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Lipomatous
medulloblastoma: a rare adult tumor variant with a uniquely favorable prognosis
Elshihabi S, Husain M, Linskey M
Department of Neurological Surgery, Little Rock, Arkansas 72205,
USA.
Background.
Lipomatous medulloblastoma is a rare but apparently distinct variant of
medulloblastoma.
There have been only 14 prior published cases.
We report an additional case of an adult who presented with a multicentric form
of this unique lesion.
Methods.
A 45-year-old woman underwent magnetic resonance imaging for evaluation of
progressive headaches, nausea/vomiting, vertigo, and ataxia.
Multiple bilateral mass lesions in the posterior fossa were noted, along with
obstructive hydrocephalus and significant mass effect.
Results.
Via a right suboccipital and retrosigmoid craniotomy the largest cerebellar
lesion was resected and a second lesion biopsied.
The third mass on the contralateral side was not disturbed.
Postoperatively the patient received fractionated radiotherapy to a dose of 54
Gy and chemotherapy with cisplatin, PCNU, and vincristine for 1 year.
She is alive without deficit, with a Karnofsky Performance Status of 100, and
with no evidence of disease on neuroimaging 3 years after diagnosis.
Conclusions.
This distinct variant of medulloblastoma appears to occur in adults only and has
a uniquely favorable prognosis, even with incomplete resection with institution
of appropriate adjuvant therapies.
Multicentricity does not imply a less favorable prognosis.
PMID: 14670682 [PubMed - in process]
Source: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=14670682&dopt=Abstract
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