Hamlat Abderrahmane, Adn Mahmoudreza, Caulet-Maugendre Sylvie, Guegan Yvon
Department of Neurosurgery, Centre Hospitalier Régional Universitaire Pontchaillou, Rennes, France.
Neurosurgery. 2004 Mar;54(3):745-51;discussion 751-2. doi: 10.1227/01.neu.0000108982.26949.f7.
Malignant fibrous histiocytoma in the central nervous system is uncommon. Fewer than 70 cases have been documented and, to the best of our knowledge, this is the first case arising from the cerebellum.
A 44-year-old woman presented with headaches, vomiting, and dizziness. A neurological examination revealed right cerebellar syndrome. Brain computed tomographic scans revealed an isodense tumor in the right cerebellar hemisphere. The breast ultrasonographic, bone scintigraphic, and thoracoabdominal computed tomographic findings were normal.
The patient was surgically treated. The tumor recurred 1.5 months later, demonstrating hemorrhagic characteristics on brain computed tomographic scans. The patient underwent a second operation, followed by radiotherapy.
Malignant fibrous histiocytoma is still a controversial entity, and the lack of specific criteria means that it must be diagnosed via the process of elimination. With currently available therapy, our review can provide only a very poor prognosis. The median survival time was 27 months. In attempts to develop better therapeutic strategies, total excision and radiotherapy seem to represent the best treatment approach.
