恶性颗粒细胞瘤(MGCTs)罕见,是一种高度恶性肉瘤,常发生转移,生存期短。现多认为该瘤起源于Schwann细胞。恶性颗粒细胞瘤在组织学形态上与良性颗粒细胞瘤有时非常相似,因此长期以来对MGCTs的组织学诊断标准一直存在着争议,病理诊断较为困难。本文对其组织起源、临床特征及病理学特征作一综合介绍。 Malignant granular cell tumor (MGCTs) rare, is a highly malignant sarcoma, often occur metastasis, short survival. Now that the tumor originated in Schwann cells. Malignant granulosa cell tumor is histologically very similar to benign granulocytoma, so it has long been controversial for histological diagnosis of MGCTs, and pathological diagnosis is difficult. This article gives a comprehensive introduction to its origins, clinical features and pathological features.