Esthesioneuroblastoma classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]

Overview

Esthesioneuroblastomas may be classified into two subtypes neuroblastoma proper and neuroendocrine carcinoma based on histopathology.

Classification

• Neuroblastomas proper
  • Neuroblastomas proper has a histologic presentation similar to that of peripheral neuroblastomas of childhood.
  • Esthesioneuroblastomas is composed of sheets of poorly demarcated groups of cells separated by fine connective tissue trabeculae.
  • The cells are small and typically show no mitotic activity.between the cells.
  • Rosettes of the Homer-Wright type are present.
  • On electronic microscopy, there is the presence of a dendritic cytoplasmic process with accumulations of small core granules within the process.
  • Neuroblastomas proper contain fibrillary material

• Neuroendocrine carcinomas.
  • Neuroendocrine carcinomas is admixture with glands.
  • In neuroendocrine carcinomas, a neurofibrillary component is absent, and the growth pattern is that of solid nests without rosettes.
  • Dense core granules similar to those of neuroblastoma are present in the cytoplasm and cytoplasmic extensions.

Further confounding accurate diagnosis is the fact that ENB is histologically similar to other small, round blue cell tumors. The acronym LEMONS ( lymphoma, Ewing sarcoma, melanoma, olfactory/ other [ENBs, rhabdomyosarcoma or Markel cell carcinoma], neuroblastoma, and small cell carcinoma) defines the other tumors from which ENB should be differentiated. Distinguishing ENBs from the other tumors is of paramount importance because the tumors respond differently to various treatment modalities.

The following list describes the outcome of each of these diseases with various

immunohistochemical tests.