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 CASE REPORT
Year : 2012  |  Volume : 7  |  Issue : 1  |  Page : 43-46

Metachronous occurrence of nonradiation-induced brain cavernous hemangioma and medulloblastoma in a child with neurofibromatosis type I phenotype


1 Department of Surgery and Anatomy Division of Pediatric Neurosurgery, Ribeirão Preto School of Medicine, University of São Paulo, Ribeirão Preto, São Paulo; PhD. Scholarship Holder CNPq, Brazil
2 Department of Surgery and Anatomy Division of Pediatric Neurosurgery, Ribeirão Preto School of Medicine, University of São Paulo, Ribeirão Preto, São Paulo, Brazil
3 Department of Pediatrics, Division of Pediatric Oncology, Ribeirão Preto School of Medicine, University of São Paulo, Ribeirão Preto, São Paulo, Brazil

Correspondence Address:
Luciano L Furlanetti
Collaborator Neurosurgeon of the Division of Pediatric Neurosurgery of the Department of Surgery and Anatomy, Ribeirão Preto School of Medicine, University of São Paulo. Doctoral fellowship at the University Hospital Freiburg, Division of Stereotactic and Functional Neurosurgery, Freiburg, Germany, Breisacherstrasse, 64, 79106, Freiburg, Germany

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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1817-1745.97623

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Cavernous hemangioma (CH) is a sporadic vascular malformation occurring either as an autosomal dominant condition or as a well-known complication of radiation exposure. Medulloblastoma is a primitive neuroectodermal tumor common in children and currently treated with surgical resection, chemotherapy, and radiotherapy. Neurofibromatosis is the most common single-gene disorder of the central nervous system. Posterior fossa malignant tumors in the context of neurofibromatosis type I (NF1) are very infrequent. This is the first documented case of an unusual metachronous occurrence of non-radiation-induced CH and medulloblastoma in a child with NF1 phenotype. We report the case of a 13-month-old boy with cafι-au-lait skin lesions associated with NF1-like phenotype who underwent surgical resection of a single CH in the temporal lobe due to recurrent seizures. Four years later he presented with signs of raised intracranial pressure associated with a posterior fossa tumor and hydrocephalus, thus requiring gross total resection of the lesion. Histological analysis revealed a medulloblastoma. After being treated with radiotherapy and chemotherapy, he achieved total remission. Six years later a massive recurrence of the tumor was observed and the child eventually died. The interest in this case lies in the rarity of NF1-like phenotype associated with a non-radiation-induced brain CH and medulloblastoma in a child.






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