Nishio Jun, Nakayama Shizuhide, Koga Kaori, Aoki Mikiko
Section of Orthopaedic Surgery, Department of Medicine, Fukuoka Dental College, 2-15-1 Tamura, Sawara-ku, Fukuoka 814-0193, Japan.
Department of Orthopaedic Surgery, Faculty of Medicine, Fukuoka University, 7-45-1 Nanakuma, Jonan-ku, Fukuoka 814-0180, Japan.
Cancers (Basel). 2024 May 20;16(10):1940. doi: 10.3390/cancers16101940.
Keratin-positive giant cell-rich tumor (KPGCT) is an extremely rare and recently described mesenchymal neoplasm that occurs in both soft tissue and bone, frequently found in young women. It has locally recurrent potential if incompletely excised but low risk for metastasis. KPGCT is histologically similar to conventional giant cell tumors of soft tissue but shows the presence of keratin-positive mononuclear cells. Interestingly, KPGCT also shares some morphological features with xanthogranulomatous epithelial tumors. These two tumors have recently been shown to harbor an - fusion, arguing in favor of a single entity. Surgery is the treatment of choice for localized KPGCT. Therapeutic options for advanced or metastatic disease are unknown. This review provides an overview of the current knowledge on the clinical presentation, pathogenesis, histopathology, and treatment of KPGCT. In addition, we will discuss the differential diagnosis of this emerging entity.
角蛋白阳性富含巨细胞肿瘤（KPGCT）是一种极其罕见且最近才被描述的间叶性肿瘤，可发生于软组织和骨骼，常见于年轻女性。若切除不完全，它具有局部复发的可能性，但转移风险较低。KPGCT在组织学上与传统的软组织巨细胞瘤相似，但可见角蛋白阳性的单核细胞。有趣的是，KPGCT与黄色肉芽肿性上皮肿瘤也有一些形态学特征相同。最近发现这两种肿瘤都存在一种融合现象，这支持了它们是单一实体的观点。手术是局限性KPGCT的首选治疗方法。对于晚期或转移性疾病的治疗选择尚不清楚。本综述概述了目前关于KPGCT的临床表现、发病机制、组织病理学和治疗的知识。此外，我们还将讨论这一新兴实体的鉴别诊断。
