Sudden, Unexpected Death Associated with Meningioangiomatosis: Case Report

University of California, San Diego, San Diego, California, United States
Pediatric and Developmental Pathology (Impact Factor: 0.87). 04/2005; 8(2):240-4. DOI: 10.1007/s10024-004-9105-4
Source: PubMed


We report a case of sudden, unexpected death associated with meningioangiomatosis in a 13-year-old, previously healthy male without a history of seizures, neurologic deficits, or clinical stigmata of neurofibromatosis. There was no family history of neurofibromatosis. The postmortem examination showed a 5-cm mass involving the right posterior frontal and orbital frontal cortex that had microscopic features diagnostic of meningioangiomatosis. Because no other cause of death was found, we postulate that he likely died as a result of a seizure secondary to meningioangiomatosis.

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    • "In cases of intractable seizures, resection is indicated(Jallo et al., 2005). At least one case of sudden death, presumably secondary to a fatal seizure, has been reported involving a previously-asymptomatic 13-year old boy(Wixom et al., 2005). "
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    ABSTRACT: Meningioangiomatosis (MA) is a rare congenital tumor that occurs mostly in 5-15 year old children. There have been only 5 cases previously reported that described the cystic nature within these tumors. We present a case of a MA accompanied by a separate macrocyst. A normally developed 2 year-old female patient presented with partial and generalized seizures. The brain computerized tomogram and magnetic resonance imaging revealed the presence of a calcified mass accompanied by a cyst in the right parietal area, surrounded by low density and high attenuation edema and hemorrhage. Upon right parietal craniotomy, a 1.6 cm x 1.2 cm x 0.5 cm sized plate-like, gray-white, slightly hard mass was seen and it was completely excised. Approximately 1 cm from the mass in the anterior lateral direction, a cyst was found and subsequent biopsy of the cyst wall revealed no tumor tissue, and therefore the cyst was not removed. Pathologic report demonstrated the meningioangiomatosis. Follow up examination 2 years later showed no recurrence of the tumor, and there was no evidence of neurological deficits. Authors suggest that cysts that arise in the surrounding tissues of tumors may not be tumor cysts, and do not require surgical removal.
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