trismus, and blindness. Hypoesthesiahas also been reported in cases involving neurovascularcanals.

Ulnerentlal uiagnosisIf internal structure is minimal or absent, fibrosarcomaor metastatic carcinoma may appear similar to osteosar-coma. If osseous structure is visible, the practitionershould also consider chondrosarcoma. If spiculatedperiosteal new bone is present, prostate and breastmetastases should be considered. Comprehensivephysical examination and laboratory tests assist indetermining whether the lesion is primary or metasta-tic. Benign tumors such as ossifying fibroma and benignconditions such as fibrous dysplasia may mimicosteosarcoma. The former conditions, however, areusually better demarcated and have a more uniforminternal structure. The histopathology of osteosarcomamay be interpreted as a benign fibroosseous lesion, andin these cases, the correct diagnosis may rely on theradiographic characteristics alone. Ewing's sarcoma,solitary plasmacytoma, and even osteomyelitis sharesome of the radiographic characteristics of osteosar-coma. Osteosarcoma is generally not associated withsigns of infection.

Kaolograprnc rea1:uresLocation. The mandible is more commonly affectedthan the maxilla. Although the lesion can occur in anypart of either jaw, the posterior mandible, including thetooth-bearing region, angle, and vertical ramus, is mostcommonly affected. The posterior areas are also morecommonly affected in the maxilla, with the most fre-quent sites being the alveolar ridge, antrum, and palate.The lesion may cross the midline.

rer'pnery ana snape. usreosarcoma nas an 111-aenneaborder in most instances. When viewed against normalbone, the lesion is usually radiolucent with no periph-eral sclerosis or encapsulation. If the lesion involves theperiosteum directly or by extension, one may see thetypical sunray spicules or "hair-on-end" trabeculae (Fig.22-7). This occurs when the periosteum is displaced,partially destroyed, and disorganized. If the periosteum

IvIClnClgt:mt:nlThe management of osteosarcoma is resection with alarge border of adjacent normal bone. This may be pos-sible in orthopedic cases but may be complicated by thepresence of important adjacent anatomic structures in


,470 PART V KALJIUtJKAPHIC INTERPRETATION OF PATHOLOGY

(;

tS

I-llJ. ZZ-7 A and IS, Kadlograpns ot a resected mandible ot a L.:>-year-old man withosteosarcoma, showing sunray spicules. C, Coronal CT image of an osteosarcoma ofthe maxilla; note the spiculated bone formation extending laterally from the maxilla(arrows).



mucosa unless secondarily ulcerated. If chondrosar-coma occurs in or near the temporomandibular jointregion, trismus or abnormal joint function may result.

Radiographic FeaturesLocation. Chondrosarcomas in the facial bones areunusual, accounting for about 10% of all cases. Theyoccur in the mandible and maxilla with equal fre-quency. Maxillary lesions typically occur in the anteriorregion in areas where cartilaginous tissues may bepresent in the maxilla. Mandibular lesions occur in thecoronoid process, condylar head and neck (Fig.. 22-9, Band C), and occasionally the symphyseal region.

Periphery and shape. Chondrosarcomas are slow-growing tumors, and their radiologic signs may be mis-leading and benign in nature. The lesions are generallyround, ovoid, or lobulated. Generally their borders arewell defined and at times corticated, whereas at othertimes they meld with adjacent normal bone. Occasion-ally peripheral periosteal new bone may be present per-pendicular to the original cortex, giving the so-calledsunray or hair-on-end appearance. Uncommonly theselesions are ill defined and invasive. Aggressive lesionssuch as these have infiltrative, ill-defined, and noncor-ticated borders.

FIG. 22-8 Cropped panoramic image of an osteosarcomaoccupying the body of the right mandible. Note thewidened ligament spaces (arrows) and that the density of themandible in the first molar region is greater than normal dueto abnormal bone formation from the tumor.

the head and neck. Generally radiation therapy andchemotherapy are used only for controlling metastaticspread or for palliation.

Internal structure. Chondrosarcomas usually exhibitsome form of calcification within their center, givingthem a mixed radiolucent-radiopaque appearance. Attimes this mixture takes the form of moth-eaten bonealternating with islands of residual bone unaffected bytumor. Lesions are rarely completely radiolucent. Thecentral radiopaque structure has been described asflocculent, implying snowlike features. This diffuse cal-cification may be superimposed on a bony backgroundthat resembles granular or ground-glass-appearingabnormal bone (see Fig. 22-9, A). Careful examinationof these areas of flocculence may reveal a central radi-olucent nidus, which is probably cartilage surroundedby calcification. The result is rounded or speckled areasof calcification.

CHONDROSARCOMA

SynonymChondrogenic sarcoma

DefinitionChondrosarcoma is a malignant tumor of cartilaginousorigin. The four histologic subtypes, which developmost commonly in the craniofacial region, are the clearcell, dedifferentiated, myxoid, and mesenchymal forms.They may occur centrally within bone, on the periph-ery of bone, or less commonly, in soft tissue. They canarise directly from cartilage or may occur within benigncartilaginous tumors. In the case of the latter, they aretermed secondary chondrosarcomas.

Effects on surrounding structures. Chondrosarcoma,being relatively slow-growing, often expands normalcortical boundaries rather than rapidly destroyingthem. In mandibular cases the inferior border oralveolar process may be grossly expanded while stillmaintaining its cortical covering. Maxillary lesions maypush the walls of the maxillary sinus or nasal fossa andimpinge on the infratemporal fossa. Lesions of thecondyle cause its expansion and perhaps remodelingof the corresponding articular fossa and eminence. Iflesions occur in the articular disk region, a widened

Clinical FeaturesChondrosarcomas generally occur at any age, althoughthey are more common in adults (mean age 47 years).They affect males and females equally. A patient withchondrosarcoma may have a firm or hard mass of rela-tively long duration. Enlargement of these lesions maycause pain, headache, and deformity. Less frequentsigns and symptoms include hemorrhage from tumoror from the necks of the teeth, sensory nerve deficits,proptosis, and visual disturbances. Invariably thetumors are covered with normal overlying skin or


\

472 PART V RADIOGRAPHIC INTFRPRFTATION OF PATHOI or:v

B

cFIG. 22-9 A, Chondrosarcoma of the anterior maxilla, with irregular calcification in theinternal structure of the tumor (arrows). B, Coronal CT image using bone algorithm of achondrosarcoma involving the mandibular condyle; note the two areas of bone destruc-tion. C, Axial CT scan using soft tissue algorithm, demonstrating the soft tissue extent ofthe lesion (arrows) and sparse calcifications. (A, Courtesy L. Hollender, DDS, Seattle, Wash.)

joint space may be present with corresponding remod-eling of the condylar neck. Erosion of the articular fossamay also occur. If lesions occur near teeth, root resorp-tion and tooth displacement may occur, as may widen-ing of the periodontal membrane space.

fications of chondrosarcoma may be absent fromosteosarcomfi, the two share many other radiologicfeatures; Fibrous dysplasia may also be difficult todifferentiate from chondrosarcoma because similaritiesin the internal pattern. (The radiopaque portion offibrous dysplasia is abnormal bone and not calcifica-tion. The calcifications in chondrosarcoma representcalcified cartilage.) Generally, the periphery of fibrousdysplasia is better defined and its margin with adjacent

Differential DiagnosisOsteosarcoma is often radiographically indistinguish-able from chondrosarcoma. Although the typical calci-



teeth differs from that of chondrosarcoma. Forinstance, fibrous dysplasia alters the bone pattern up toand including the lamina dura, leaving a normal or thinperiodontal ligament space. The greatest danger resultsfrom the misleading benign characteristics of chon-drosarcoma, which may delay correct diagnosis.

ptosis, epistaxis, ulceration, shifted teeth, trismus, andsinusitis. Cervical lymphadenopathy has also been

reported.

Radiographic FeaturesLocation. Mandibular cases outnumber maxillary casesby about 2 to 1, with the highest frequency found inposterior areas in both jaws. Generally the lesionsdevelop within the marrow space first and then extendto involve overlying cortical plates. This neoplasm rarelyoccurs in the jaws.

ManagementThe management of chondrosarcoma is surgical. Radi-ation therapy and chemotherapy generally have no roleto play. Patients ~th chondrosarcomas have a relativelygood 5-yearsurvival rate but poor 10-year survival rate.

Periphery and shape. Ewing's sarcoma is a radiolu-cency that is poorly demarcated and never corticated.Its advancing edge destroys bone in an uneven fashion,resulting in a ragged border. The lesions are usuallysolitary and may cause pathologic fracture with adja-cent radiographically visible soft tissue masses (Fig. 22-10). They may be round or ovoid but generally have no

typical shape.

EWING'S SARCOMA

SynonymsEndothelial myeloma and round cell sarcoma

DefinitionEwing's sarcoma is of indeterminate histogenesis. It isa tumor of long bones and is relatively rare in the jaws.Lesions arise in the medullary portion of the bone andspread to the endosteal and later periosteal surfaces.

Internal structure. Ewing's sarcoma is a destructiveprocess with little induction of bone formation.Because it commences on the internal aspect of thebone and involves the endosteal and periosteal surfaceslater in its course, it is usually entirely radiolucent.

Clinical FeaturesEwing's sarcoma is most common in the second decadeof life with most patients being between the ages of5 and 30 years. Males are twice as likely to manifestthe disease as females. In addition, multicentric lesionshave been reported. Other reported findings at 'thetime of presentation include, in descending frequency,swelling, pain, loose teeth, paresthesia, exophthalmos,

Effects on surrounding structures. Ewing's sarcoma maystimulate the periosteum to produce new bone. This isusually _the result of gross disturbances to the overlyingperiosteum and takes the form of Codman's triangleor the sunray or hair-on-end spiculation. Laminar

A D

FIG. 22-10 A and 8, Coronal CT images using bone algorithm, demonstrating Ewing'ssarcoma involving the left mandibular condyle; note the irregular margins, destruction ofthe medial cortex of the condyle, and a small pathological fracture (arrow).


474 RADIOGRAPHIC INTERPRETATION OF PATHOLOGYPART V

periosteal ne'w bone formation has been reported tooccur but is not a common feature of active Ewing'ssarcoma lesions. Adjacent normal structures such as themandibular neurovascular canal, inferior border of themandible, and alveolar cortical plates may be effaced.If the lesion abuts teeth or tooth follicles, the 'corticesof these structures are destroyed. This tumor does notcharacteristically cause root resorption, although itdoes destroy the supporting bone of adjacent teeth.

FIBROSARCOMA

DefinitionFibrosarcoma is a neoplasm composed of malignantfibroblasts that produce collagen and elastin. Theetiology is unknown, although it may arise secondarilyin tissues that have received therapeutic levels ofradiation.

Clinical FeaturesThese lesions occur equally in males and females witha mean age in the fourth decade. A slowly to rapidlyenlarging mass is the usual presenting symptom. Themass may be within bone, in which case it usually isaccompanied by pain. Peripheral lesions or thoseexiting from bone may invade local soft tissues, causinga bulky, clinically obvious lesion. If central or periph-erallesions reach a large size, pathologic fracture mayoccur. If fibrosarcomas involve the course of peripheralnerves, sensory-neural abnormalities may occur. Over-lying mucosa, although initially normal, may becomeerythematous or ulcerated. Involvement of the tem-poromandibular joint or paramandibular musculatureis often accompanied by trismus.

DifferentiaJ DiagnosisInflammatory or infectious lesions such as osteomyelitisof the jaw may share some of the radiographic featuresof Ewing's sarcoma. Although both lesions are radiolu-cent, osteomyelitis is likely to have demonstrable seques-tra present within the confines of the lesion, whereasEwing's sarcoma does not. Inflammatory lesions containsome sign of reactive bone formation, resulting in somesclerosis internally or at the periphery, and differ in theassociated periosteal bone formation.

Another destructive process that occurs in the samepart of the bone is eosinophilic granuloma of the jaw.This condition is associated with laminar periosteal bonereaction, whereas Ewing's sarcoma in the jaw is not. Theother central primary malignancies of bone such asosteosarcoma, chondrosarcoma, and fibrosarcoma maybe difficult to differentiate from this condition.

Radiographic FeaturesLocation. Most cases of fibrosarcoma of the jaws occurin the mandible, With the greatest number of theseoccurring in the premolar-molar region.Management

Too few cases of maxillofacial Ewing's sarcoma havebeen managed at any single treatment center for anyspecific treatment policy to have been adopted.Surgery, radiation therapy, and chemotherapy may beused alone or in combination.

Periphery and shape. Fibrosarcomas have ill-definedborders that are best described as ragged (Fig. 22-11).They are poorly demarcated and noncorticated andlack any semblance of a capsule. These tumors are

FIG. 22-11 A fibrosarcoma involving the right maxillary sinus has destroyed the corti-cal boundaries of the sinus, zygomatic process, hard palate and posterior maxilla, and the

alveolar process in this panoramic film.



ManagementThe management of fibrosarcoma is chiefly surgical.A wide margin of adjac~nt normal bone is takenif anatomically possible. Radiation therapy andchemotherapy are usually reserved for palliation.

generally shaped' in a fashion that suggests that theyhave grown along a bone; therefore they tend to beelongated through the marrow space. The radiographicborder may underestimate the extent of the tumorbecause these lesions typically are infiltrative. If softtissue lesions occur adjacent to bone, they may cause asaucer-like depression in the underlying bone or invadeit as would a squamous cell carcinoma. Finally, sclerosismay occur in the adjacent normal bone whether thefibrosarcoma is peripheral to bone or central.

.Internal structure. Fibrosarcomas have little internalstructure. In most cases the lesions are entirely radi-olucent. If the lesions have been present for some timeand are not overly aggressive, either residual jawboneor reactive osseous bone formation occurs.

Malignancies of the

Hematopoietic SystemMULTIPLE MYELOMA

SynonymsMyeloma, plasma cell myeloma, and plasmacytoma

DefinitionMultiple myeloma is a malignant neoplasm of plasmacells. It is the most common malignancy of bone inadults. Single lesions are called plasmacytoma, andmultiple lesions are termed multiPle myeloma.

Effects on surrounding structures. The most commoneffect on adjacent structures is destruction. In themandible, the alveolar process, inferior border of thejaw, and cortices of the neurovascular canal are lost. Inthe maxilla, the inferior floor of the maxillary sinus,posterior wall of the maxilla, and nasal floor can bedestroyed. In either jaw, lamina dura and follicular cor-tices are obliterated. Destruction of the outer corticalplate is usually accompanied by a protruding soft tissuemass. Root resorption is uncommon. Teeth are morelikt:ly to be grossly displaced and lose their supportbone so that they appear to be floating in space. Inaddition, widening' of the periodontal membr~nespace occurs with this tumor, as in other malignancies.Periosteal reaction is uncommon; however, if the lesiondisrupts the periosteum, a Codman's triangle or sunrayspiculation may be evident.

Clinical FeaturesMultiple myeloma is a fatal systemic malignancy. Apatient with multiple myeloma is usually between theages of 35 and 70 years (mean age 60 years). Thepatient may complain of fatigue, weight loss, fever, bonepain, and anemia, although the typical presentingfeature is low back pain: Secondary signs include amy-loidosis and hypercalcemia. In half of all patients, char-acteristic Bence Jones protein is present in the urine,which causes the urine to be foamy. The disease is morecommon in men. When this clonal cellular prolifera-tion occurs, these cells occupy first cancellous and latercortical bone, replacing the normally radiopaque bonewith areas of radiolucency.

Orally, patients may complain of dental pain,swelling, hemorrhage, paresthesia, and dysesthesia, orthey may have no complaints. The number of patientswith demonstrable radiologic findings in the jaws at thetime of diagnosis is relatively small.

Differential DiagnosisThis solitary, ragged radiolucency with little internalstructure is difficult to differentiate from other centralmalignancies. If the lesion does not cause enlargementof the jaw, the practitioner must rule out metastatic car-cinoma, multiple myeloma, and primary or secondaryintraosseous carcinoma. Another possibility is a grosslyinfected dental cyst, although this condition usuallyshows some degree of induced peripheral sclerosis inadjacent bone. If a fibrosarcoma exhibits enlargementof the affected jaw with an associated soft tissue mass,other sarcomas such as chondrosarcoma or osteosar-coma (both usually have internal structure) should beruled out. Ewing's sarcoma and radiolucent osteosar-comas may not be distinguishable from this tumor.Finally, peripheral invasive squamous cell carcinomashares some of these radiologic features, but its ulcera-tive surface features differentiate it from fibrosarcoma,which usually lacks these.

Radiographic FeaturesLocation. Multiple myeloma (Fig. 22-12) is seen morefrequently in the mandible than the maxilla but isuncommon in either. The incidence of jaw involvementhas been reported to vary from 2% to 78%. In themandible the posterior body and ramus is favored.Maxillary lesions usually appear in posterior sites.

Periphery and shape. The periphery of multiplemyeloma lesions is well defined but not corticated andlack any sign of bone reaction (Fig. 22-13). The lesionshave been described as appearing "punched out."However, many appear ragged and even infiltrative.


,476 PART V RADIOGRAPHIC INTERPRETATION OF PATHOLOGY

A

c

BFIG. 22-12 Multiple myeloma, seen as multiple circular radiolucent lesions in the skull(A). In a different case (8) multiple small lesions of multiple myeloma are present throughthe body and ramus of the mandible in this cropped panoramic image. C, Croppedpanoramic image shows a solitary lesion in the condylar neck region and a pathologicfracture (arrow).

Some lesions have an oval or cystic shape. Untreated oraggressive areas of destruction may become confluent,giving the appearance of multilocularity. If the lesion islocated in the periapical periodontal ligament space, itmay have a border similar to that seen in inflammatoryor infectious periapical disease. Soft tissue lesions havebeen reported in the jaws and nasopharynx. Whenvisible on radiographs, they appear as smooth-borderedsoft tissue masses, possibly with underlying bonedestruction.

unaffected by tumor, give the appearance of thepresence of new trabecular bone within the mass. Very'rarely the lesions appear radiopaque internally.

Effects on surrounding structures. If a good deal ofbone mineral is lost, teeth may appear to be "tooopaque" and may stand out conspicuously from theirosteopenic background. Lamina dura and follicles ofimpacted teeth may lose their typical corticated sur-rounding bone in a manner analogous to that seen inhyperparathyroidism. The same may be said of themandibular neurovascular canal, which, althoughusually visible, loses its cortical boundary in whole or in

Internal structure. No internal structure is radiographi-cally visible. Occasionally islands of residual bone, yet



if present with generalized radiolucency of the jaws andsimilar symptomology, can readily be confused withmultiple myeloma radiographically. Other metabolicdiseases such as thalassemia, Gaucher's disease oroxalosis may cause many of the changes similar to mul-tiple myeloma that are observed on dental radiographsbut may be ruled out on the basis of the medical history.

ManagementThe management of multiple myeloma is usuallychemotherapeutic, with or without autologous or allo-geneic bone marrow transplantation. Radiation therapymay be used for treatment of symptomatic osseouslesions when palliation is required.

NON-HODGKIN'S LYMPHOMA

SynonymsMalignant lymphoma and lymphosarcoma

FIG. 22-13 Panoramic radiograph depicting multipleareas of well-defined bone destruction lacking any corticalboundary. The lesions are multiple and separate and appearto be "punched out," typical of changes seen in multiplemyeloma. (Courtesy G. Petrikowski, DDS, Toronto, Ontario,Canada.)

DefinitionNon-Hodgkin's lymphoma is a malignant tumor of cellsnormally resident in the lymphatic system. In general,lymphomas occur within lymph nodes; however, extra-nodal sites such as bone, skin, gastrointestinal mucosa,tonsils, and Waldeyer's ring can be involved. The termnon-Hodgkin's lymPhoma describes a family of heteroge-neous tumors of varying type and severity. The classifi-cation of these diseases is difficult, and numerousmeans exist of subdividing these tumors. Currently theworking formulation for clinical usage classifies tumorsbased on their histologic appearance into low-grade,intermediate-grade, or high-grade tumors, with the lastclassification being the most aggressive.

part. These changes are profound when there is asso-ciated renal disease. Mandibular lesions may cause thin-ning of the lower border of the mandible or endostealscalloping. Any cortical boundary may be effaced iflesions involve them. Periosteal reaction is uncommon,but if present, it takes the form of a single radiopaqueline or more rarely a sunray appearance.

Clinical FeaturesNon-Hodgkin's lymphoma occurs in all age groups butis rare in patients in the first decade. The maxillarysinus, palate, tonsillar area, and bone may be sites ofprimary or secondary lymphoma spread. Lesions occur-ring outside lymph nodes in the head and neck arepresent in as much as lout of 5 cases. Patients may feelunwell, experiencing night sweats, pruritus, and weightloss. Palpable painless swelling, lymphadenopathy, andsensorineural deficits may accompany isolated lesionsof the jaws. Lesions present for some time may causepain and ulceration. Teeth resident ina lymphoma maybecome mobile, as the supporting bone is lost.

Differential DiagnosisThe most likely disease to be mistaken for multiplemyeloma is the radiolucent form of metastatic carci-noma. Knowledge of a prior malignancy in a patientmay help differentiate multiple myeloma from metasta-tic carcinoma. Osteomyelitis, if severe, may yield aradiologic picture similar to that of multiple myeloma,however, a visible cause for it usually exists. In addition,inflammatory lesions and infections in general causesclerosis in adjacent bone, whereas multiple myelomadoes not. Simple bone cysts may be bilateral in themandible and therefore may be mistaken for multiplemyeloma. They are usually corticated in part and char-acteristically interdigitate between the roots of the teethin a much younger population. Generalized radiolu-cency of the jaws may be caused by hyperparathyroidismbut is differentiated based on abnormal blood chem-istry. However, brown tumors of hyperparathyroidism,

Radiographic FeaturesLocation. Most non-Hodgkin's lymphomas of the headand neck occur in the lymph nodes. Those that areextranodal are likely to affect the maxillary sinus, pos-terior mandible, and maxillary regions.


RADIOGRAPHIC INTERPRETATION OF PATHOLOGY478 PART V

Periphery and shape. Most non-Hodgkin's lymphomasinitially take the shape a~d form of the host bone. Ifuntreated, however, they are capable of causingdestruction of the overlying cortex (Fig. 22-14). Theymay appear rounded or multiloculated and lack adefining outer cortex. Generally the borderS' are illdefined and invasive. Occasionally, lymphoma appearsas multiple areas of destruction, which likely appear asfingerlike extensions of malignant tumor cells in abuccal or lingual direction. Visible lesions occurring inthe maxillaJOY sinus or nasopharynx have a smooth

periphery.

ManagementThe management of extranodal or isolated nodaldisease is radiation therapy with or without concomi-tant chemotherapy. Treatment depends on histologicvariants and the location and extent of disease.

BURKITT'S LYMPHOMA

SynonymMrican jaw lymphoma

Internal structure. The internal structure of lymphomais almost always entirely radiolucent. It is rare to seereactive bone formation. Occasionally patchy radiopac-ity may be present, but this is rare.

DefinitionBurkitt's lymphoma is a high-grade B cell lymphomathat differs from other B cell lymphomas with respectto its histologic appearance and clinical behavior. It wasfirst described by Denis Burkitt in East Africa as Aftican

jaw lymPhoma.Two separate forms of the disease have been

described: the endemic African Burkitt's lymphomaand the American form. The latter is not characterizedby jaw involvement (although it occurs), but byinvolvement of abdominal viscera. African Burkitt'slymphoma affects young children, whereas AmericanBurkitt's lymphoma affects adolescents and youngadults. Cases of endemic and nonendemic Burkitt'stumor have been described throughout the world.

Effects on surrounding structures. In maxillary sinuslesions the antral walls may be effaced.and a soft tissuemass may be visible radiographically, either internallywithin the sinus or external to the maxillary sinus.Lesions involving the mandible destroy the cortex ofthe neurovascular canal. This tumor has a propensity togrow in the periodontal ligament space of mature teeth(Fig. 22-15). The cortex of the crypts of developingteeth may be lost when the lymphoma is located in thedeveloping papilla, and the involved teeth may bedisplaced in an occlusal direction and exfoliated.Periosteal reaction is not common but may take theform of laminated or spiculated bone formation. Withthe advent of soft tissue imaging with MR!, it hasbecome apparent that this tumor has a habit of growingalong soft tissue spaces (fat layers) and along thesurface of bone.

Clinical FeaturesThe disease affects more males than females. Clinicallythe hallmark of this tumor is rapidity of growth, witha tumor doubling time of less than 24 hours. It mayinvolve children as young as 2 years and adults intheir seventh decade, although it is primarily a diseaseof youth. Jaw tumors are rapidly growing and causefacial deformity very early in their course. They arecapable of blocking nasal passages, displacing orbitalcontents, causing gross facial swelling, and erodingthrough skin. These rapidly growing tumors are morecharacteristic of African Burkitt's lymphoma than theAmerican form and cause pain and paresthesia. Teethmay become rapidly loosened and alveolar bone grosslydistended. Paresthesia of the inferior alveolar nerve orother'sensory facial nerves is common.

Differential DiagnosisMultiple myeloma and metastatic carcinoma are easilyconfused with non-Hodgkin's lymphoma of the jaws.However, Ewing's sarcoma and Langerhans' histiocyto-sis, although also capable of producing the same effects,occur in a slightly younger age group. Osteolyticosteosarcoma and any of the central squamous cellcarcinomas may not be radiographically distinguish-able from non-Hodgkin's lymphoma. Squamous cellcarcinoma arising in the maxillary sinus may be diffi-cult to differentiate from lymphoma of the maxillarysinus. Other lesions that can displace developingteeth in an occlusal direction include leukemia andLangerhans' histiocytosis. Differentiation from apicalrarefying osteitis may be difficult; however, carefulinspection of the radiographic film may reveal thepresence of an infiltrative border and adjacent bonedestruction.

Radiographic FeaturesLocation. Extranodal disease is the norm in Burkitt'stumor. Mrican cases may involve one jaw or both themaxilla and mandible and affect the posterior partsof the jaws. By contrast, American cases may notinvolve the facial bones but are more likely to affectthe abdominal viscera and testes.

Periphery and shape. The lesions may begin as multi-ple, ill-defined, noncorticated radiolucencies, which


1-11... ~~- 14 A, t'anoramlc Image revealing d ITJdllglldlll

lymphoma invading the right maxilla. Note the ill-definedbone destruction and loss of the anterior aspect of thefloor of the maxillary antrum (arrows). The intraoral radio-graphs (B) also show ill-defined bone destruction and thelack of any bone reaction or formation.

"/~


,480 RADIOGRAPHIC INTERPRETATION OF PATHOLOGYPART V

A BFIG. 22-15 Cropped panoramic image (A) revealing an ill-defined lymphoma invadingthe left body of the mandible; note the irregular widening of the periodontal ligamentspaces (arrows) and intraoral films of the same case (8). Note the widened periodontalligament spaces (white arrows) compared with the normal periodontal ligament space ofthe right mandibular cuspid (black arrow).

later coalesce into larger, ill-defined radiolucencies withan expansile periphery. They are of no specific shape,although they expand rapidly and have been likened toa balloon. This expansion breaches its outer corticallimits, causing gross balloon-like expansion with thin-ning of adjacent structures and production of a softtissue tumor mass adjacent to the osseous lesion.Lesions that abut the orbital contents or the maxillarysinus may show a smooth surface soft tissue mass

radiologically.

alveolar canal is lost, although it is difficult to see in thenormal pediatric patient in any case. If periosteum isinvolved, the border may show sunray spiculation,although this is rare. Cases that involve the orbit dis-place the orbital contents, seen both clinically andradiologically.

Differential DiagnosisMetastatic neuroblastoma may give similar changesclinically and radiologically, as may Ewing's tumor.Osteolytic osteosarcoma can grow rapidly and may beindistinguishable from Burkitt's tumor on clinical andradiologic grounds. Cherubism has more internal struc-ture, does not breach bony borders, is bilateral, andgrows much more slowly. Finally, non-Hodgkin'slymphoma must be considered, although it occurs ina much older age group in most cases.

Internal structure. Burkitt's lymphoma does notproduce bone and rarely induces production of reac-tive bone within its center. For this reason, the lesionsare radiolucent in almost all cases. It is particularlyradiolucent in the jaw of a child.

Effects on surrounding structures. Erupted teeth inthe area of Burkitt's tumor are grossly displaced, asare developing tooth crypts. Tumor cells within thecrypt may displace the developing tooth bud to oneside of its crypt. A tumor that is located apical to adeveloping tooth may cause it to be displaced suchthat it appears to erupt with little if any root forma-tion. After tumor involvement of the developing dentalstructures occurs, root development ceases. Laminadura of teeth in the area is destroyed, and corticalboundaries such as the maxillary sinus, nasal floor,orbital walls, and inferior border of the mandible arethinned and later destroyed. The cortex of the inferior

ManagementThe management of Burkitt's tumor is chemothera-peutic. Chemotherapy regimens vary from geographiclocales, but the tumor is exquisitely sensitive to combi-nations of chemotherapeutic agents.

LEUKEMIA

SynonymsAcute myelogenous leukemia, acute lymphoblasticleukemia, chronic myelogenous leukemia, and chroniclymphocytic leukemia



ized around the periapical region of a tooth, giving theappearance of a rarefying osteitis.

DefinitionLeukemia is a malignant tumor of hematopoietic stemcells. These malignant cells displace normal bonemarrow constituents and spill out into the peripheralblood. They are subdivided into acute leukemias andchronic leukemias and further subdivided by the cell oforigin. The acute leukemias occur with a bimodal agedistribution, with very young patients and very oldpatients being the most commonly affected. Most casesof leukemia are associated with nonrandom chromoso-mal abnormaliti~s.

Periphery and shape. Leukemia must be considered asystemic malignancy, and as such its oral radiologic fea-tures may be present bilaterally as ill-defined, patchyradiolucent areas. With time and lack of treatment,these patchy areas may coalesce to form larger areas ofill-defined radiolucent regions of bone (Fig. 22-16).The teeth may appear to stand out conspicuously fromtheir surrounding, osteopenic bone.

Internal structure. The internal structure of leukemiais characterized by patchy areas of radiolucency andgeneralized radiolucency of the bone. Rarely, granularbone may be seen within these lesions; Occasionally,foci of leukemic cells may be present as a mass that maybehave like a localized malignant tumor. These lesionsare called chloromas and are rare in the jaws.

Clinical FeaturesThe patient with chronic leukemia may have no pre-senting signs or complaints. Acute leukemia patientsgenerally feel unwell with weakness and bone pain.They may exhibit pallor, spontaneous hemorrhage,hepatomegaly, splenomegaly, lymphadenopathy, andfever. Oral symptoms are generally absent but if presentinclude loose teeth, petechiae, ulceration, and boggy,

enlarged gingiva. Effects on surrounding structures. Leukemia does notcause expansion of bone, although occasionally a singlelayer of periosteal new bone may be seen in associationwith the disease, this being uncommon in chronicleukemia. Developing teeth in their crypts and teethundergoing eruption may be displaced in an occlusaldirection (Fig. 22-17) or into the oral cavity before rootdevelopment. Less commonly, developing teeth maybe displaced from their normal position. The result ofthis is premature loss of teeth. The lamina dura andcortical outlines of follicles may be effaced. If lesions

Radiographic FeaturesRadiologic signs associated with chronic leukemia arecomparatively rare.

Location. Leukemia affects the entire body, because itis a malignancy of bone marrow, which dischargesmalignant cells into circulating blood. Its manifesta-tions in the jaws may be seen more often in areas ofdeveloping teeth. Frequently, leukemia may be local-

A BFIG. 22-16 A and B, Periapical radiographs of the left mandible illustrating multifocalareas of bone destruction and widening of portions of the periodontal ligament space(arrow) characteristic of infiltration of the mandible with leukemia.



ologic examination may be more important than for ahealthy patient receiving a routine examination. Somepatients who have received a full course of radiationtherapy are concerned about the additional exposurefrom a dental radiographic examination. Such patientsshould be assured that this exposure is not a danger,because the small dose associated with dental radi-ographic examinations is negligible compared with theradiation dose received from cancer therapy.

The patient treated for head and neck malignancywith radiation therapy is prone to develop postradiationdental caries and osteoradionecrosis. Careful clinicalexamination and a thorough dental radiologic exami-nation may be required periodically to ensure that theremaining dentition and periodontal apparatus is ingood shape. Radiation caries occur in many patientsand appear clinically different from typical dentalcaries. If untreated, these carious teeth become nonvi-tal and may cause infection in the underlying jaw. Ifthey require extraction, healing can be expected to beslow and occasionally osteoradionecrosis may result.

The role of radiology in these patients, however, isnot restricted to examination of the teeth and sup-porting structures. Equally important is the monitoringof the outcome of treatment and specifically theexamination of dental radiographs for evidence oftumor recurrence, development of metastases, andosteoradionecrosis.

FIG. 22-17 Cropped panoramic film demonstratingocclusal displacement of the developing mandibular secondmolar out of its follicle (arrow).

affect the periodontal structures, the crestal bone maybe lost.

Differential DiagnosisGenerally, by the time oral radiologic signs of leukemiaare present, a. medical diagnosis has been reached.However, the development of radiologic changes maybe the first indication of the relapse of treatment. Occa-sionally lymphoma or neuroblastoma may mimic someof the features of destruction seen in leukemia. Meta-bolic disorders may be considered in those cases inwhich generalized rarefaction of bone is seen. Theseconditions are all excluded based on blood testing.With apical lesions, careful examination of the involvedtooth clinically and radiologically typically shows noapparent cause for rarefying osteitis.

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