Tumors of nasal cavity & paranasal

sinuses

By

Dr, Ibrahim Habib (M.D)

ENT consultant

بسم هللا الرحمن الرحيم

{ مشهوداإلى غسق الليل وقرآن الفجر إن قرآن الفجر كان أقم الصالة لدلوك الشمس }

78: اإلسراء

Introduction

Cancers of nose & PNS : 3% of Head & Neck cancers .

Age : 5th up to 7th decade .

Predominately of older males .

Exposure:

Wood, nickel-refining processes

Industrial fumes, leather tanning

:Cigarette and Alcohol consumption

No significant association has been shown

location

1%

3%

20%

70%

• Floor : palatine process of maxilla

• Roof : cribriform plate .

Anatomy of maxillary antrum

Anterior : soft tissue of face .

Posterolateral : ITF , pterygopalatine F

Superior : Inferior orbital plate .

Inferiorly : hard palate ,

superior alveolar ridge

Anatomy of ethmoid sinuses

Anterior : lacrimal bone .

.Medialy : lateral nasal wall

Superior : Fovea ethmoidalis .

Anatomy of sphenoid sinus

Anteriorly : nasal cavity , ethmoid .

Posteriorly : clivus , brainstem .

Superiorly : pituitary fossa .

Laterally : cavernous sinuses & optic N .

Anatomy of frontal sinus

Anteriorly :

soft tissue of forehead .

Inferiorly :

orbit .

Posteriorly :

anterior cranial fossa .

1- frontal sinus

2- ant. Ethmoid sinus

3- infundibulum

4- middle. Ethmoid sinus

5- post. Ethmoid sinus

6- middle concha

7- sphenoid sinus

8- inf. concha

9- hard palate

Drainage of PNS

Maxillary sinus : middle meatus

Ethmoid sinuses “ anterior “ : middle meatus .

Ethmoid sinuses “ posterior “ : sphenoethmoid recess .

Sphenoid sinus : sphenoethmoid recess .

. Frontal sinus : frontonasal duct

Classification of sinonasal tumors

Malignant (non epithelial ) sinonasal tumours

Malignant (epithelial ) sinonasal tumours

Benign ( non epithelial ) sinonasal tumours

Benign ( epithelial ) sinonasal tumors

- chondrosarcoma . - Rabdomyosarcoma .

- squamous cell carcinoma : Differentiated . Basaloid squamous . Adeosquamous

Leiomyoma chondromyxoid fibroma

- Schneiderian papilloma : inverted . Papillary ( septal ). Cylinderical - Squamous papilloma ( nasal vestibule )

- lymphoproliferative Lymphoma Midline malignant reticulosis Plasmacytoma - Terato carcinosarcoma

Adenocarcinoma . Adenoid cystic . Mucoepidermoid

- Adenoma . - Dermoid

Hemangiopercytoma Angiosarcoma kaposi’sarcoma

Neuroendocrine carcinoma . Hyallinizing clear cell carcinoma

- Lobular capillary hemangioma . - Hemangiopericytoma . - peripheral nerve sheath tumors

Fibrosarcoma Osteogenic sarcoma Malignant fibrous Histocytoma

- Melanoma . - olfactory neuroblstoma . - sinonasal undifferentiated carcinoma (SNUC)

myxoma , fibromyxoma. ameloblastoma

- Fibrous histocytoma . - fibroma . - osteoma . - fibrosseus lesios .

N.B. Secondary malignancy – Melanoma ,Thyroid , lung , kidney and G I T

Squamous Cell Carcinoma

• Most common sinonasal malignancy • 70% arise in antrum • 30% arise in nasal cavity • 15% with synchronus or metachronus lesion • Pre or co-existing papilloma is risk factor • 4-9% • Look for necrosis on imaging N.B. Squamous Cell Carcinoma in Inverted Papilloma

Adenocarcinoma

• 13-19% of SN malignancies • Arise from surface epithelium and seromucinous glands • Intestinal, salivary, neuroendocrine types • Non-specific imaging features • Predilection for ethmoid sinuses

Adenoid Cystic Ca

• <10% of SN malignancies • 25% of adenocarcinomas • Glandular origin • Perineural growth pattern (60%) • Neural cell adhesion molecule (NCAM) in 93% • Small lesions extend beyond what is apparent • Difficult to entirely remove • Late recurrences and mets

Sinonasal Melanoma

• < 4% of SN neoplasms

• Melanocytes in mucosa

• Prefers nasal cavity

• Epistaxis

• Worse prognosis than

cutaneous types

• High recurrence and

mortality rates

Esthesioneuroblastoma

• Originate from olfactory epithelium • Two incidence peaks • Adolescence • 50 - 60 years • Epistaxis • High survival with multimodality therapy • Ca++ and peripheral cysts

Sinonasal Undifferentiated Ca (SNUC)

• Separate entity from SCCa, ENB, and others • Rare, high-grade malignancy • 2-3:1 male predominance • Broad age range from 3rd to 9th decades • Characterized by aggressive local growth, regional and distant mets, and poor survival

Sinonasal Lymphoma

• 44% of extranodal lymphomas arise in SN • Prefers nasal cavity • Types • T-cell (Asian) • B-cell (US, Europe) • T/NK-cell (LMG) • Remodeling or erosion • Homogeneous enhancement

Sarcomas and Other Malignancies

• Sarcomas • Rhabdomyosarcoma • Liposarcoma • Leiomyosarcoma • Fibrosarcoma • Chondrosarcoma • Osteosarcoma • Plasmacytoma • Metastases

symptoms

Early : asymptomatic .

Oral symptoms: 25-35%

, Toothache , trismus, alveolar ridge fullness, erosion , malocclosion .

Nasal findings: 50%

Obstruction, epistaxis, rhinorrhea , post nasal discharge , anosmia .

Ocular findings: 25%

Epiphora, diplopia, proptosis

Facial signs

Paresthesias, asymmetry

Physical examination

Nasal mass or polyposis .

Mass in the check or medical canthus .

Broadening of nasal dorsum .

Maxillary sinus involvement :

Mass in palate or upper alveolus .

Mass in upper gingivobuccal sulcus .

Malocclusion or loose teeth .

Advanced : Trismus .

Orbital :

Periorbital swelling , proptosis .

Epiphora , impaired occular mobility

Uncommon : Neck mass

Nasal endoscopy that shows a tumor in the left nasal wall

Investigations

Aim : detect the disease & its extention .

Extention : orbit , skull base , dura , Intracranial , great

vessels .

Presence of regional or distant metastasis

Presentation of tumours of nose & PNS

mass in check )) Nasal mass or polyposis

Broadening of nasal dorsum , proptosis , restricted occular mobility

C T scan

- Ideal

- surrounding bone erosion or destruction .

-Tumour :

Calification .

Soft tissue denisty

Necrosis or hge

Vascular tumors : enhancement increase with contrast

Entrapped secretion : with low density

.L.N Lymph node : regional L.N. , ( retropharyngeal )

. Staging

• Guide biopsy and surgery

• Treatment responseDistant metastasis .

Coronal section of nose & PNS shows soft tissue mass in region of Rt ethmoid air cell B))pushing septum to other side with bony erosion of septum and fovea ethmoidalis

CT Scan, of paranasal sinus, that shows the tumor( angiosarcoma ) in the left nasal cavity

MRI

Advantages :

- excellent delineation of tumour from surrounding inflammatory soft tissue and retained secretions.

- obtained in multiple planes .

- no exposure to ionizing radiation .

- no artifact in the presence of dental filling .

Figures 1 and 2: MR shows a 3.0 x 4.0-cm mass arising from the mucosa of the right ethmoid region with some areas of necrosis; the surrounding bony structure is intact

but its growth expands nasal septum and lamina papiracea -

inflammation secretion Tumour

Low signal No enhancement Intermediate signal T1

Low signal No enhancement Diffuse enhancement T1 with contrast

High signal High signal Intermediate signal T2

N.B. flow void --- vascular lesion . With contrast -- perineural invasion, dural or intracranial involvements L.N. -- Heterogenous on T2 , > 1 cm , peripheral enhancement with contrast using fat suppresion

Angiography

Indications :

1- Evaluations of vascular tumours extention , vascular anatomy ,

selective embolization .

2- Skull base surgery with brain retraction , delineate intracranial

arterial and venous anatomy .

3- tumour encroaching on carotid a. , assess collaterals , may be

used with balloon occlusion testing .

P.E.T. - Agent : 18 – F flurodeoxy glucose .

C – 11 methionine .

- Principle : image metabolic activity of head & neck . Tumors including nose

& PNS

-Assess : Local , regional or systemic metastasis .

-. Direct biopsy

• Therapy response

• Recurrence vs.

treatment change

• Re-staging

- Result : inferior to C.T. & MRI .

Biopsy

Aim : confirm diagnosis & plan appropriate ttt.

Route : 1- transnasl .

2- transoral .

3- direct access to the sinus :

Maxillary sinus : Transnasal , medial wall of

maxillary sinus .

Caldwell – Luc . Procedure .

-Ethmoid sinuses : Endoscopic ethmoidectomy

External ethmoidectomy .

Sphenoid sinus : endoscopically

Trans – septally

Frontal sinus : its floor .

Staging of sinonasal tumours

Ohngern 1933 staged maxillary sinus cancers(Suprastructure)

Ohngern 1933 staged maxillary sinus cancers (Infrastructure )

Suprastructure to Ohngern line Infrastructure to Ohngern line

Site

Late

Early Symptoms

Pterygomaxillary fossa , middle & anterior cranial fossa

Oral , nasal , I.T.F Spread

Less amenable to surgical resection More amenable to surgical resection

Treatment

Bad Good

prognosis

Ohngern line : an imaginary line drawn from maxillary tuberosity to inner canthus . Ohngern 1933 staged maxillary sinus cancers

Staging of non maxillary sinonasal malignancies

Stage I : tumor confined to site of origin .

Stage II : spread to adjacent sinuses , skin , nasopharynx ,

ptergomaxillary fossa , and or orbit .

Stage III : involvement of skull base , pterygoid plate and

or intracranial extension .

Staging system for olfactory neuroblastoma

Stage I : confined to primary site .

Stage II : presence of nodal metastasis .

Stage III : presence of distant metastasis .

AJCC staging for PNS primary tumor ( T ) of maxillary sinus

- Tx primary T can’t be assessed .

- To : no evidence of primary T.

- Tis : carcinoma in situ .

- T1 : T limited to antral mucosa with no erosion nor

destruction of bone .

- T2 Tumour causing erosion or destruction except for

posterior antral wall , including extention into m.m. of

hard palate and / or middle nasal meatus .

AJCC staging for PNS primary tumor ( T ) of maxillary sinus

- T3 Tumour invade any of the following : bone of posterior wall of

maxillary sinus , subcutaneous tissue , skin of check , floor or

medial wall of orbit , I.T.F. , pterygoid plates , ethmoid sinuses .

- T4a (resectable): anterior orbit,

skin, infratemporal fossa, pterygoid

plates, cribriform plate, frontal or

sphenoid sinuses

- T4b (unresectable): orbital apex,

dura, brain, middle fossa, clivus,

nasopharynx, CNs (other than V2)-

Staging of ethmoid sinus

- T1 tumour confined to the ethmoid with or without bone

erosion .

- T2 Tumour extends into nasal cavity .

- T3 Tumour extends into ant. Orbit and / or maxillary

sinus .

- T4 Tumour with intracranial extension , orbital

extension including apex , involving sphenoid and / or

frontal sinus and / or skin of external nose .

Nodal involvement in sinonasal tumours

. Nodal involvement infrequent despite advanced stage

• Depends on primary site, extent, and histology

• 8-18% with nodes at presentaion

. Nodal stage based on:

• Number

• Uni- or bilateral

• Size

-Nodal drainage

• Facial, parotid, submandibular

• Retropharyngeal

• Then L II

N1: Single ipsilat ≤ 3cm • N2: • a: Single ipsilat 3 – 6cm • b: Multiple ipsilat ≤ 6cm • c: Bilat or contralat ≤ 6cm • N3: ≥ 6cm node

staging - stage o Tis No Mo

- stage I T1 No Mo

- stage II T2 No Mo

- stage III T3 No Mo

- T1-T3 N1 Mo

- stage IV A T4 No Mo

T4 N1 Mo

- stage IV B any T N2 Mo

any T N2 Mo

- stage IV c any T any N M1

( N ) lymph node . ( M ) distant metastasis .

TNM Staging of Maxillary Carcinomas

• Stage I: Limited to mucosa

• Stage II: Bone involvement

(NOT posterior wall)

• Stage III:

• T3 lesion

• TI or T2 lesions with N1

• Stage IV

• T4 lesion

• Any T with N2/N3 or M1

Management of sinonasal tumours

Indication Surgical management of

Advanced primary lesion

Indication Surgical management of early primary

lesion

advanced lesions confined to maxillary sinus advanced lesions confined to maxillary sinus

Radical maxillectomy lesions confined to floor of maxillary sinus .

Infrastructure maxillectomy

extension of disease into the frontal sinuses and / or cribriform plate

Craniofacial resection lesions confined to medial wall of maxillary sinus

Medial maxillectomy

disease is extended into brain , sphenoid rostrum , cavernous sinus & internal carotid a

Palliative radiotherapy

lesions confined to septum

Partial or complete septectomy

Midfacial degloving approach.. Surgical Treatment of Squamous Cell Carcinoma of the Sinuses.

Combined bicoronal approach and Dieffenbach-Weber-Fergusson incision. Surgical Treatment of Squamous Cell Carcinoma of the Sinuses..

N.B. Orbital complications where R.T.

Indication Management of orbit in sinonasal tumors

complications with pre-operative R.T. are mostly minor and transient .

epiphora , keratitis , diplopia , pain ,

exophthalmos , and loss of vision .

cases with minimal periorbital involvement without full penetration into the orbital fat .

Resection of a small portion of the periorbita & reconstruct with fascial graft

complications are more frequent when post operative R.T. is used

with invasion of the periorbita , the infraorbital nerve , or the orbital apex

Resection of orbit

Reconstruction and Prosthetic Rehabilitation

- Aim : - prevent contracture of the check , to separate

oral & nasal cavities , and to provide support for the

globe .

- An obturator should be made preoperatively from an

impression of the hard palate .

. Algorithm to depict tissue options for midface reconstruction

Treatment of maxillary sinus carcinoma(A) 66-year-old woman with total maxillectomy defect and orocutaneous fistula status after surgery and radiotherapy. (B) Cranial bone grafts used to

reconstruct orbitozygomatic structure surrounded by rectus abdominus free flap. (C) 3-year postoperative result. (D) Intraoral view of 3-year postoperative result.

Management of tumours of nose &

PNS (1) The Neck

No :

T1 – T2 :

electve ND is not generally performed.

T3 – T4 :

R.T. post. Operative . Upper neck & retro-ph. L.Ns .

N+ve with resectable 1ry :

MRND . Or dissect 1-V & retropharyngeal chain .

Management of tumours of nose &

PNS late node metastasis)) (1) The Neck

- 5 – 45% occure after 2-3 yrs .

- rarely occurs in absence of synchronous local or distant

recurrence you should search for .

- TTT aggressively : R.N.D.

- 5 yr survival rate was 39% after ttt of delayed metastasis

.

- N.B. None with nodes at presentation survived 3 years .

Radiotherapy as an adjuvant therapy in

management of sinonasal tumours

- 1- combined with surgery in advanced resectable

lesions . Pre. Or post. Operative .

- 2- Single modality for :

- advanced unresectable lesions .

- patients unwilling or unable to undergo surgery .

- Average 5 yrs survival rates 10 – 15 % ( total doses up to 79 Gy ) .

chemotherapy as an adjuvant therapy in

management of sinonasal tumours

- Combination chemotherapy with pre. Or post.

Operative R.T. in :

- Olfactory neuroblastoma & SN undifferentiated ca.

- Japanese researchers use combination of R.T. , intra-

arterial 5 – fluorouracil ( 5 FU ) and local debridement

or cryosurgery for maxillary sinus cancer .

- Knegt ‘s regimen in using topical chemotherapy as an

adjuvant Therapy in management of sinonasal tumours

.The regimen

1-antrostomy and debulking of the tumour .

2-The tumour bed is then packed with topical 5FU

emulsion .

3- The pack are removed and any residual necrotic

material is debrided as often as necessary .

He reported 5-yr survival of 71% .

prognosis

The advancement of skull base surgery , cure rates for

patients with sinonasal tumours ,

form 39-76% have been achieved

Tumours have good chance of cure :

1- early maxillary tumours .

2- patients with nasal cavity tumours .

3- well differentiated adenocarcinoma 90% .

4- low grade minor salivary gland tumour .

5- olfactory neuroblastoma :

100% stage A & 75% stage B & 60% stage C . Survival .

6- sq. cell ca. arising in inverted papilloma .

Tumours with bad prognosis

1- Advanced maxillary cancer .

2- lesions involving pterygoid plates or

pterygopalatine fossa .

3- lesions involving brain , dura , nasopharynx ,

sphenoid .

4- lesions involving orbital contents .