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Presented by George Netto, M.D. & Kathryn Jockovic, M.D. and prepared by Andrea Subhawong, M.D.
Case 2: A 54 year old male presented with severe persistent headaches.
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1. Question
Week 398: Case 2
A 54 year old male presented with severe persistent headaches. A sinonasal mass was evident on MRI studies. A biopsy was performed.images/6.29.09.05a.jpg
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images/6.29.09.05e.jpgCorrect
Answer: Esthesioneuroblastoma (Olfactory neuroblastoma)
Histology: The tumor is composed of numerous relatively well-circumscribed lobules. The latter, consist of uniform cells with small round nuclei with dispersed “salt and pepper” chromatin pattern surrounded by only a scant cytoplasm. Nucleoli are inconspicuous. There is minimal nuclear pleomorphism, and necrosis is absent. Neurofibrillary matrix can be seen in the background.
Discussion: Esthesioneuroblastoma (Olfactory Neuroblastoma) is a malignant neuroectodermal tumor thought to arise from the olfactory membrane of the sinonasal tract. Esthesioneurblastoma is an uncommon neoplasm, which occurs over a wide age range with a peak incidence in the sixth decade. A bimodal age distribution, affecting those in the 2nd and 6th decades of life has been described. The tumor most commonly arises in the upper nasal cavity, in the region of the cribriform plate. The presenting symptoms include unilateral nasal obstruction, epistaxis, anosmia and headache.
The tumor cells are immunoreactive for neuron specific enolase (NSE), synaptophysin, and neurofilament protein (NF). Characteristically, S100 protein staining is limited to the periphery of the neoplastic lobules, in a sustentacular-like distribution. Focal positive reactivity with Cytokeratin should not exclude the diagnosis.
The differential diagnosis includes other small round cell malignancies which occur in the sinonasal tract, such as sinonasal undifferentiated carcinoma, lymphoma, rhabdomyosarcoma, and neuroendocrine carcinomas. Immunohistochemical stains can be of some utility in resolving the differential diagnosis.
Hyams’ grading system is commonly used for grading esthesioneuroblastoma. It incorporates several elements such as architecture, rosette formation, mitoses, necrosis, and pleomorphism. Hyams’ grading scheme has been shown to correlate with survival.
Reference(s):
– Hyams VJ, Batsakis JG, Michaels L (1988). Tumors of the upper respiratory tract and ear. 2nd ed. Armed Forces Institute of Pathology: Washington.
– Dulguerov P, Allal, AS, Calcaterra, TC. Esthesioneuroblastoma: a meta-analysis and review. Lancet Oncol 2001; 2(11): 683-690.Incorrect
Answer: Esthesioneuroblastoma (Olfactory neuroblastoma)
Histology: The tumor is composed of numerous relatively well-circumscribed lobules. The latter, consist of uniform cells with small round nuclei with dispersed “salt and pepper” chromatin pattern surrounded by only a scant cytoplasm. Nucleoli are inconspicuous. There is minimal nuclear pleomorphism, and necrosis is absent. Neurofibrillary matrix can be seen in the background.
Discussion: Esthesioneuroblastoma (Olfactory Neuroblastoma) is a malignant neuroectodermal tumor thought to arise from the olfactory membrane of the sinonasal tract. Esthesioneurblastoma is an uncommon neoplasm, which occurs over a wide age range with a peak incidence in the sixth decade. A bimodal age distribution, affecting those in the 2nd and 6th decades of life has been described. The tumor most commonly arises in the upper nasal cavity, in the region of the cribriform plate. The presenting symptoms include unilateral nasal obstruction, epistaxis, anosmia and headache.
The tumor cells are immunoreactive for neuron specific enolase (NSE), synaptophysin, and neurofilament protein (NF). Characteristically, S100 protein staining is limited to the periphery of the neoplastic lobules, in a sustentacular-like distribution. Focal positive reactivity with Cytokeratin should not exclude the diagnosis.
The differential diagnosis includes other small round cell malignancies which occur in the sinonasal tract, such as sinonasal undifferentiated carcinoma, lymphoma, rhabdomyosarcoma, and neuroendocrine carcinomas. Immunohistochemical stains can be of some utility in resolving the differential diagnosis.
Hyams’ grading system is commonly used for grading esthesioneuroblastoma. It incorporates several elements such as architecture, rosette formation, mitoses, necrosis, and pleomorphism. Hyams’ grading scheme has been shown to correlate with survival.
Reference(s):
– Hyams VJ, Batsakis JG, Michaels L (1988). Tumors of the upper respiratory tract and ear. 2nd ed. Armed Forces Institute of Pathology: Washington.
– Dulguerov P, Allal, AS, Calcaterra, TC. Esthesioneuroblastoma: a meta-analysis and review. Lancet Oncol 2001; 2(11): 683-690.
