1. GROSS DESCRIPTION
— fine needle aspirate/biopsy/resection, e.g. rhinectomy, maxillectomy, ethmoidectomy, craniofacial resection/neck dissection.
Clinical presentation is with nasal obstruction, rhinorrhoea, epistaxis or facial pain. Investigation is by endonasal endoscopy with biopsy. Plain X-ray, CT and MRI scan can demonstrate and stage a soft tissue mass and any bone destruction. Tumour can be removed piece-meal by fibre-optic endoscopic (sinus) surgery (FE(S)S) or more formal resection used. Nasal septal lesions are excised via a lateral rhinotomy. Medial maxil-lectomy is the commonest procedure for low-grade tumours of the lateral nasal cavity, or maxillary, ethmoid and frontal sinuses, e.g. transitional papilloma or olfactory neuroblastoma. Craniofacial resection is for aggressive tumours or those of the frontal or ethmoid sinus that extend into the anterior cranial fossa. Orbital exenteration may be required if there is involvement of its bony wall. Neck dissection is carried out when there are proven metastases. These are complex specimens and they require careful marking by and liaison with the surgeon.
— nasal cavity, maxillary sinus, ethmoid sinus, sphenoid/frontal sinuses.
— maxillary and ethmoid sinuses are the commonest tumour sites.
— length x width x depth (cm) or maximum dimension (cm). Appearance
2. HISTOLOGICAL TYPE
Squamous cell carcinoma
— 85% of cases of sinonasal carcinoma.
— usually moderately differentiated keratinizing.
— verrucous: elderly, tobacco usage, broad based exophytic and "church spire" hyperkeratosis with a pushing deep margin of cytologically bland bulbous processes. Locally invasive (75% 5-year survival) but may become aggressive after radiotherapy.
— papillary: >70% exophytic or papillary malignant epithelial fronds with focal invasion at the base, 70% 5-year survival.
— spindle cell: polypoid and pleomorphic, cytokeratin (AE1/AE3—70%) positive, distinguish from sarcoma. A more obvious in-situ or invasive squamous component may be seen and nodal metastases can show a spectrum of epithelial and spindle cell changes. Prognosis (80% 5-year survival) relates to the depth of invasion.
— basaloid: poor prognosis, nests of palisaded basaloid cells with central comedonecrosis, hyalinised stroma.
— adenoid squamous: usual prognosis, acantholytic (pseudoglandular) pattern.
— adenosquamous: poor prognosis, mixed differentiation squamous carcinoma and adenocarcinoma (either obvious glands or solid with mucin positive cells).
— "transitional type": possible origin in Schneiderian papilloma which is a benign but locally recurrent sinonasal tumour. A papillary exophytic or inverted growth pattern neoplasm with features intermediate between transitional and squamous epithelia. Complicated by carcinoma in 3% of cases, which is either focal (good prognosis) or diffusely infiltrative (25% survival rate). Variable keratinization and differentiation.
— absence of squamous or glandular differentiation.
— particularly nasopharynx where it is EBV related and associated with a prominent lymphocytic component (lymphoepithelioma).
— cytokeratin/EMA positive.
Carcinoid/atypical carcinoid/small cell carcinoma/sinonasal neuroendocrine carcinoma
— polypoid/well-differentiated intestinal pattern/woodworker's tumour (wood dust exposure)/middle turbinate or ethmoid sinus/locally aggressive and recurrent. Prognosis related to the degree of glandular differentiation.
— mucoepidermoid carcinoma.
— acinic cell carcinoma.
— adenoid cystic carcinoma.
— adenocarcinoma of no specific type.
— rare (commoner in nasal cavity), 2% of malignant paranasal tumours, antrum, ethmoid, frontal sinuses.
— ± adjacent mucosal junctional activity.
— poor prognosis (most dead within 5 years).
— renal, lung, breast, gut, malignant melanoma.
Well/moderate/poor/undifferentiated, or Grade 1/2/3/4.
— for squamous carcinoma based on cellular atypia, keratinization and intercellular bridges.
— the majority are moderately differentiated but this varies according to tumour site and type, e.g. undifferentiated nasopharyngeal carcinoma (grade 4).
— for adenocarcinoma based on the percentage tumour gland formation (well/G 1 > 95%; moderate/G2 50-95%; poor/G3 < 50%; undifferenti-ated/G4 no glands).
4. EXTENT OF LOCAL TUMOUR SPREAD
Lymphocytic reaction: prominent/sparse.
The TNM classification applies only to carcinomas.
pTis carcinoma in situ.
Maxillary sinus pT1 tumour limited to the antral mucosa pT2 tumour causes erosion or destruction of bone (including extension into hard palate and/or middle nasal meatus), except posterior antral wall and pterygoid plates pT3 tumour invades any of: posterior wall maxillary sinus, subcutaneous tissues, floor/medial wall orbit, pterygoid fossa, ethmoid sinus(es) pT4 tumour invades or any of:
4a: anterior orbital contents, skin of cheek, pterygoid plates, infratemporal fossa, cribriform plate, sphenoid or frontal sinuses
4b: orbital apex, dura, brain, middle cranial fossa, cranial nerves (other than maxillary division trigeminal nerve V2), nasopharynx, clivus.
Figure 13.3. Maxillary sinus carcinoma.
Nasal cavity and ethmoid sinus pT1 tumour confined to one subsite of nasal cavity or ethmoid ± bone invasion pT2 tumour involves two subsites in a single site or extends to involve an adjacent site within the nasoethmoidal complex ± bone invasion
pT3 tumour extends to medial wall or floor of orbit, maxillary sinus, palate, or cribriform plate pT4 tumour invades any of:
4a: anterior orbital contents, skin of nose or cheek, minimal extension to anterior cranial fossa, pterygoid plates, sphenoid or frontal sinuses 4b: orbital apex, dura, brain, middle cranial fossa, cranial nerves other than V2, nasopharynx, clivus.
Invasion of bone includes only involvement of the spongiosa, not the cortex.
Presentation is not infrequently late with bone destruction already present.
5. LYMPHOVASCULAR INVASION
6. LYMPH NODES
Site/number/size/number involved/limit node/extracapsular spread. Regional nodes: cervical.
Level I: submental, submandibular
Level II: upper jugular
Level III: middle jugular
Level IV: lower jugular
Level V: posterior triangle.
A selective neck dissection will ordinarily include a minimum of six lymph nodes, a (modified) radical dissection 10 lymph nodes.
pN0 no regional lymph node metastasis pN1 metastasis in a single ipsilateral node < 3 cm pN2 metastasis in a. ipsilateral single node > 3 cm but <6cm b. ipsilateral multiple nodes < 6 cm c. bilateral, contralateral nodes < 6 cm pN3 metastasis in a lymph node > 6 cm.
7. EXCISION MARGINS
Distance (mm) to the nearest painted excision margin.
Due to anatomical limitations on resection, margins are usually only several millimetres.
Malignant tumours are more common than benign in the paranasal sinuses with the reverse being the case in the nasal cavity. Equivalent nasal cavity tumours have a better prognosis. Markers are of use in differentiating carcinoma from malignant melanoma and lymphoma. Fifty-five percent of sinonasal malignancies occur in the maxillary sinus, 35% in the nasal cavity and 9% in the ethmoid sinus. The majority of lesions
(85%) are squamous cell carcinoma and its variants with adenocarcinoma representing only 5-10% of cases. About 10% of patients present with nodal metastases.
Prognosis is strongly related to tumour stage. Treatment is by a combination of surgery and radiotherapy, with 5-year survival about 60%. Undifferentiated carcinoma has a very poor prognosis and histologically squamous and glandular differentiation are precluded by definition. Undifferentiated carcinoma of nasopharyngeal type is radiosensitive with remission in 80% and 10-year survival in 40%.
9. OTHER MALIGNANCY
— diffuse large B-cell lymphoma is commonest; CD20 positive.
— angiocentric T-cell lymphoma [sinonasal NK (natural killer)/T cell lymphoma]: destructive nasal/midline tumour with large areas of zonal necrosis and vasculocentric/destructive. It comprises polymorphic tumour cells (CD56 positive/CD3±) which may be hard to recognize amongst the inflammatory infiltrate. EBV associated and of poor prognosis. Serum ANCA levels help to distinguish it from Wegener's granulomatosis—another cause of sinonasal lethal midline granuloma.
— development of myeloma may take a number of years.
— K, % light chain restriction and clinical evidence of myeloma, e.g. elevated erythrocyte sedimentation rate (ESR), serum immune paresis, and monoclonal gammopathy, Bence-Jones proteinuria, radiological lytic bone lesions.
Rhabdomyosarcoma (embryonal—children), angiosarcoma, fibrosarcoma, malignant fibrous histiocytoma, chondrosarcoma, osteosarcoma, odontogenic tumours by direct spread.
Olfactory neuroblastoma, primitive neuroectodermal tumour (PNET)
— olfactory neuroblastoma: small, round blue cell tumour aggregates (lobules/nests ± rosettes) in a fibrillary stroma with calcification. Tumours confined to the nasal cavity have a reasonable prognosis while those in the nasal cavity and paranasal sinuses an intermediate outlook. Extranasal/paranasal and visceral lesions are of poor prognosis. Treatment is by a combination of surgery and radiotherapy. Overall 5-year survival is 50-60% with a tendency for late recurrences. Variably positive neuron-specific enolase (NSE), neurofilament, chro-mogranin, cytokeratin, S100 and glial fibrillary acidic protein (GFAP)
Immunohistochemistry aids in distinction from the differential diagnoses of malignant melanoma, lymphoma, plasmacytoma and embryonal/alveolar rhabdomyosarcoma. The less differentiated PNET tumour is MIC 2 (CD99) positive and variably neuroendocrine marker positive (NSE, PGP 9.5, neurofilament) but potentially responsive to high-dose irradiation and multi-drug chemotherapy (75% 5-year survival). There is overlap with cytoteratin positive sinonasal neuroendocrine carcinoma.
— locally destructive low-grade malignancy derived from notochordal remnants with characteristic vacuolated physaliphorous cells positive for S100, cytokeratins (CAM5.2, AE1/AE3, CK8, CK19), EMA and rarely CEA.
Ewing's sarcoma, malignant teratoma
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