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目的报道3例肺转移性腺样囊性癌(ACC),并总结分析此类疾病的临床病理特征。方法通过组织形态、免疫组化及组织化学染色分析其临床病理特征及免疫组化特点。结果 3例肺转移性ACC患者年龄分别为37岁、54岁和60岁,男女之比为2∶1;既往均有头颈部腺样囊性癌病史,确诊后9~14年(平均11年)出现肺内结节。胸部CT或X线检查示肺内多发结节,未见气管病变。1例经皮肺穿刺活检,2例行肺楔形切除术。镜下示3例均为筛状/管状型,未见实性区及高级别转化区域。3例均可见粉染基底膜样物质和蓝染黏液样物。3例随访3~48个月(平均25个月),1例穿刺确诊后接受化疗,23个月后死亡;2例肺楔形切除术后放疗,病灶缩小,未见其他脏器受累。免疫组化:3例CD117、bcl-2和p16均(+);SMA和p63肌上皮(+);3例基底膜样物胶原Ⅳ均(+);3例GFAP及TTF-1均(-);仅1例p53(+);Ki-67阳性指数2%~10%,平均6%。组织化学:3例AB/PAS均(+)。结论肺内原发性ACC非常罕见,诊断应首先排除转移性。筛状/管状型ACC转移到肺可以经历较长时间,发现肺内多发结节应考虑到转移癌。手术切除及术后放疗有助于治疗。
Objective To report 3 cases of lung metastatic adenoid cystic carcinoma (ACC) and summarize the clinical and pathological features of these diseases. Methods The clinicopathological features and immunohistochemical characteristics of the three groups were analyzed by histomorphology, immunohistochemistry and histochemical staining. Results The age of patients with lung metastatic ACC was 37 years old, 54 years old and 60 years old respectively, the ratio of male to female was 2:1. Previously, all patients had a history of adenoid cystic carcinoma of head and neck, 9-14 years after diagnosis (mean 11 Year) pulmonary nodules. Chest CT or X-ray examination showed multiple pulmonary nodules, no tracheal lesions. 1 case of percutaneous lung biopsy, 2 cases of pulmonary wedge resection. Microscope showed three cases were sieve / tubular type, no real area and high-grade transformation area. 3 cases were found dyed basement membrane-like substance and blue dye mucus. Three patients were followed up for 3 to 48 months (average 25 months). One patient was diagnosed with puncture and received chemotherapy after 23 months. Two patients died after wedge resection radiotherapy. The lesions were reduced and no other organs were involved. Immunohistochemistry: Three cases of CD117, bcl-2 and p16 (+); SMA and p63 myoepithelium (+); three cases of basal membrane collagen IV (+); three cases of GFAP and TTF- ); Only 1 case of p53 (+); Ki-67 positive index of 2% to 10%, an average of 6%. Histochemistry: 3 cases of AB / PAS were (+). Conclusion Pulmonary primary ACC is very rare, the diagnosis should first rule out the metastatic. Screening / tubular ACC transfer to the lungs can go through longer periods of time and multiple nodules in the lungs should be considered for metastatic cancer. Surgical resection and postoperative radiotherapy contribute to the treatment.