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SHR Neuro Cancer Cardio Lipid Metab Microb

Smolle, E; Al-Qubati, S; Stefanits, H; Haberler, C; Kleinert, R; Haybaeck, J.
Medullomyoblastoma: a case report and literature review of a rare tumor entity.
Anticancer Res. 2012; 32(11):4939-4944 (- Case Report)
Web of Science PubMed

 

Leading authors Med Uni Graz
Haybäck Johannes
Taucher Elisabeth
Co-authors Med Uni Graz
Kleinert Reinhold
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Abstract:
Medullomyoblastoma (MMB) is a very rare medulloblastoma (MB) variant consisting of primitive neuroectodermal cells intermixed with cells featuring myogenic differentiation. MMBs are a subtype of primitive neuroectodermal neoplasm (PNET) predominantly occurring in children. We describe a case of a one-year-old girl who presented with headache, emesis and ataxia. The symptoms had started seven weeks before hospital admission. Magnet resonance imaging of the brain was performed, and revealed a lesion with a maximal diameter of 5 cm, located in the cerebellum close to the vermis. Histologically, the poorly-differentiated lesion was diagnosed as a type of PNET, but it was the immunohistochemical staining that assured the diagnosis of MMB. Immunohistochemistry and interphase fluorescence in situ hybridization (I-FISH) were performed on formalin-fixed paraffin-embedded tissue. FISH did not reveal any amplification of CMYC or NMYC. No nuclear expression of β-catenin was detectable. Since MMB is a very rare tumor entity, standard treatment today is the same as that for conventional MB due to the lack of larger study series. Some authors assume that MMBs behave especially aggressive in comparison to conventional MBs. Therefore, new treatment regimes should be tested to optimize the prognosis of MMB. Further data is needed to determine the differences between MB and MMB.
Find related publications in this database (using NLM MeSH Indexing)
Cerebellar Neoplasms - genetics
Fatal Outcome -
Female -
Humans -
Immunohistochemistry -
In Situ Hybridization, Fluorescence -
Infant -
Medulloblastoma - genetics

Find related publications in this database (Keywords)
Medullomyoblastoma
central nervous system
neoplasm
treatment
medulloblastoma
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