Nevoid Basal cell carcinoma syndrome with medulloblastoma and meningioma--case report.
A 35-year-old man presented with a rare case of nevoid basal cell carcinoma syndrome, or Gorlin's syndrome, associated with both medulloblastoma and meningioma, manifesting as visual field constriction due to multiple parasellar tumors. He had undergone resection of a medulloblastoma at the age of 1 year 9 months, followed by adjunctive irradiation with a total dose of 40 Gy. He presented with multiple subcutaneous nodules on his face and neck. Histological examination of biopsy specimens established the diagnosis of nevoid basal cell carcinoma syndrome. Tuberculum sellae meningioma was removed through a craniotomy, and his symptoms improved. Meningioma is known to occur in the field of therapeutic irradiation, so chemotherapy may be a better option for medulloblastoma associated with nevoid basal cell carcinoma syndrome.
Fukushima
Yutaka
Y
Department of Neurosurgery, Kitasato University School of Medicine, Sagamihara, Kanagawa, Japan.
Oka
Hidehiro
H
Utsuki
Satoshi
S
Iwamoto
Kazuhisa
K
Fujii
Kiyotaka
K
D000328:Adult; D001478:Basal Cell Nevus Syndrome / Q000150:complications; D002528:Cerebellar Neoplasms / Q000532:radiotherapy / Q000601:surgery; D006801:Humans; D008297:Male; D008527:Medulloblastoma / Q000532:radiotherapy / Q000601:surgery; D008577:Meningeal Neoplasms / Q000601:surgery; D008579:Meningioma / Q000601:surgery; D016609:Neoplasms, Second Primary; D012658:Sella Turcica; D014786:Vision Disorders / Q000209:etiology; D014794:Visual Fields