Takashi Sogabe

Primary malignant rhabdoid tumor of the central nervous system: Case report and review of the literature

Malignant rhabdoid tumor (MRT) is well known as a pediatric malignant tumor of the kidney. Only nine cases of primary MRT of the central nervous system (CNS) have been reported. We are reporting in detail the clinical course and treatment of a patient with primary MRT of the CNS. We cared for a 3-year-old girl with an MRT that extended from the internal auditory canal to the cerebellopontine angle. Despite three surgical attempts at resecting the tumor combined with whole craniospinal axis irradiation, as well as chemotherapy consisting of intravenous nimustine hydrochloride and intrathecal methotrexate injections, the patient died 13 months after her initial hospitalization. The origin of CNS-MRT development is still a question of pathologic debate. Like renal MRT, the prognosis of MRT of the CNS is very poor. The dissemination of MRT of the CNS occurred in most cases.

Spontaneous rupture of craniopharyngioma cysts. A report of five cases and review of the literature.

Reports of spontaneous rupture of a craniopharyngioma cyst are extremely rare. Five cases of spontaneous rupture of a craniopharyngioma cysts are reported. Clinical symptoms included chemical meningitis in three patients, alleviation of headache in one, and improvement in a visual disturbance in one. Reduction in cyst size was confirmed by computed tomography or magnetic resonance imaging in three of five patients, and the histopathological diagnosis was confirmed histologically in four patients. Cerebrospinal fluid findings were abnormal in the three patients with chemical meningitis. Spontaneous rupture of craniopharyngioma cysts tended to occur more frequently in adult males. Computed tomography and magnetic resonance imaging were useful in diagnosing cyst rupture, and cerebrospinal fluid findings, especially the presence of cholesterol crystals and an elevated cholesterol concentration, are suggestive, even when no reduction in cyst size is observed radiologically.

Ganglioglioma of the optic pathway: A case report

A case of ganglioglioma of the optic pathway associated with congenital exophthalmos and strabismus is presented. Since the tumor extended from the right optic nerve to the right geniculate body, it was diagnosed as an optic glioma before operation. However, optic nerve biopsy showed that the lesion was a ganglioglioma. Although a literature review yielded two previous cases of ganglioglioma of the optic pathway, this is the first case in which the tumor involved the whole optic pathway.