Patt S, Oppel F, Cervós-Navarro J
Institut für Neuropathologie, Freien Universität Berlin.
Zentralbl Allg Pathol. 1989;135(5):445-55.
We report a 7 year old female presenting with a primary sarcoma of the frontal cortex, spinal cord metastases, and a cerebellar tumor. The latter was comprised of undifferentiated small cells and a component resembling rhabdomyosarcoma. On formal grounds it could be diagnosed either as medullomyoblastoma or rhabdomyosarcoma. At the ultrastructural level the tumor appeared more typical for a medullomyoblastoma but it seem highly improbable that both (rare) tumors appeared simultaneously by coincidence. Rather, we assume that we are dealing with a metastasis of the sarcoma with differentiation towards rhabdomyosarcoma. The apparent ability of a sarcoma to imitate morphological features of medullomyoblastoma in the cerebellum is further proof of the fact that nosological differentiation of both tumors remains a problem. Our findings did not corroborate the concept of medullomyoblastoma as a separate nosological entity.
我们报告了一名7岁女性,患有额叶原发性肉瘤、脊髓转移瘤和小脑肿瘤。后者由未分化的小细胞和类似于横纹肌肉瘤的成分组成。从形式上看,它可以被诊断为髓母细胞瘤或横纹肌肉瘤。在超微结构水平上,肿瘤看起来更符合髓母细胞瘤的典型特征,但两种(罕见)肿瘤同时巧合出现的可能性似乎极小。相反,我们认为我们处理的是肉瘤转移并向横纹肌肉瘤分化的情况。肉瘤在小脑中模仿髓母细胞瘤形态特征的明显能力进一步证明了这两种肿瘤的分类学区分仍然是一个问题。我们的研究结果并未证实髓母细胞瘤作为一个独立分类实体的概念。