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颗粒细胞肿瘤是一少见软组织肿瘤,源自神经鞘细胞(Schwann细胞),首由Abrikossoff于1926年描述,迄今文献上已约报道1000例.恶性颗粒细胞肿瘤首由Ravich于1945年作了描述,可能是最为罕见的软组织肉瘤,多数在局部或远处转移时才获确诊.作者报道了1例.病人系一48岁肥胖妇女,被发现左侧后外位胸壁有一肿块,约 8×8cm大小,活动,无压痛,自肩胛骨尖端伸向腋中线,未扪及淋巴结肿大.磁共振成像显示左胸壁肌层内软组织肿块,圆形,边界清,左腋淋巴结肿大,最大者0.9×1.0×1.2cm.切除活检示恶性颗粒细胞肿瘤,CT扫描示两腋淋巴结均肿大,肺无浸润,作肿块广泛切除,左腋窝探查发现9个淋巴结有转移灶,同时做了淋巴结清扫,术后顺利,给予胸壁放射和顺铂、阿霉素化疗.术后1年随访未见复发灶.
Granular cell tumor is a rare soft tissue tumor derived from Schwann cells, first described by Abrikossoff in 1926, and has reported about 1000 cases in the literature. The malignant granulosa cell tumor was first described by Ravich in 1945. Probably the most rare soft tissue sarcoma, most diagnosed locally or distantly. The author reported 1 case. The patient was a 48-year-old obese woman and was found to have a lump on the left and right external chest wall, about 8 x 8cm in size , activity, no tenderness, since the tip of the scapula extends to the midaxillary line, no palpation and lymph node enlargement. Magnetic resonance imaging shows a soft tissue mass in the left chest wall muscular layer, round, clear boundary, left axillary lymph nodes, the largest 0.9 × 1.0 1.2cm. Excisional biopsy showed malignant granulosa cell tumors. CT scans showed enlargement of lymph nodes in both orbits. No infiltrates were found in the lungs. Extensive resection of the tumor was performed. The left axillary probe revealed metastases in 9 lymph nodes. At the same time, lymph node dissection was performed. Smoothly, chest wall radiation and cisplatin and adriamycin chemotherapy were given. No recurrences were observed after 1 year of follow-up.