Neuroblastomas and neuroendocrine carcinomas of the nasal cavity. A proposed new classification

Abstract

The histologic characteristics of 29 nasal tumors previously diagnosed as neuroblastomas, unclassified carcinomas, or unclassified malignant neoplasms were reviewed. Electron microscopy was performed in 17. Nine tumors were neuroblastomas; six of these were classical neuroblastomas while the other three exhibited olfactory differentiation in addition to the classical neuroblastoma component. Areas of ganglioneuroblastoma were found in the metastasis of one of the three olfactory neuroblastomas. Twenty tumors were classified as neuroendocrine carcinoma because all showed a neuroendocrine pattern with remarkably uniform cells growing from benign glandular epithelium; membrane bound granules were present in the cytoplasm of cells of the ten cases in this group examined by electron microscopy. The mean age of the patients with neuroblastomas was 20 years; survival in this group was 75% at five and seven years, respectively, and 67% at ten years. Recurrences, metastasis, and death occurred within 3 years of diagnosis. There was a low percentage (25%) of multiple recurrences. The metastases were located in cervical lymph nodes, brain and spine. The mean age of the patients with neuroendocrine carcinoma was 50 years. Survival was 100% at five years, 88% at seven years, and 77% at ten years. Recurrences and metastasis in 70% of the cases occurred later than the third year. Multiple recurrences were present in 54% of the cases. The metastases affected lymph nodes, brain and spine in all cases except in one in which lungs and femur were involved. In the latter case adenocarcinoma was also present in addition to the neuroendocrine carcinoma. Three patients died, all more than five years from the time of diagnosis. No correlation was found between staging and prognosis in either group, except for Stage I disease.

Esthesioneuroblastoma: a meta-analysis and review

“…These were supplemented by another 19 relevant publications found in references cited. 4,[9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26] The studies were divided into three types, according to their principal objectives: origin and aetiology of ENB, 9,11,15,[17][18][19]21,24,[27][28][29][30][31][32][33][34][35][36][37][38][39][40] histopathological diagnosis; 10,[12][13][14]23,37,[41][42][43][44]…”

“…23 Another aggressive neoplasm that must be distinguished from ENB is neuroendocrine sinonasal carcinoma, as originally suggested by Silva and colleagues. 10 Cytokeratin immunopositivity, especially in a punctate paranuclear distribution is the main diagnostic marker for differentiation from ENB. 51 Electron microscopy may be useful in demonstrating junctional complexes and tonofilaments.…”

Small cell neuroendocrine carcinoma of nose and paranasal sinuses: a study of three cases with short review of the literature

“…These cells contain membrane bound granules in the cytoplasm which can be demonstrated by Grimelius stain [4], electron microscopy [6] and their endocrine differentiation can be demonstrated by immunohistochemistry [4,7]. The tumour cells originate from the basal cells in the olfactory mucosa [8].…”

“…For staging of neuroendocrine tumours of sinonasal tract, the Kadish staging system is often used [8]. Accordingly the tumours are reported as stage-A (tumours limited to nasal cavity), stage-B (tumours localized in the nasal cavity and paranasal sinuses) and stage-C (tumours extending beyond nasal cavity and paranasal sinuses).…”

Non-Small Cell Neuroendocrine Carcinoma of the Sinonasal Tract and Nasopharynx. Report of 2 Cases and Review of the Literature

“…The development of a clinically meaningful and pathologically reproducible classification of NSNECs has been precluded by their rarity and confusion with other neoplasms such as ONB [9]. In its 2005 Classification of Head and Neck Tumors, the WHO categorized these neoplasms as ''typical carcinoid tumor'', ''atypical carcinoid tumor'', and neuroendocrine carcinoma ''not otherwise specified'' [1].…”