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Abstract：
OBJECTIVE: This study aimed to investigate the clinicopathological features and prognostic indicators of alveolar soft part sarcoma (ASPS).
METHODS: The characteristics of 26 ASPS patients diagnosed at our hospital between January 2011 and January 2019 were retrospectively analysed.
RESULTS: The data for 12 male and 14 female patients, with a median age of 27.5 years, were assessed. The clinical symptoms mainly included painless enlarged masses in deep soft tissues. ASPS had a characteristic pathological morphology. Twenty-four patients were positive for TFE3, and TFE3 gene rearrangement was detected in 12 patients. Among the 26 patients who completed follow-up, 14 had metastasis, 1 had local recurrence, and 7 died. Kaplan-Meier survival analysis revealed that prognosis was significantly correlated with sex, tumour size and metastasis (P < 0.05). Multivariate Cox regression analysis revealed that sex and metastasis were independent prognostic risk factors for patients with ASPS (P < 0.05).
CONCLUSIONS: ASPS is a rare soft tissue sarcoma of unknown origin that occurs in young people, has a slow but metastatic course, and is associated with a poor 5-year survival rate among patients with metastasis. ASPS has character TFE3 protein and gene expression, and the diagnosis is relatively specific. The diagnosis requires comprehensive analysis of clinical history, histological morphology, and immunohistochemistry.
METHODS: The characteristics of 26 ASPS patients diagnosed at our hospital between January 2011 and January 2019 were retrospectively analysed.
RESULTS: The data for 12 male and 14 female patients, with a median age of 27.5 years, were assessed. The clinical symptoms mainly included painless enlarged masses in deep soft tissues. ASPS had a characteristic pathological morphology. Twenty-four patients were positive for TFE3, and TFE3 gene rearrangement was detected in 12 patients. Among the 26 patients who completed follow-up, 14 had metastasis, 1 had local recurrence, and 7 died. Kaplan-Meier survival analysis revealed that prognosis was significantly correlated with sex, tumour size and metastasis (P < 0.05). Multivariate Cox regression analysis revealed that sex and metastasis were independent prognostic risk factors for patients with ASPS (P < 0.05).
CONCLUSIONS: ASPS is a rare soft tissue sarcoma of unknown origin that occurs in young people, has a slow but metastatic course, and is associated with a poor 5-year survival rate among patients with metastasis. ASPS has character TFE3 protein and gene expression, and the diagnosis is relatively specific. The diagnosis requires comprehensive analysis of clinical history, histological morphology, and immunohistochemistry.
摘要：
目的：本研究旨在探讨肺泡软组织肉瘤（ASPS）的临床病理特征和预后指标。
方法：回顾性分析2011年1月至2019年1月在我院确诊的26例ASPS患者的临床特征。
结果：12名男性和14名女性患者的数据，平均年龄为27.5岁，被评估。临床症状主要为深部软组织无痛性肿大。ASPS具有特征性的病理形态。24例患者TFE3阳性，12例患者检测到TFE3基因重排。在完成随访的26例患者中，14有转移，1例局部复发,7人死亡。Kaplan-Meier生存分析显示预后与性别显著相关,肿瘤大小和转移(P<0.05)。多因素Cox回归分析显示,性别、转移是影响ASPS患者预后的独立危险因素(P<0.05)。
结论：ASPS是一种罕见的不明原因的软组织肉瘤，发生于年轻人，有一个缓慢但转移的过程，并且与转移患者的5年生存率低相关。ASPS具有性状TFE3蛋白和基因表达,诊断相对具体。诊断需要全面分析临床病史，组织学形态学,和免疫组织化学。
方法：回顾性分析2011年1月至2019年1月在我院确诊的26例ASPS患者的临床特征。
结果：12名男性和14名女性患者的数据，平均年龄为27.5岁，被评估。临床症状主要为深部软组织无痛性肿大。ASPS具有特征性的病理形态。24例患者TFE3阳性，12例患者检测到TFE3基因重排。在完成随访的26例患者中，14有转移，1例局部复发,7人死亡。Kaplan-Meier生存分析显示预后与性别显著相关,肿瘤大小和转移(P<0.05)。多因素Cox回归分析显示,性别、转移是影响ASPS患者预后的独立危险因素(P<0.05)。
结论：ASPS是一种罕见的不明原因的软组织肉瘤，发生于年轻人，有一个缓慢但转移的过程，并且与转移患者的5年生存率低相关。ASPS具有性状TFE3蛋白和基因表达,诊断相对具体。诊断需要全面分析临床病史，组织学形态学,和免疫组织化学。
