Fig 2

Magnetic resonance imaging of the brain from a male age 25 years shows a large meningioma in the left cerebellopontine angle measuring 3 × 6 cm causing mass effect upon the adjacent brain stem and left middle cerebellar peduncle with deformity of the fourth ventricle. Additional 1.5 cm diameter enhancing extraaxial masses were also present in both middle cranial fossae anteriorly. The patient underwent resection of left trigeminal schwannoma and left frontotemporal meningioma, the etiology of which was considered to be prior radiation therapy. He had a medulloblastoma at age 2 years, which was treated with resection, followed by radiation and cobalt treatment. He developed subsequent hydrocephalus, right exotropia with vision loss, and development delay. He developed an osteochondroma of the left 3rd rib at age 12 years, which required resection. BCCs developed at age 13 years in the distribution of the radiation treatment. He has a few palmar pits but no jaw cysts. He has a lumbar meningocele associated with marked lumbar lordosis, neurogenic bowel, and bladder. MRI of the lumbosacral spine revealed a lipoma in the posterior aspect of the sacral canal from S1-S4 and bony defects of the posterior elements of the sacral vertebrae.