Katiyar Vatsala, Vohra Ishaan, Uprety Alok, Yin Wei, Gupta Shweta
Internal Medicine, John H. Stroger, Jr. Hospital of Cook County, Chicago, USA.
Internal Medicine, West China School of Medicine, Sichuan University, Sichuan, CHN.
Cureus. 2020 May 26;12(5):e8287. doi: 10.7759/cureus.8287.
Malignant granular cell tumors (MGCTs) are rare and aggressive variants of granular cell tumors. They usually involve the head and neck region, skin and soft tissues. There are no standard therapeutic guidelines for management; however, surgical resection, whenever feasible, is considered to be first line. We report a patient with recurrent unresectable MGCT of lower lip who responded to pazopanib monotherapy. This drug has been recently approved for the treatment of advanced soft tissue sarcomas. It is a potent oral tyrosine kinase inhibitor and acts on multiple receptors, including vascular endothelial growth factor receptor (VEGFR), epidermal growth factor receptor (EGFR), c-kit, platelet-derived growth factor receptor (PDGFR) and fibroblast growth factor receptor (FGFR). Due to the overexpression of multiple genes by the tumor and multiple targets of this drug, it is difficult to establish the mechanism of action responsible for disease response.
恶性颗粒细胞瘤（MGCTs）是颗粒细胞瘤的罕见且侵袭性变体。它们通常累及头颈部区域、皮肤和软组织。目前尚无标准的治疗管理指南；然而，只要可行，手术切除被认为是一线治疗方法。我们报告了一名下唇复发性不可切除MGCT患者，该患者对帕唑帕尼单药治疗有反应。这种药物最近已被批准用于治疗晚期软组织肉瘤。它是一种强效口服酪氨酸激酶抑制剂，作用于多种受体，包括血管内皮生长因子受体（VEGFR）、表皮生长因子受体（EGFR）、c-kit、血小板衍生生长因子受体（PDGFR）和成纤维细胞生长因子受体（FGFR）。由于肿瘤多个基因的过表达以及该药物的多个靶点，很难确定导致疾病缓解的作用机制。
