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 olfactory neuroepithelioma, olfactory groove meningioma/haemangiopericytoma, sinonasal carcinoma, rhabdomyosarcoma, melanoma metastases, lymphoma, chordoma, juvenile nasopharyngeal angiofibroma, and pituitary macroadenoma.
Differential Diagnosis
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.
Esthesioneuroblastomas stain positive for S-100 protein and/or neuron-specific enolase, while the stain usually is negative for cytokeratin, desmin, 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. Rosettes consist of a central space ringed by columnar cells with radially oriented nuclei. Lymphoma can be excluded when the majority of tumor cells are negative for CD45 (the remaining positive cells demonstrate no atypical immunophenotype). Ewing sarcoma is positive for MIC2/CD99 gene products that result from an 11;22 translocation. [13] Melanoma can be identified using a combination of immunohistochemical markers: MART-1/Melan-A, HMB-45, and S-100. S-100 is expressed in more than 95% of melanomas. Rhabdomyosarcoma displays a loss of chromosome 11 and stains positive for desmin (expressed in 95%), muscle-specific actin, and myoglobin. Markel cell carcinoma stains positively for low-molecular-weight cytokeratin 20 and NSE. Neuroblastoma often stains positive for NSE, synaptophysin, Leu7, and neurofilament protein. Elevated serum catecholamines are also suggestive of neuroblastoma. Small cell carcinomas stain positively for chromogranin, NSE, and synaptophysin (presynaptic nerve cell vesicles). Most small cell carcinomas are positive for TTF-1.
The differential diagnosis of esthesioneuroblastoma includes the following:
- Olfactory neuroepithelioma
- Olfactory groove meningioma/haemangiopericytoma
- Sinonasal carcinoma (including SCC, minor salivary gland adenocarcinoma)
- Rhabdomyosarcoma
- Melanoma metastases
- Lymphoma
- Chordoma
- Juvenile nasopharyngeal angiofibroma
- Pituitary macroadenoma