右小腿腺泡状软组织肉瘤一例

Alveolar soft part sarcoma in the right lower extremity: a case report

腺泡状软组织肉瘤(ASPS)是一种罕见的高度恶性软组织肿瘤。该文报道了1例罕见的ASPS,患者为男性、17岁,肿瘤发生于右小腿深部软组织。镜下肿瘤呈实性生长,腺泡结构消失,被纤维组织分隔,并且有大量血窦形成,血管内可见瘤栓,胞质内可见棒状结晶体。免疫组织化学染色示转录因子3(TFE3)核阳性,SMA部分阳性,F8部分阳性,Vimentin少数散在阳性,过碘酸-雪夫染色阳性,余指标均阴性。扩大切除肿物后,患者14个月无瘤存活。该例提示,结合临床、组织学形态及免疫组织化学检测,可以对ASPS做出正确诊断,ASPS的鉴别诊断需排除血窦丰富、血管周源性或胞浆嗜伊红、透亮的肿瘤,TFE3是其诊断的特异性标志物。

Alveolar soft part sarcoma（ ASPS） is a rare highly malignant soft tissue neoplasm. In this article,we reported one 17-year old male patient diagnosed with ASPS in the deep soft tissues of his right lower extremity. Microscopically,the neoplasm was manifested with solid texture,loss of alveolar structure and separated by fibrovascular septa. A large quantity of blood sinus was observed and tumor thrombosis was identified within the blood vessels. Needle-like crystals were detected in the cytoplasm of tumor cells. Immunohistochemical staining revealed that the tumor cells were positive for TFE3 nucleus,partially positive for SMA,partially positive for F8,dispersely positive for Vimentin,positive for Periodic acid-Schiff staining and negative for alternative parameters. After enlarged tumor resection,the tumor-free survival of the patient achieved 14 months.This case prompts that combined application of clinical,histological and morphological characteristics and immunohistochemical examinations contributes to accurate diagnosis of ASPS. Abundant blood sinus and perivascular or cytoplasmic eosin-stained transparent tumors should be excluded for the differential diagnosis of ASPS.TFE3 is a specific biomarker.