Objective: To investigate the clinicopathological features of pulmonary granular cell tumors (pGCTs) and to improve the diagnostic accuracy of the tumor. Methods: A total of 5 pGCTs were diagnosed from February 2016 to January 2022 at Shanghai Pulmonary Hospital, Tongji University School of Medicine and Fudan University Shanghai Cancer Center, China. Immunohistochemical staining, and analysis of the clinicopathological characteristics were performed. Results: The average age of the pGCTs patients was 46 years (ranging from 24 to 54 years), with 3 females and 2 males. One case occurred in the bronchus with multiple nodules in the lung, 2 cases occurred in the bronchial opening, and 2 cases were solitary nodules in the lung. The maximum diameter of the tumors ranged from 12 to 15 mm (mean size 14 mm). Microscopically, the tumor showed infiltrative growth and consisted of round, oval or polygonal cells. Abundant eosinophilic cytoplasm was noted, and the nucleoli were prominent. None of the 5 cases showed any mitosis or necrosis. Immunohistochemical and histochemical study showed positive staining for S-100 (5/5), SOX10 (5/5), Vimentin (5/5), TFE3 (4/5), PAS (3/5), and amylase-digested-PAS (3/5), while 4 cases were negative for CD68. TFE3 FISH analyses on 2 cases showed that no signal abnormality was detected in these 2 cases. The average proliferation index of Ki-67 was 2.2% (range 0-5%). There was no recurrence in 4 cases of pGCTs with a follow-up time ranging from 2 months to 60 months. Conclusions: pGCTs are very rare tumors, most likely originating from Schwann cells. Immunohistochemical staining is the conventional diagnostic tool for pGCTs diagnosis. Recognition of this entity is essential for pathologists to avoid misdiagnosis and unnecessary treatments.
目的: 探讨肺颗粒细胞瘤(granular cell tumors,GCT)的临床病理学特征,以提高对该肿瘤的认识水平。 方法: 回顾性分析2016年2月至2022年1月上海市肺科医院与复旦大学附属肿瘤医院确诊的5例肺GCT的临床资料、病理形态学特征及免疫表型特点,并复习相关文献。 结果: 5例肺GCT患者平均年龄46岁(范围24~54岁),女性3例,男性2例。其中1例发生于支气管内并肺内多发结节,2例发生于支气管开口处,2例呈肺内孤立性结节。肿瘤最大径12~15 mm,平均14 mm。镜下特征是肿瘤呈浸润性生长,由实性巢团或条索状排列的圆形或多角形细胞组成,细胞边界清楚或不清,无明显异型性,胞质呈粗颗粒状、嗜酸性,核圆形、卵圆形,核仁明显,5例病例均未见核分裂象及坏死。免疫组织化学染色显示5例S-100蛋白、SOX10、波形蛋白均阳性,其中4例TFE3阳性,4例CD68阴性表达,Ki-67阳性指数平均2.2%(范围0~5%)。特殊染色显示3例过碘酸雪夫(PAS)染色及淀粉酶消化后PAS染色均阳性。其中2例行TFE3荧光原位杂交检测,2例均未见所检测的信号异常。随访4例(2~60个月),均无复发。 结论: 肺GCT是一种罕见发生于肺的具有施万细胞分化的肿瘤,免疫组织化学染色在肿瘤的诊断与鉴别诊断中有重要价值,该肿瘤预后好,应避免误诊为其他肺原发性疾病或转移性的恶性肿瘤,防止造成不适当的治疗。.