Abstract：
BACKGROUND: Alveolar soft part sarcoma (ASPS) is a rare kind of malignant soft tissue tumor with undefined differentiation, of which the incidence rate accounts for only 0.5-1.0% among all kinds of soft tissue tumors. An even rarer ASPS occurs in kidney.
METHODS: Here we reported a case of a 7-year-old girl diagnosed with nephrogenic ASPS, regarding the analyses of the incidence, clinical manifestation, pathology and genetic diagnosis, in order to deepen the recognition of the disease.
CONCLUSIONS: ASPS is very rare, and tends to occur to young patients. It is very significant to precisely diagnose ASPS at an early stage, which will be the key point for the following treatment choices and prognosis.
METHODS: Here we reported a case of a 7-year-old girl diagnosed with nephrogenic ASPS, regarding the analyses of the incidence, clinical manifestation, pathology and genetic diagnosis, in order to deepen the recognition of the disease.
CONCLUSIONS: ASPS is very rare, and tends to occur to young patients. It is very significant to precisely diagnose ASPS at an early stage, which will be the key point for the following treatment choices and prognosis.
摘要：
背景：肺泡软组织肉瘤（ASPS）是一种罕见的恶性软组织肿瘤，分化不明确，其中在各种软组织肿瘤中发病率仅占0.5-1.0%。更罕见的ASPS发生在肾脏中。
方法：这里我们报道一例7岁女孩被诊断为肾源性ASPS，关于发病率的分析，临床表现,病理学和遗传学诊断，以加深对疾病的认识。
结论：ASPS非常罕见，并且往往发生在年轻患者身上。早期准确诊断ASPS具有重要意义,这将是以下治疗选择和预后的关键点。
方法：这里我们报道一例7岁女孩被诊断为肾源性ASPS，
结论：ASPS非常罕见，
