Department of Urology, Faculty of Medicine, School of Health Sciences, University of Thessaly, University Hospital of Larissa, 41100 Larissa, Greece.
Department of Urology, Faculty of Medicine, School of Health Sciences, University of Thessaly, University Hospital of Larissa, 41100 Larissa, Greece; Department of Medicine, Division of Hematology and Medical Oncology, Weill Cornell Medicine, New York, NY 10065, USA.
Cancer Treat Res Commun. 2023;35:100695. doi: 10.1016/j.ctarc.2023.100695. Epub 2023 Mar 17.
Granular cell tumors (GCTs) are a rare type of mesenchymal tumors that are histologically derived by Schwann cells and rise within soft tissues such as skin and mucosal surfaces. Differentiation between benign and malignant GCTs is often difficult and relies on their biological behavior and metastatic potential. While there are no standard guidelines for management, upfront surgical resection, whenever feasible, is key as a definitive measure. Systemic therapy is often limited by poor chemosensitivity of these tumors; however, accumulating knowledge of their underlying genomic landscape has opened some opportunities for targeted approaches, for example, the vascular endothelial growth factor tyrosine kinase inhibitor pazopanib, which is already in clinical use for the treatment of many types of advanced soft tissue sarcomas.
颗粒细胞瘤（GCT）是一种罕见的间叶组织肿瘤，组织学上来源于施万细胞，发生于皮肤和黏膜表面等软组织中。良性和恶性 GCT 的鉴别往往较为困难，主要依赖于其生物学行为和转移潜能。目前尚无管理的标准指南，但只要可行，手术切除是关键的明确治疗方法。由于这些肿瘤对化疗的敏感性较差，全身治疗通常受限；然而，对其潜在基因组特征的认识不断增加，为靶向治疗提供了一些机会，例如血管内皮生长因子酪氨酸激酶抑制剂帕唑帕尼，已经用于治疗多种晚期软组织肉瘤。
