Congenital meningeal sarcoma — A case report

Abstract

A 30-year-old multigravida woman was admitted to the obstetrical unit in the third trimester of gestation, because of a large-for-date uterus. Repeated ultrasonic examinations over a 2-week period revealed an increase in biparietal diameter far too large to be normal. This progressive asymmetrical hydrocephalus was diagnosed as caused by an intracranial space-occupying lesion in the middle cranial fossa. Because of the only minimal thickness of the cerebral cortex prognosis was judged so poor, that labor was induced. A stillborn female infant was delivered vaginally after an ultrasonically guided transabdominal and later transcervical encephalocentesis. Autopsy revealed hydrocephalus and section through the fixed brain showed that the mentioned mass was an undifferentiated meningeal sarcoma that filled the left middle cranial fossa. Microscopically the sarcoma was invading the brain tissue. Definitions of the term "congenital brain-tumors" vary among authors. "Congenital" can be interpreted as "derived from embryonal tissue" or as "originating in utero". Congenital intracranial neoplasms are very rare. Antenatally intracranial tumors have been reviewed by Wells, Solitare and Farwell. Only a few cases of congenital brain tumors arising in the meninges have been reported in these reviews. The genesis of the tumor is explained as derived from multipotential cells. The undifferentiated type of a meningeal sarcoma has a poor prognosis. Most meningeal sarcoma present with progressive hydrocephalus.(ABSTRACT TRUNCATED AT 250 WORDS)