AND 1 awdWt J

DOD 60%

Fig. 1.22 Sinonasal small cell carcinomas, neuroendocrine type (SCCNET) are aggressive tumours with a mortality rate of > 60% despite multimodal therapy. (DOD, dead of disease; AWD, alive with disease; AND, alive no disease; DOC, dead other causes).

type is a high-grade carcinoma composed of small to intermediate sized cells resembling those of small cell carcinoma of pulmonary or extrapulmonary origin. Necrosis, large numbers of apop-totic cells, high mitotic rate, and lack of neurofibrillary stroma are microscopic hallmarks of this tumour.

ICD-O code

8041/3

Synonyms

Small cell carcinoma, small cell neuroendocrine carcinoma, oat cell carcinoma, poorly differentiated neuroendocrine carcinoma.

Epidemiology

SCCNET of the sinonasal tract is a rare tumour with no sex, racial, or geographic predilection and no known association

Fig. 1.23 MRI of a small cell carcinoma, neuroendocrine type (SCCNET) involving the superior nasal cavity, ethmoid and sphenoid sinuses, with extensive invasion of skull base and frontal lobe.

with smoking or radiation. The age range is from 26-77 years with a mean of 49 years.

Localization

SCCNET most commonly arise in the superior or posterior nasal cavity, and often extend into the maxillary or ethmoid sinuses. Primary tumours of the maxillary or ethmoid sinuses without nasal involvement can be seen in approximately 45% of cases. Secondary involvement of the nasopharynx is present in a minority of patients. Advanced tumours may invade the skull base, orbit, or brain.

Clinical features

The most common symptoms are epistaxis and nasal obstruction, followed by facial pain, palpable facial mass, and exophthalmos. Rare tumours have shown elevated serum levels of ACTH, calci-

Fig. 1.24 Sinonasal carcinoid tumour. A Trabecular and insular patterns. B Diffuse and strong staining for neuron specific enolase (NSE).

Fig. 1.25 Small cell carcinoma, neuroendocrine type (SCCNET) A Typical sinonasal SCCNET showing confluent sheets of tumour cells surrounding vessels of nasal mucosa. B Sinonasal SCCNET composed of small cells with high nucleo/cytoplasmic ratio and extensive apoptosis. The nuclei are hyperchromatic and lack visible nucleoli. C Sinonasal SCCNET with perineural invasion and numerous hyperchromatic apoptotic cells.

Fig. 1.25 Small cell carcinoma, neuroendocrine type (SCCNET) A Typical sinonasal SCCNET showing confluent sheets of tumour cells surrounding vessels of nasal mucosa. B Sinonasal SCCNET composed of small cells with high nucleo/cytoplasmic ratio and extensive apoptosis. The nuclei are hyperchromatic and lack visible nucleoli. C Sinonasal SCCNET with perineural invasion and numerous hyperchromatic apoptotic cells.

tonin, pro-gastrin releasing peptide (pro-GRP), or antidiuretic hormone with syndrome of antidiuretic hormone and hyponatremia {1259,1901,2042}.

Pathology

See Chapter 3 under "Neuroendocrine Neoplasms of the Larynx." An important differential diagnosis is olfactory neuroblastoma.

Prognosis and predictive factors

SCCNET are aggressive tumours with a poor prognosis and frequent local recurrence and distant metastasis despite multimodal therapy. Among twenty reported patients {849,1259,1358,1901, 2009,2042,2134,2153,2728,2742}, twelve (60%) died of disease, three (15%) were alive with no evidence of disease, four were alive with disease (20%), and one died of other causes. In a study of extrapulmonary small cell carcinomas {845}, which included seven cases involving the paranasal sinuses, the median survival of 14 patients with pri

Fig. 1.26 Sinonasal SCCNET. A Patchy but strong staining for synaptophysin. Note the extensive tumour necrosis. B Diffuse and strong keratin expression. Note the characteristic perinuclear "dot". C Diffuse staining for CD56.

mary head and neck small cell carcinomas was only 14.5 months {845}. Follow-up data have shown a local recurrence rate of 45% and a distant metastasis rate of 35%. Common sites of metastases include cervical lymph nodes, lung, liver, bone marrow, and vertebrae.

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