Abstract：
Granular cell tumor, which is thought to recapitulate a Schwann cell phenotype, is a very rare neoplasm that belongs to soft tissue tumors. It can be classified as benign, atypical or malignant, based on specific histological criteria, with the majority of cases exhibiting an indolent behavior. Its biology and clinical course are poorly understood and its optimal management is yet to be defined, given the rarity of cases. Here we describe an atypical granular cell tumor in the upper middle back skin that evolved after a thirty-year indolent period. Despite complete surgical removal, the patient experienced a recurrence, both local and in the lungs, following an aggressive clinical course. Data on management of metastatic disease are extremely scarce, comprised exclusively of case reports. Therefore, we administered to the patient systemic therapy according to soft tissue sarcoma guidelines, which led to disease progression, with fatal outcome. In conclusion, recurrent and/or metastatic granular cell tumor is a rare disease that can be life-threatening, for which response to different therapies is unknown. The biologic behavior of atypical and malignant granular cell tumor is quite different from its benign counterpart, evoking soft tissue sarcomas, and its diagnosis should alert clinicians. The role of adjuvant chemotherapy and radiation therapy in this setting should be explored, to limit disease recurrence.
摘要：
颗粒细胞瘤，这被认为是一种雪旺氏细胞表型，是一种非常罕见的肿瘤，属于软组织肿瘤。它可以被归类为良性的，非典型或恶性，根据特定的组织学标准，大多数情况下表现出懒惰的行为。它的生物学和临床课程知之甚少，其最佳管理尚未确定，鉴于罕见的案件。在这里，我们描述了在30年的惰性期后发展的中上背部皮肤中的非典型颗粒细胞肿瘤。尽管手术完全切除，病人经历了复发，局部和肺部，经过积极的临床过程。转移性疾病的管理数据极其匮乏，完全由病例报告组成。因此,我们根据软组织肉瘤指南对患者进行全身治疗，导致疾病进展，致命的结果。总之,复发性和/或转移性颗粒细胞瘤是一种罕见的疾病，可能危及生命，对不同疗法的反应是未知的。非典型和恶性颗粒细胞瘤的生物学行为与良性颗粒细胞瘤的生物学行为有很大不同，引起软组织肉瘤，其诊断应提醒临床医生。应该探讨辅助化疗和放疗在这种情况下的作用，以限制疾病复发。
