Rare Tumors

Olfactory Neuroblastoma

Author: André Mascarenhas Oliveira - Pathology Resident
Mayo Clinic, Rochester - USA

Olfactory neuroblastoma or esthesioneuroblastoma is a malignant tumor of the sinonasal region that, in contrast to neuroblastoma, affects mainly adults with no sex predilection^1,2. It is composed by small round cells which express neuroendocrine markers such as neuron-specific enolase, PGP 9.5, synaptophysin and chromogranin A¹ .

Nasal obstruction and epistaxis are the most common symptoms¹ and a case associated with Cushing’s syndrome has been described³. Local aggressiveness is the usual behavior but distant metastasis occur in 20% of cases, mainly to lymph nodes and lung⁴. The treatment includes surgery supplemented by radiotherapy and chemotherapy^1,5. The Five-year survival rate is superior to 50%^1,6.

References

1) Lund, V. J. & Milroy, C. Olfactory neuroblastoma: clinical and pathological aspects. Rhinology 1993; 31: 1-6.
2) Elkon, D., Hightower, S.I. et al. Esthesioneuroblastoma. Cancer 1979; 44: 1087-94.
3) Amensen, M.A., Scheithauer, B.W., Freeman, S. Cushing’s syndrome secondary to olfactory neuroblastoma.Ultrastruc Pathol 1994; 18: 61-8.
4) Rosai, J. (ed). Ackerman’s surgical pathology 8th ed. St. Louis, Mosby, 1996.
5) Jekunen, A.P., Kairemo, K.J et al. Treatment of olfactory neuroblastoma. Am J Clin Oncol 1996; 19 (4): 375-8.
6) Olsen, K.D., DeSanto, L. W. Olfactory neuroblastoma: biologic and clinical behavior. Arch Otolaryngol 1983; 109: 797-802.

If you have suggestions or comments send an e-mail to Andre Mascarenhas Oliveira

Back to Rare Tumors

Back to Pathology for Clinicians

Back to Medstudents' Homepage