ABSTRACT
Burkitt's lymphoma is a malignant endemic neoplasia with a mandibular localization, described for the first time in 1958, in African children. The World Health Organization classification recognises several variants of Burkitt's lymphoma; all are highly malignant B cell lymphomas. Besides Burkitt's sporadic, endemic lymphoma and Burkitt's lymphoma associated with AIDS, the World Health Organization classification includes an "atypical or pleomorphic" variant of Burkitt's lymphoma. This subtype includes those cases diagnosed as "Burkitt-like" lymphoma in the REAL (Revised European-American Classification of Lymphoid Neoplasm). The therapeutic protocol is similar to that used for classic Burkitt's lymphoma, with chemotherapy being standard treatment. Prognosis is extremely poor, with a mean survival of < 1 year. The case is described of a sinus-nasal "Burkitt-like lymphoma", originating within sphenoid sinus. The extremely rare localisation of this histological variant of Burkitt's lymphoma is stressed as well as the extremely aggressive nature of the neoplasm.
