Une tumeur primitive rare de l’angle pontocérébelleux : le schwannome mélanocytique, une tumeur pigmentée au pronostic réservé

Abstract

Schwannomas are common ubiquitous benign tumours of the nervous sheaths. Among the various histopathological types, the pigmented schwannoma has to be distinguished from melanoma. We report a case of melanotic schwannoma of the cerebellopontine angle in a 52-year-old man with a slowly progressive cerebellar syndrome since 1978, who presented with a posterior cerebral fossa bleeding. Melanotic schwannoma is a rare form of schwannoma. The diagnosis remains difficult and melanotic schwannoma has been described as a part of the Carney complex. Distinguishing melanotic schwannoma from malignant melanoma is of paramount importance in planning the management, but these two tumours are probably sharing the same origin. It must be distinguished from classic schwannoma because its behaviour is unpredictable, requiring long-term follow-up.