Leiomyosarcoma (LMS) with osteoclast-like giant cells (OLGCs) is a rare entity with only 18 reported cases thus far. It is not known whether these OLGCs are a reactive or malignant component of LMS. Herein we describe the clinical, histologic, and molecular characteristics of 2 cases of LMS with OLGCs and perform a brief literature review. In 2 of our cases, the OLGCs, marked with CD68, had a low proliferation index with Ki67 and did not show diffuse positivity for smooth muscle markers by immunohistochemistry. By next-generation sequencing, one case harbored a clinically significant TP53 mutation, which has been reported in a significant subset of conventional LMSs. In this case, based on immunohistochemistry, OLGCs showed different molecular alterations as compared with LMS. Although we did not show a distinct immunophenotype or molecular profile for LMS with OLGCs, this study provides additional data on this rare entity.

中文翻译：

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子宫平滑肌肉瘤伴破骨细胞样巨细胞2例报告并文献复习。

伴有破骨细胞样巨细胞 (OLGC) 的平滑肌肉瘤 (LMS) 是一种罕见的实体瘤，迄今为止仅报告了 18 例病例。目前尚不清楚这些 OLGC 是否是 LMS 的反应性或恶性成分。在此，我们描述了 2 例伴有 OLGC 的 LMS 的临床、组织学和分子特征，并进行了简短的文献回顾。在我们的 2 个病例中，用 CD68 标记的 OLGC 的 Ki67 增殖指数较低，并且免疫组织化学未显示平滑肌标记物的弥漫性阳性。通过下一代测序，一个病例存在临床上显着的 TP53 突变，这种突变已在传统 LMS 的一个重要子集中报告过。在这种情况下，基于免疫组织化学，OLGC 与 LMS 相比显示出不同的分子改变。