Department of Pathology and Laboratory Medicine, University of California, Los Angeles, Los Angeles, CA 90095-1732, USA.
Hum Pathol. 2013 Jun;44(6):1177-83. doi: 10.1016/j.humpath.2012.11.002. Epub 2013 Jan 26.
Histiocytic sarcoma (HS) is a rare neoplasm that occurs most commonly in the intestinal tract, skin, soft tissue, and lymph node. The incidence of primary central nervous system (CNS) HS is even rarer, with a total of 6 cases reported in the literature. An etiologic link has not been identified for CNS HS, and the current case of primary CNS HS is unique in that an etiologic link to prior radiation therapy is identified, associated with complex cytogenetic abnormalities in the tumor. Although radiation-associated sarcomas can present as any number of different pathologic entities, this is the first reported case of a radiation-associated CNS HS. The pathologic and immunophenotypic characteristics of this case, with a nearly obscuring heavy inflammatory infiltrate and expression of monocytic/histiocytic markers (CD163, CD68, CD4, fascin), are characteristic of CNS HS. A discussion of the differential diagnosis and review of relevant literature are presented.
组织细胞肉瘤（HS）是一种罕见的肿瘤，最常发生于肠道、皮肤、软组织和淋巴结。原发性中枢神经系统（CNS）HS 的发病率甚至更低，文献中总共报告了 6 例。目前尚未确定 CNS HS 的病因学联系，而本例原发性 CNS HS 是独特的，因为确定了与先前放射治疗相关的病因学联系，伴有肿瘤中复杂的细胞遗传学异常。虽然放射相关肉瘤可以表现为多种不同的病理实体，但这是首例报道的放射相关 CNS HS。该病例的病理和免疫表型特征，几乎掩盖了大量炎症浸润和单核细胞/组织细胞标志物（CD163、CD68、CD4、 fascin）的表达，是 CNS HS 的特征。本文还讨论了鉴别诊断并复习了相关文献。
