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转移至中枢神经系统的高级别恶性皮肤浆细胞瘤。一例伴有电子显微镜、免疫组织学及神经病理学研究的病例报告。

High-grade malignant cutaneous plasmacytoma metastatic to the central nervous system. A case report with electron microscopy, immunohistological, and neuropathological studies.

作者信息

Prost C, Reyes F, Wechsler J, Gaston A, Richard I, Poirier J

出版信息

Am J Dermatopathol. 1987 Feb;9(1):30-6. doi: 10.1097/00000372-198702000-00007.

Abstract

A case of high-grade malignant extramedullary plasmacytoma (EMP), unusual in its initial cutaneous localization and major involvement in the central nervous system rapidly resulting in death, is reported. Light- and electron-microscopic studies of a skin nodule revealed a high proportion of immature and mature plasmacytes. Immunohistological study revealed that both cells synthesized different amounts of a monotypic cytoplasmic kappa light chain. Neuropathological study revealed extensive infiltration of the leptomeninges and perivascular spaces as far as the deep parenchyma. In several places, effraction of vessels resulted in parenchymatous, tumor-like, plasmacytic infiltration. The findings of this case were compared with those of the few previously reported cases of EMP with initial cutaneous or intracranial localization, and with those of immunoblastic sarcoma occurring in some cases of myeloma.

摘要

本文报告了一例高级别恶性髓外浆细胞瘤(EMP),其初始表现为皮肤局部病变,且主要累及中枢神经系统,病情迅速发展导致患者死亡,这种情况较为罕见。对一个皮肤结节进行光镜和电镜研究发现,其中存在高比例的未成熟和成熟浆细胞。免疫组织学研究表明,这两种细胞均合成了不同量的单型细胞质κ轻链。神经病理学研究显示,软脑膜和血管周围间隙广泛浸润,直至深部实质。在几个部位,血管破裂导致了实质性、肿瘤样的浆细胞浸润。将该病例的研究结果与之前少数报告的初始表现为皮肤或颅内病变的EMP病例,以及某些骨髓瘤病例中出现的免疫母细胞肉瘤的研究结果进行了比较。

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