intravascular papillary endothelial hyperplasia manifesting as a submandibular mass: an unusual...

Resorption of the zygomatic arch is a possible com-plication, especially in a multiple operated TMJ, be-cause every time a flap is raised there are morechances for degenerative osseous changes. This canbe a cause of concern for the patient because ofincreased facial asymmetry following zygomatic boneresorption. Moreover, the arch can easily fractureeven due to minor trauma and manipulation duringany subsequent surgery on the TMJ. The cases ofopen TMJ surgery should be followed for a long pe-riod of time to observe this complication. We haveseen resorption of zygomatic arch in 2 other casesfollowing open TMJ surgery. One should carefullycompare the sizes of zygomatic arches in preopera-tive and follow-up CT scans to observe this complica-tion. A long-term study with preoperative and post-operative CT scans in cases of open TMJ surgery canshed more light regarding this complication.

References

1. Vallerand WP, Dolwick MF: Complications of temporomandib-ular joint surgery. Oral Maxillofac Surg Clin North Am 2:481,1990

2. Keith DA: The long term unfavourable results in temporoman-dibular joint surgery, in Kaban LB, Pogrel MA, Perrott DH (eds):Complications in Oral and Maxillofacial Surgery. Philadelphia,PA, Saunders, 1997, pp 297-307

3. Dolwick MF, Armstrong JW: Complications of temporomandib-ular joint surgery, in Kaban LB, Pogrel MA, Perrott DH (eds):Complications in Oral and Maxillofacial Surgery. Philadelphia,PA, Saunders, 1997, pp 89-103

4. Keith DA: Success, failure and complications in temporoman-dibular joint surgery, in Keith DA (ed): Surgery of the Temporo-mandibular Joint. Boston, MA, Blackwell Scientific, 1997, pp298-315

5. Keith DA: Complications of temporomandibular joint surgery.Oral Maxillofac Surg Clin North Am 15:187, 2003

6. Levy MI, Monaco F: Resorption of the zygomatic arch afterelevation of a depressed fracture and subsequent osteomyelitis:Report of a case. J Oral Surg 36:220, 1978

J Oral Maxillofac Surg65:786-790, 2007

Intravascular Papillary EndothelialHyperplasia Manifesting as a

Submandibular Mass: An UnusualPresentation in an Uncommon Location

Don Kim, DDS, MD,* Howard Israel, DDS,†

Mark Friedman, DDS,‡ William Kuhel, MD,§

Claude-Jean Langevin, DMD, MD,� and Tara Plansky, DMD¶

Intravascular papillary endothelial hyperplasia (IPEH),also known as Masson’s tumor, intravascular angiomato-sis, and vegetant intravascular hemangioendothelioma,is a rare benign lesion of vascular origin caused by

abnormal proliferation of endothelial cells. Masson firstdescribed the lesion in a 1923 case report of a 68-year-old man with an ulcerated hemorrhoid that could not bereduced.1 Masson noticed that the internal lining of thelarge veins sprouted papillae that filled the lumen of thevein and were covered by endothelium.

IPEH occurs in all areas of the body, however, it hasa predilection for the head and neck region.2 Mostcommonly, it affects the skin and subcutaneous tissuesof the face and scalp, with the oral cavity being lessfrequently involved.3 A histological review of 103 con-secutive cases of vascular malformations of the oralcavity showed 6 cases of IPEH.4 The lower lip mucosawas the most common intraoral site, followed by thetongue and the upper lip. To date, there have been nodocumented cases of an IPEH in the submandibularspace associated with the submandibular gland. In thisarticle, we report an unusual case of IPEH located in thesubmandibular space that presented initially with com-mon symptoms of obstructive sialadenitis.

Received from the Department of Oral and Maxillofacial Surgery,

New York Presbyterial Hospital, New York, NY.

*Junior Resident.

†Full-time Faculty.

‡Oral Pathology Faculty.

§Otolaryngology Faculty.

�Former Chief Resident.

¶Former Chief Resident.

Address correspondence and reprint requests to Dr Kim: De-

partment of Oral and Maxillofacial Surgery, New York Presbyterian

Hospital, 525 E 68th Street, F2120, New York, NY 10021; e-mail:

[email protected]

© 2007 American Association of Oral and Maxillofacial Surgeons

0278-2391/07/6504-0032$32.00/0

doi:10.1016/j.joms.2005.11.095

786 INTRAVASCULAR PAPILLARY ENDOTHELIAL HYPERPLASIA

Report of a Case

An 18-year-old female presented for consultation with anoral and maxillofacial surgeon with a sudden painful swell-ing on the right side of her neck below the mandible. Theswelling had begun 48 hours prior to her presentation andwas noticed in the morning upon awakening. The swellingsubsided throughout the course of the first day but returnedin the morning of the following day, at which time shesought consultation with an oral and maxillofacial surgeon.She had no history of trauma, recent dental work or oro-pharyngeal infection, and her medical history was unre-markable. On clinical examination, her oral temperaturewas 37.6°C and pulse rate was 80 beats per minute. Generaland systemic examinations were normal.

Clinical examination of the neck showed a tender,fluctuant, nonpulsatile swelling measuring 5 � 4 cmextended from the right angle to midbody of the rightmandible and superiorly into the soft tissue in the sub-mandibular space (Fig 1). The overlying skin was normal,and there were no cervical lymphadenopathy. The masswas mobile, and there were no bruits, trills, trismus, orintraoral signs of infection. The nose, nasopharynx, andpharynx were normal on examination, and the cranialnerves were intact. Intraoral examination of the rightsubmandibular duct showed no salivary flow, or exudatefrom Wharton’s duct. Bimanual examination of the sub-

mandibular space did not show any palpable stones.Occlusal radiographs were obtained and no radiopaquestones were visualized. The initial diagnosis was obstruc-tive sialadenitis secondary to a lucent sialolith, or mucousretention plug, and the treatment recommendations in-cluded an increased oral hydration and clindamycin 300mg by mouth 3 times a day.

On the following day, there was significant decrease in theright submandibular swelling, with clear salivary flow from theright Wharton’s duct. The patient was maintained on clinda-mycin 300 mg 3 times a day (1 week) and increased hydration.She was asymptomatic until 2 weeks later when she presentedwith a recurrent right submandibular swelling, which begansuddenly in the morning upon awakening. Two occlusal ra-diographs were taken once again and no radiopaque stoneswere seen. The working diagnosis was recurrent obstructivesialadenitis secondary to a mucous plug or lucent stone, andthe patient was placed on the same treatment regimen, whichhad been prescribed initially. Four days later, the patient re-turned with an unresolved submandibular swelling. A rightsubmandibular sialogram was performed to obtain better visu-

FIGURE 1. Initial presentation shows a sudden-onset large rightsubmandibular swelling.

Kim et al. Intravascular Papillary Endothelial Hyperplasia. J OralMaxillofac Surg 2007.

FIGURE 2. First sialogram with multiple filling defects suggestingsubmandibular duct obstruction from mucous plugging or radiolucentstones.


FIGURE 3. Second sialogram with the addition of more contrastmedia, demonstrating one filling defect within the ductal system. Thereis better visualization of the proximal ductal architecture and resolutionof earlier obstruction. Note the void in contrast material inferior to theducts on both sialograms. Retrospectively, this filling defect reflected a“ball in socket” appearance indicating a space-occupying mass.


FIGURE 4. Axial CT with contrast showing right submandibular masslesion adjacent to the right submandibular gland. Note enhancingvascular structures within the lesion and compression of the rightsubmandibular gland.


KIM ET AL 787

alization of the ductal tree (Figs 2, 3). The sialogram showedmultiple filling defects in the ductal tree, consistent with mu-cous plugging or radiolucent stones. A void in the presence ofcontrast media was present inferiorly on the sialogram, how-ever, this was not considered a significant finding initially. Apanoramic radiograph obtained 15 minutes after the sialogramshowed no residual contrast dye present. Because the lastradiograph showed a functional submandibular gland withoutresidual dye, it was decided to continue to treat the patientwith increased fluid intake, clindamycin 300 mg 3 times a day,and physical “milking” of the submandibular gland. We pre-sumed that the mucous plugging may have been disruptedfrom the pressure generated by the contrast media and thatthis could have been a therapeutic sialogram.

The patient was asymptomatic for 1 week, however, shereturned with recurrent swelling of the submandibular re-gion, which was most prominent upon awakening in themorning. Due to the recurrent nature of this lesion, a com-puted tomography (CT) scan with contrast was obtained(Fig 4). This showed a mass in the right submandibularspace 5 cm in diameter. The mass was clearly separatedfrom the submandibular gland and showed it to be com-

pressing the gland itself. The lesion was low in density, andit displayed intralesional vascular enhancement on postcon-trast scanning. Calcification was notably absent.

Surgical removal of the mass was indicated and per-formed at New York Presbyterian Hospital, Weill CornellMedical Center, by a joint team from the Departments ofOtolaryngology and Oral and Maxillofacial Surgery. A sub-mandibular approach was used to gain access to the lesion,and a poorly encapsulated vascular mass was encounteredbeneath the superficial layer of deep cervical fascia. Themass was adherent to the capsule of the submandibulargland (Fig 5) and was excised in its entirety along with theright submandibular gland. A frozen section came back as abenign lesion with papillary structures (Fig 6).

The final histopathological examination showed the presenceof a myriad of papillary structures with central collagenized coreslined by a single layer of endothelial cells. The papillae formedirregular anastomosing clefts, and a large organizing thrombuswas seen within the dilated vessel (Figs 7-9). The surroundingstroma featured a mononuclear inflammatory cell infil-trate. There was no cytologic atypia or necrosis. Exami-

FIGURE 5. Intraoperative view of submandibular mass. Note thelocation of the lesion below the superficial layer of the deep cervicalfacial and its adherence to the submandibular gland.


FIGURE 6. Medium power H & E stain frozen section. Organizingthrombus and hyperplastic endothelial cells.


FIGURE 7. Low power H & E stain. Adhesion of lesion to thesubmandibular gland.


FIGURE 8. Low power H & E stain. Organizing thrombus.


788 INTRAVASCULAR PAPILLARY ENDOTHELIAL HYPERPLASIA

nation of the adjacent submandibular gland showed achronic sialadenitis with interstitial fibrosis.

The patient’s postoperative course was uneventful. Sixmonths after the surgery there were no symptoms or anyevidence of recurrence of swelling.

Discussion

There are 3 types of IPEH: 1) a primary form thatarises de novo in dilated vascular spaces; 2) a mixedform that occurs in preexisting varices, hemagiomas,and arteriovenous malformations; and 3) a rare extravas-cular form that arises in hematomas.5 IPEH typicallymanifests as a firm mass with possible tenderness andreddish-blue color to the overlying skin or mucous mem-brane. There is a wide age distribution from 9 months to80 years,6 and a slightly higher incidence in females.7

The typical signs and symptoms are nonspecific anddepend primarily on the anatomic location of the lesion.An accurate diagnosis of IPEH is challenging, as it isdifficult to differentiate IPEH from neoplastic and vascu-lar lesions solely based on clinical findings. Furthermore,the histopathology of IPEH resembles angiosarcoma8,9

and, therefore, differentiation between these 2 lesions isvery important, as the treatment approach for benignversus malignant disease is quite different. At one point,Masson considered this lesion to be a pseudoangiosar-coma because it failed to show invasion. IPEH differsfrom angiosarcoma microscopically in that the endothe-lial proliferative lesion is confined entirely to the vascu-lar lumen, and a solid, undifferentiated mass of cells withmitoses is lacking.9 Histologically, IPEH is a well-circum-scribed lesion consisting of stromal papillae with hyalinecores lined with endothelial cells. The papillary struc-tures often fuse to form irregular anastomosing vascularclefts, as seen with thrombosis.10

In our case, the proximity of the lesion to thesubmandibular gland posed another hurdle to ob-

taining an accurate diagnosis. There were acute andrecurrent episodes of submandibular swelling withWharton’s duct obstruction, highly suggestive ofobstructive submandibular gland pathology. Theinitial improvement with hydration and antibiotictreatment seemed to be consistent with obstructivesialadenitis secondary to mucus plugging or a lu-cent sialolith. The sialogram (Figs 2, 3) also sup-ported our initial clinical diagnosis, demonstratingfilling defects within the ductal tree. However, thefilling defect with a void in contrast media belowthe ductal system was misinterpreted initially. This“ball in socket” on the sialogram is characteristic ofa mass lesion that is displacing the ductal system.Because the histopathologic findings in the finalspecimen did show evidence of sialadentitis, it islikely that the IPEH caused intermittent obstructionof the ductal system. The clinical presentation ofrecurrent swelling, particularly in the morningupon awakening, represents the “wattle” sign.11

This is caused by a transient increase in swelling ofa vascular lesion, associated with the head being ina horizontal, dependent position.

The CT scan findings of IPEH often present asa homogenous, nonhomogenous, and constrast-enhanc-ing or nonenhancing lesion.12 This is related to thevarying amounts of parenchymal tissue and anasto-motic, stagnant, or low-flow vascular channels. In ourcase, after the CT scan showed a mass in the subman-dibular space, the differential diagnosis included pleo-morphic adenoma, adenoid cystic carcinoma, adenocar-cinoma, mucoepidermoid carcinoma, Hodgkin’s andnon-Hodgkin’s lymphoma, plunging ranula, dermoid/epidermoid cyst, and arterio-venous malformation.However, the intermittent regression in size of the le-sion, and distinct separation between the lesion and thesubmandibular gland, was uncharacteristic of the neo-plastic lesions that were included in our differentialdiagnosis. Our patient’s lesion was homogenous withareas of contrast enhancement, likely secondary to low-flow vascular channels.

The pathogenesis remains unknown, however, ithas been suggested that IPEH is a benign reactiveprocess, possibly related to trauma.13 Our patient wasunaware of any recent trauma to the submandibularregion, but the possibility of a forgotten minimaltrauma to the region existed.

The appropriate treatment for IPEH is conservativesurgical excision.14 In our case, the right submandibulargland was removed because the lesion was adherent tothe gland, and there was clinical evidence of subman-dibular gland obstruction. Frozen section showed thelesion to be benign and there was no need to remove anyadditional tissue. Recurrences are rare and only occurwhen the lesion is incompletely excised.15 The keyto proper management of this unusual lesion is

FIGURE 9. High-power H & E. Stromal papillae with central hyalinecores lined with endothelial cells. Note lack of inflammatory cells.


KIM ET AL 789

establishment of the correct diagnosis, primarilybased on histolopathologic findings. Because of thevariable location of IPEH, the clinical diagnosis of-ten mimics other pathologic conditions.

References1. Steffen C: The man behind the eponym. Am J Dermatopathol

25:71, 20032. McClatchey KD, Batsakis JG, Young SK: Intravascular angioma-

tosis. Oral Surg Oral Med Oral Pathol 46:70, 19783. Bodner L, Dayan D: Intravascular papillary endothelial hyper-

plasia of the mandibular mucosa. Int J Oral Maxillofac Surg20:273, 1991

4. De Courten A, Kuffer R, Samson J, et al: Intravascular papillaryendothelial hyperplasia of the mouth: Report of six cases andliterature review. Oral Dis 5:175, 1999

5. Salyer WR, Salyer DC: Intravascular angiomatosis: Develop-ment and distinction from angiosarcoma. Cancer 36:995, 1975

6. Clearkin KP, Enzinger FM: Intravascular papillary endothelialhyperplasia. Arch Pathol Lab Med 100:441, 1976

7. Amerigo J, Berry CL: Intravascular papillary endothelial hyper-plasia in the skin and subcutaneous tissue. Virchows Arch APathol Anat Histol 387:81, 1980

8. Moon WS, Chung GH, Hong KH: Intravascular papillary endo-thelial hyperplasia in a vascular lesion of the paranasal sinus.Arch Pathol Lab Med 124:1224, 2000

9. Stern Y, Braslavsky D, Spitzer T, et al: Papillary endothelialhyperplasia in the tongue: A benign lesion that may be mis-taken for angiosarcoma. J Otolaryngol 23:81, 1994

10. Pantanowits L, Muc R: Intravascular papillary endothelial hy-perplasia (Masson’s tumor) manifesting as a lateral neck mass.Ear Nose Throat J 79:806, 2000

11. Mandel L, Surattanont F: Clinical and imaging diagnoses ofintramuscular hemangiomas: The wattle sign and case reports.J Oral Maxillofac Surg 62:754, 2004

12. Stern Y, Braslavsky D, Segal K, et al: Intravascular papillaryendothelial hyperplasia in the maxillary sinus. A benign lesionthat may be mistaken for angiosarcoma. Arch Otolaryngol Headand Neck Surg 117:1182, 1991

13. Axiotix CA, Merino MJ, Ain K, et al: Papillary endothelialhyperplasia in the thyroid following fine-needle aspiration.Arch Pathol Lab Med 115:240, 1991

14. Chen TJ, Kuo T: Giant intracranial Masson’s hemangi-oma. Report of a fatal case. Arch Pathol Lab Med 108:555,1984

15. Katzman B, Caligiuri DA, Klein DM, et al: Recurrent intravas-cular papillary endothelial hyperplasia. J Hand Surg [Br] 22:113, 1997

J Oral Maxillofac Surg65:790-794, 2007

Pseudoaneurysm of the Maxillary ArteryAfter a Stab Wound Treated by

Endovascular EmbolizationZiad A. Ali, DMD, MD,* Didier D. Malis, DMD, MD,†

and James W. Wilson, DDS‡

Pseudoaneurysms of the maxillary artery are unusual butpotentially life threatening. Out of 394 reported cases ofpseudoaneurysms of the face and temple between 1644and 2005, only 35 involved the maxillary artery and itsbranches.1-8 The usual mechanism of these lesions iseither iatrogenic or traumatic. In traumatic cases, mostare secondary to high velocity injuries such as motorvehicle collisions or gunshot wounds.2,6,7 We present acase of pseudoaneurysm of the maxillary artery after a

low-impact penetrating injury. The pathophysiology andtreatment of such lesions will be reviewed.

Report of a Case

A 27-year-old Hispanic man with no significant past medicalhistory presented to the emergency room at Lyndon BainesJohnson Hospital in Houston, TX, with the chief complaint ofswelling involving the left lower jaw. The patient claimed thathe had been stabbed with a knife in the left side of the jaw 4weeks prior. At that time, he sought medical attention atanother hospital where paralysis of the left side of his face wasnoted and the laceration was sutured. Over the next few days,he noticed a gradually increasing swelling of the left side of hisface. The patient sought medical attention for the swelling at awalk-in primary care clinic where he was given a 1-week courseof antibiotics without resolution. The patient denied having sub-sequent dysphagia, odynophagia, or oral or external bleeding.

Clinical examination revealed a 10 � 8 � 3 cm swellingover the left angle of the mandible. A 2-cm well-healed scarwas present at the center of the swelling (Fig 1). Clear salivawas obtained from the parotid ducts. The mass was soft,pulsatile, and fluctuant. It was neither warm nor tender topalpation. A bruit was audible on auscultation. Completeleft facial paralysis of peripheral origin was evident by the

Received from the Department of Oral and Maxillofacial Surgery,

University of Texas Health Science Center at Houston, Houston, TX.

*Resident.

†Assistant Professor.

‡Associate Professor.

Address correspondence and reprint requests to Dr Wilson:

Department of Oral and Maxillofacial Surgery, University of Texas

Health Science Center at Houston, 6431 Fannin St, MSB 4.162,

Houston, TX 77030; e-mail: [email protected]

© 2007 American Association of Oral and Maxillofacial Surgeons

0278-2391/07/6504-0033$32.00/0

doi:10.1016/j.joms.2005.11.070

790 PSEUDOANEURYSM OF THE MAXILLARY ARTERY

intravascular papillary endothelial hyperplasia manifesting as a submandibular mass: an unusual...

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