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Case Report
- Medullomyoblastoma with Neuronal and Rhabdomyoblastic Differentiation.
- Tae Woong Noh, Tai Seung Kim
- Korean J Pathol. 2001;35(4):357-360.
- 1,565 View
- 15 Download
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Abstract
PDF - Medullomyoblastoma is a very rare central nervous system tumor and is regarded to be a variant of medulloblastoma showing a rhabdomyoblastic component. We found 32 cases of medullomyoblastoma in English literature. We recently experienced a case of a cerebellar medullomyoblastoma with neuronal differentiation in a 15-year-old girl who displayed headaches and vomiting. The tumor displayed extensive neuronal and myoblastic differentiation on microscopic and immunohistochemical examination. On ultrastructural study, the tumor obviously demonstrated rhabdomyoblastic features showing myofilaments composed of actin and myosin with well developed Z-bands.
Original Articles
- Differentiation, Proliferative Index, and Caspase 3 Expression Rate in the Immunohistochemical Stains of Medulloblastoma as Prognostic Factors.
- Sung Eun Kim, Woo Ick Yang, Tai Seung Kim
- Korean J Pathol. 2001;35(6):536-543.
- 1,532 View
- 10 Download
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Abstract
- BACKGROUND
Medulloblastoma is a highly malignant neuroepithelial tumor of the childhood, less frequently, of adults, located in the posterior cranial fossa. It shows multiple lines of differentiation, expressing neuronal, glial, mesenchymal and ectodermal markers. The prognostic significance of cell differentiation has been studied, but received little agreement. In highly malignant tumors, very high proliferative index has been demonstrated. A major contributor to cell loss in medulloblastoma is reported to be apoptosis. In medulloblstoma, a linear relation between apoptotic index and proliferative index has not been convincingly demonstrated.
METHODS
We analyzed the immunohistochemical features, proliferative indices and apoptotic indices in medulloblastoma patients with regard to their clinical courses. Clinical features of 58 patients with medulloblastoma were reviewed. The presence of glial fibrillary acidic protein, synaptophysin, vimentin, and epithelial membrane antigen were examined with immunohistochemical method. The proliferative index (Ki-67) and caspase 3 expressing rate were calculated.
RESULTS
There was no significant correlation between the prognosis and the degree of cell differentiation. The positive correlation was noted between proliferative index and apoptotic index in a tumor mass.
CONCLUSIONS
Only proliferative index could be used as a prognostic factor.
- Vimentin and Survivin Expression Rates as Prognostic Factors in Medulloblastoma.
- Jae Yeon Seok, Se Hoon Kim, Yoon Hee Lee, Jieun Kwon, Tai Seung Kim
- Korean J Pathol. 2007;41(2):87-94.
- 1,876 View
- 27 Download
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Abstract
PDF
- BACKGROUND
A medulloblastoma is a primitive neuroepithelial tumor of the cerebellum that occurs in children and metastasizes through the cerebrospinal fluid. It is highly malignant and invasive, and the 5-year survival rate is only 60%. Surgical resection techniques, radiation, and chemotherapy have improved the overall survival but the patients suffer life-long cognitive dysfunctions or endocrine abnormalities as the side effects of treatment. Therefore it is essential to identify prognostic markers to determine the appropriate treatment strategy in order to minimize the side effects.
METHODS
This study evaluated the immunohistochemical differentiation and survival rate with synaptophysin, glial fibrillary acidic protein, epithelial membrane antigen, vimentin and primitive neuroepithelial marker nestin of 55 paraffin-embedded medulloblastomas, using a tissue microarray. The expression of survivin, the apoptotic inhibitor, and the survival rate with regard to the proliferation index of Ki-67 were also investigated.
RESULTS
The group testing positive to vimentin, a mesenchymal differentiation marker, had a worse prognosis and there was a strong correlation between vimentin expression and nestin expression. Patients with a survivin expression rate >35% had a significantly poorer clinical course and there was a correlation between the survivin expression rate and Ki-67 expression rate.
CONCLUSION
In conclusion, vimentin and survivin are negative prognostic markers in medulloblastomas.
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