Esthesioneuroblastoma or malignant lymphoma? A case report

Esthesioneuroblastoma is an uncommon tumor arising from the olfactory epithelium of the nasal vault. Differentiation from other tumors is often difficult, but techniques like immunocytochemistry and electron microscopy can help to distinguish a neuroblastoma from a malignant lymphoma with certainty. A patient is presented in whom difficulty was experienced in deciding on the diagnosis; the pathological data are presented to illustrate why confusion about the finite diagnosis can occur despite the use of these techniques. The initial diagnosis was a malignant lymphoma of the poorly differentiated lymphocytic type. The disease responded well to chemotherapy with cyclophosphamide, vincristine, procarbazine, and doxorubicin (COPA). When tumor recurred, rebiopsy of the small cell tumor was considered to be esthesioneuroblastoma, and the patient was treated with cyclophosphamide, vincristine, dacarbazine, and radiotherapy. The patient is now disease-free and has been without evidence of disease for more than 2.5 years; it is more than 5 years since the initial diagnosis of a small cell malignant neoplasm was made. This report illustrates the problems of diagnosis and treatment of this rare condition.