Wang Bin, Wang Huanhuan, Wei Jinlong, Qu Limei, Meng Lingbin, Xin Ying, Jiang Xin
Department of Radiation Oncology.
Department of Pathology, The First Hospital of Jilin University, Changchun, China.
Medicine (Baltimore). 2020 Jan;99(5):e18952. doi: 10.1097/MD.0000000000018952.
Alveolar soft part sarcoma (ASPS) is a rare malignant soft tissue neoplasm with controversial histogenesis. ASPS accounts for 0.5% to 1% of all soft tissue sarcomas. Because of its rarity, ASPS is easily misdiagnosed, increasing the risk of incorrect treatment.
A 6-year-old female patient presented with a history of a 2.0 × 2.5 × 3.0-cm mass in the deep soft tissues of her right lower extremity.
Histopathological features indicated the diagnosis of ASPS. Microscopically, a diffuse arrangement of tumor cells or pseudoalveolar architectures separated by thin and well-vascularized fibrous septa were observed. Immunohistochemical staining of the tumor cells indicated positivity for transcription factor E3, myogenic determination factor 1, and periodic acid-Schiff-diastase (PAS-D) and showed a Ki-67 proliferating index of approximately 20%.
The patient underwent enlarged resection of the tumor and was treated with radiotherapy.
During the 3-year follow-up, the patient has remained in good condition, with no symptom recurrence, distant metastatic spread, or significant toxicity during or after treatment. The patient remains under regular surveillance.
Its low incidence, lack of characteristic clinical manifestations, and atypical location often lead to ASPS misdiagnosis and subsequent incorrect treatment. Nuclear expression of transcription factor E3 is of diagnostic value for ASPS. At present, there is no consensus on the treatment for ASPS. In-depth pathological analysis is needed to better understand the characteristics of this tumor.
肺泡软组织肉瘤(ASPS)是一种罕见的恶性软组织肿瘤,其组织发生存在争议。ASPS占所有软组织肉瘤的0.5%至1%。由于其罕见性,ASPS容易被误诊,增加了治疗错误的风险。
一名6岁女性患者,右下肢深部软组织有一个2.0×2.5×3.0厘米肿块的病史。
组织病理学特征表明为ASPS。显微镜下,观察到肿瘤细胞呈弥漫性排列或由薄且血管丰富的纤维间隔分隔的假腺泡结构。肿瘤细胞的免疫组织化学染色显示转录因子E3、生肌决定因子1和高碘酸-希夫-淀粉酶(PAS-D)呈阳性,Ki-67增殖指数约为20%。
患者接受了肿瘤扩大切除术并接受了放疗。
在3年的随访中,患者状况良好,治疗期间及治疗后均无症状复发、远处转移扩散或明显毒性。患者仍在定期监测中。
ASPS的低发病率、缺乏特征性临床表现和非典型位置常导致误诊及后续治疗错误。转录因子E3的核表达对ASPS具有诊断价值。目前,对于ASPS的治疗尚无共识。需要进行深入的病理分析以更好地了解该肿瘤的特征。