Anaplastic medulloblastoma in a child with Duchenne muscular dystrophy.
A 9-year-old boy with known Duchenne type muscular dystrophy (DMD) presented with signs of increased intracranial pressure. Radiological investigations revealed a lesion in the midline of the posterior fossa. Subtotal resection was performed. Pathology findings were consistent with the diagnosis of anaplastic medulloblastoma. The postoperative lumbar CSF was positive for malignant cells. Postoperatively, the patient showed severe neurological deterioration and lost his capacity to walk. He was treated with craniospinal radiation followed by nonintensive chemotherapy. At 30 months postsurgery, he was still in complete remission but had not recovered his walking ability. This is the second report of a malignant brain tumor in a boy with DMD. The possible link between the 2 conditions is discussed, as are ethical considerations regarding the management of medulloblastoma in children with DMD.
Division of Haematology/Oncology, Hospital for Sick Children, University of Toronto, Ontario, Canada. machielvdakker@gmail.com
Machiel
M
van den Akker
Paul
P
Northcott
Michael D
MD
Taylor
William
W
Halliday
Ute
U
Bartels
Eric
E
Bouffet
D002528:Cerebellar Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology / Q000532:radiotherapy / Q000601:surgery*; D002555:Cerebrospinal Fluid; D017024:Chemotherapy, Adjuvant; D002648:Child; D006801:Humans; D019586:Intracranial Hypertension; D008297:Male; D008527:Medulloblastoma / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology / Q000532:radiotherapy / Q000601:surgery*; D020388:Muscular Dystrophy, Duchenne / Q000150:complications*; D018714:Radiotherapy, Adjuvant; D013129:Spinal Puncture; D016896:Treatment Outcome; D016138:Walking