NMC Case Report Journal
Online ISSN : 2188-4226
ISSN-L : 2188-4226
Primary Intracerebral Alveolar Soft Part Sarcoma in an 11-Year-Old Girl: Case Report and Review of the Literature
Hakan EmmezAydemir KaleSevinç ÇelikAlp Özgün BörcekGüldal YilmazMemduh KaymazÖmer UluoğluAydın Paşaoğlu
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JOURNALS FREE ACCESS FULL-TEXT HTML Advance online publication

Article ID: 2014-0009


Alveolar soft part sarcoma (ASPS), a rarely observed tumor, is a soft tissue sarcoma with an unidentified cell origin. It constitutes 0.5–1.0% of all soft tissue sarcomas. It may appear in various parts of the body, but mostly observed in the trunk and the extremities. It has a high metastasis potential. To the best of our knowledge, only three cases of primary intracranial ASPS without a demonstrable lesion elsewhere is encountered. An 11-year-old girl was operated because of fronto-parietal mass lesion by craniotomy. Pathological examination revealed ASPS and no primary focus was detected. In spite of radiotherapy and chemotherapy as an adjuvant therapy, after 45 months she had a second operation for recurrence of the tumor. Since it is possible to observe metastases in late phases, up to 30 years, the patients must be followed up for a long period. Although radiotherapy and chemotherapy followed by surgery is the most accepted treatment strategy, the prognosis is still poor.

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© 2014 The Japan Neurosurgical Society

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