Desmoplastic Medulloblastoma with Atypical Presentation in an Adult Patient: A Case Report
Journal Title: Biomedical Journal of Scientific & Technical Research (BJSTR) - Year 2017, Vol 1, Issue 3
Abstract
Medulloblastomas are usually in desmoplastic type in adults. This type of medulloblastoma is generally a well-circumscribed tumor. In this report we present an adult patient with desmoplastic medulloblastoma with atypical appearance on magnetic resonance imaging mimicking cerebellar infarction, infectious or demyelinating diseases. He was followed for atypical radiological imaging with misdiagnoses of those diseases. However, histological diagnosis was desmoplastic type medulloblastoma.Medullablastoma(MB) that is the most frequently seen malignant brain tumor in childhood constitutes less than 1% of the brain tumors in adults [1], and nearly 50% of them was in desmoplastic type [2]. It has been reported that adult MB is also distinct from pediatric MB in genomic aberrations, histopathology, and prognostic outcomes [3-5]. Unlike childhood cases, adult MBs tend to be desmoplastic in histological type and to locate at cerebellar hemisphere rather than cerebellar vermis [2]. The adult desmoplastic tumors are usually well-circumscribed tumors and they can be easily dissected and resected. However, it has been reported that they may rarely be presented within atypicalinfiltrative appearance [6,7]. We reported an adult patient with an infiltrating diffuse desmoplastic MB affecting both cerebellar hemispheres and vermis.A 34-year-old male patient was admitted with complaints of severe headache and ataxia for 3 months. His clinical history revealed that he had been followed by another center because of diffuse cerebellar lesion with pre-diagnoses of cerebellar infarction, infectious or demyelinating diseases. He had been hospitalized and external ventricular drainage had been applied for a few days, but a biopsy had not been performed. Then he had been discharged for outpatient follow-up. The patient was admitted to the emergency service of our hospital because his headache was extremely increased 1 month after he was discharged.There was cerebellar ataxia in his physical examination. On magnetic resonance imaging (MRI), a diffuse lesion extending to the both cerebellar hemispheres and the vermis with mass effect and a moderate hydrocephalus were seen. The diffuse lesion had a small cystic part on the left cerebellar hemisphere. It had irregular hypointensity on T1-weighted sections and hyperintensity on T2- weighted sections. After intravenous contrast administration, a weak/insignificant heterogeneous staining was seen (Figure 1). Magnetic resonance spectroscopy (MRS) revealed increase in the choline peak and choline/creatinine ratio and extreme decrease in N acetyl aspartate (NAA) peak and NAA/ choline ratio led to diagnosis of malignancy.
Authors and Affiliations
Murat Karacan, Feyza Karagoz Guzey, Ozgur Yusuf Aktas, Azmi Tufan, Abdurrahim Tas, Burak Eren, Ilker Gulecc, Mehmet Sar
Keywords
Related Articles
- EP ID EP570633
- DOI 10.26717/BJSTR.2017.01.000277
- Views 149
- Downloads 0
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