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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November 26, 2015
Esthesioneuroblastoma
- Background:
- Esthesioneuroblastoma (ENB) is an uncommon malignant tumor that arises from the olfactory epithelium. It was first described by Berger and colleagues in 1924.
- ENB constitutes aproximately 3% of all nasal neoplasms.
- Bimodal pattern of age distribution has been described, with peaks between 11–20 years of age and 40–70 years of age. A single peak during the sixth decade has also been reported.
- Relevant Clinical Information:
- Nasal pressure, epistaxis, anosmia, nasal obstruction, visual symptoms, SIADH, and Cushing syndrome
- Key Diagnostic Features:
- On CT, ENB tends to be isodense or hyperdense compared to the surrounding soft tissues. There is moderate contrast enhancement. Bone erosion and remodeling are common.
- On MRI, tumors may be hypointense on T1WI and isointense or hyperintense (areas of cystic degeneration or hemorrhage) on T2WI. Avid enhancement after the gadolinium administration. Heterogeneous in areas of necrosis.
- Differential Diagnosis:
- Sinonasal undifferentiatted carcinoma (generally in older patients)
- Squamous cell carcinoma (more common in maxillary antrum, and with less enhancement)
- Meningioma (may produce hyperostosis, and it is not associated with cysts at the tumor and brain interface)
- Lymphoma (does not enhance to same intensity as ENB)
- Treatment:
- Surgery, chemotherapy, and radiation therapy. Prognosis depends on the Hyams grade (ENB maduration), tumor extension (Kadish stage), and lymph node metastasis.
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