Glioblastoma in a patient with tuberous sclerosis who required emergency surgery to relieve elevated intracranial pressure

Abstract

  Tuberous sclerosis complex (TSC) is a disease characterized by systemic hamartoma. Among brain tumor lesions associated with TSC, subependymal giant cell astrocytoma (SEGA) is a common complication, whereas glioblastoma is rarely reported. A 9-year-old boy was diagnosed with tuberous sclerosis due to the onset of infantile spasms at 5 months of age. Head CT revealed a subcortical nodule accompanied by a calcification in the left frontal lobe. He experienced several episodes of sudden vomiting without trigger for 2 weeks. Head CT showed a neoplastic lesion with hemorrhage in the left frontal lobe. After several hours, frequent vomiting and consciousness disorder were observed. Re-examination of head CT revealed that intratumoral hemorrhage was rapidly exacerbated and intracranial pressure was elevated. Thus, urgent tumor resection was performed, and glioblastoma was diagnosed by pathological examination. Although SEGA is pathologically benign, glioblastoma is highly malignant, exhibits rapid growth, and is generally associated with poor prognosis. In TSC head lesions, the onset of tumors other than SEGA must also be considered, and prompt image evaluation was deemed to be necessary upon the occurrence of symptoms. Clinicians should be aware that neurological symptoms may be difficult to transmit, due to a high rate of complications of intellectual disability or autism spectrum disorder.