Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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October 11, 2010
Olfactory Neuroblastoma (ONB)
- Historically referred to as esthesioneuroblastoma, ONB is a neural crest derived neoplasm arising from the olfactory mucosa.
- Bimodal age distribution, occuring in the second and sixth decades.
- Poor prognostic factors include female gender, age greater than 50 years, presence of metastasis.
- Five-year survival rate is approximately 69%.
- Key Diagnostic Features: Enhancing nasal cavity mass is seen. Often intracranial extension, and extension into the orbits and adjacent paranasal sinuses is noted. Intracranial extension can be associated with cysts along the margins of the tumor. Presence of such cysts is highly suggestive of ONB.
- DDx: Sinonasal undifferentiated carcinoma (SNUC), lymphoma, metastasis, menigioma.
- Treatment: surgical resection, radiotherapy, chemotherapy.
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