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[A clinicopathological analysis of primitive neuroectodermal tumors of the CNS]
1Department of Pathology, Affiliated Hospital, Shandong Medical University, Jinan 250012.
Objective:
To investigate the difference between cerebellar medulloblastoma (MB) and primary cerebral small cell tumor in histogenesis, morphologic features and biological behavior.
Methods:
210 cases of MB and 9 cases of small cell tumor of cerebrum were observed with histologic and immunohistochemical techniques.
Results:
Both tumors were composed of primitive cells with focal evidence of glial and/or neuronal differentiation. In 63.2% of MB and five-eighths of small cell tumor of cerebrum coexpressed GFAP and Syn. Both types of tumors were highly malignant. The overall 1 year survival rates were 34.63% and 25.65% respectively. The survival rate was lower for patients with high proliferative index, with necrosis or without receiving radiation therapy.
Conclusion:
MB is similar to other small cell tumors of CNS in morphological features, specific marker expression and biological behavior. These tumors can be classified as primitive neuro-ectodermal tumors (PNET).