Esthesioneuroblastoma differential diagnosis

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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

Esthesioneuroblastoma must be differentiated from other tumors with similar histological appearance, such as lymphoma, Ewing sarcoma, melanoma, rhabdomyosarcoma or Merkel cell carcinoma, and small cell carcinoma. Distinguishing esthesioneuroblastomas from the other tumors is of paramount importance because the tumors respond differently to various treatment modalities.

Differential Diagnosis

The differential diagnosis of esthesioneuroblastoma includes the following:

• Olfactory neuroepithelioma
• Olfactory groove meningioma/haemangiopericytoma
• Sinonasal carcinoma (including SCC, minor salivary gland adenocarcinoma)
• Rhabdomyosarcoma
• Nasopharyngeal carcinoma
• Melanoma metastases
• Lymphoma
• Chordoma
• Juvenile nasopharyngeal angiofibroma
• Pituitary macroadenoma

The outcome of following diseases with various immunohistochemical tests is shown below in a tabular form:

Disease	Immunohistochemical Tests
Esthesioneuroblastoma	Esthesioneuroblastomas stain positive for neuron-specific enolase and/or S-100 protein, The stain is negative for desmin, cytokeratin, vimentin, actin, glial fibrillary acidic protein, UMB 45, and the common leukocytic antigen. For difficult cases, electron microscopy can be useful. Common features are small, round neuroepithelial cells arranged in rosette or pseudorosette patterns, separated by fibrous elements. The rosettes consist of a central space ringed by columnar cells with radially oriented nuclei.
Lymphoma	Lymphoma can be excluded when the majority of tumor cells are negative for CD45 (the remaining positive cells demonstrate no atypical immunophenotype).
Ewing sarcoma	Ewing sarcoma is positive for MIC2/CD99 gene products that result from an 11;22 translocation.
Melanoma	Melanoma can be identified using a combination of immunohistochemical markers: HMB-45, MART-1/Melan-A, and S-100. S-100 is expressed in more than 95% of melanomas.
Rhabdomyosarcoma	Rhabdomyosarcoma displays a loss of chromosome 11 and stains positive for desmin (expressed in 95%), muscle-specific actin, and myoglobin.
Merkel cell carcinoma	Merkel cell carcinoma stains positively for low-molecular-weight cytokeratin 20 and NSE.
Neuroblastoma	Neuroblastoma often stains positive for NSE, synaptophysin, Leu7, and neurofilament protein. Elevated serum catecholamines are also suggestive of neuroblastoma.
Small cell carcinomas	Small cell carcinomas stain positively for chromogranin, NSE, and synaptophysin (presynaptic nerve cell vesicles). Most small cell carcinomas are positive for TTF-1.