Department of Pathology, Laboratory Medicine Programme, University Health Network/University of Toronto, 200 Elizabeth Street, Toronto, Ontario, Canada.
Int J Gynecol Pathol. 2010 Sep;29(5):432-7. doi: 10.1097/PGP.0b013e3181d32106.
In this report we document an unusual co-mingling of rhabdoid and osteoclast like giant cells in a leiomyosarcoma arising from the broad ligament. A 38-year old female was found to have a rapidly enlarging adnexal mass which was resected and histologically found to be a leiomyosarcoma with marked pleomorphism. Completion surgery, shortly thereafter showed spread to the ileum, which was also resected. The tumor now showed a prominent population of both osteoclast-like giant cells and rhabdoid cells. Interestingly, the immunophenotype of the tumor had also changed with complete loss of desmin, caldesmon and only focal expression of smooth muscle actin. This is the first documentation of the coexistence of rhabdoid and osteoclast-like giant cells within a gynecological tract leiomyosarcoma. This tumor was characterized by rapid clinical progression despite extensive surgery and chemotherapy. These two cells types have individually been implicated as harbingers of a poor prognosis in leiomyosarcomas. Their prominence in the recurrent tumor and the loss of muscle marker expression are in keeping with biologic progression.
在本报告中，我们记录了阔韧带来源的平滑肌肉瘤中罕见的横纹肌样细胞和破骨样巨细胞的合并存在。一名 38 岁女性被发现附件区有一个迅速增大的肿块，切除后组织学检查发现为具有显著多形性的平滑肌肉瘤。随后的根治性手术显示肿瘤已扩散至回肠，也一并切除。肿瘤现在表现出明显的破骨样巨细胞和横纹肌样细胞群体。有趣的是，肿瘤的免疫表型也发生了变化，完全丧失了结蛋白、钙调蛋白，只有平滑肌肌动蛋白的局灶性表达。这是首例在妇科生殖道平滑肌肉瘤中同时存在横纹肌样细胞和破骨样巨细胞的报道。尽管进行了广泛的手术和化疗，该肿瘤仍具有快速的临床进展。这两种细胞类型单独被认为是平滑肌肉瘤预后不良的标志。它们在复发性肿瘤中的突出表现和肌肉标志物表达的丧失与生物学进展一致。
