肺泡软组织肉瘤（ASPS）是一种罕见的恶性肿瘤，在形态上具有独特的结节状，类器官，或嵌套生长模式，其中细胞被血管化的隔片分开。诊断是基于病理和免疫组织化学结果的组合以及下一代测序揭示的ASPSCR1-TFE3基因融合的存在。ASPS最常见于下肢的无痛肿块，如果存在转移，可能累及肺部。在这里，我们报告了一例ASPS，该病例表现出特征性的ASPSCR1-TFE3基因融合以及TFE3-ASPSCR1的相互融合，该融合出现在一名31岁女性的鼻唇沟中。采用口内方法对恶性肿瘤进行完整的手术切除，导致11个月后持续缓解。
Alveolar soft part sarcoma (ASPS) is a rare malignancy that is morphologically characterized by a distinctive nodular, organoid, or nested growth pattern in which the cells are separated by vascularized septa. The diagnosis is based on a combination of pathologic and immunohistochemical findings and the presence of an ASPSCR1-TFE3 gene fusion revealed by next-generation sequencing. ASPS most commonly occurs as a painless mass in the lower extremity, with likely involvement in the lungs if metastasis is present. Here we report a case of ASPS that exhibited the characteristic ASPSCR1-TFE3 gene fusion along with a reciprocal fusion of TFE3-ASPSCR1, which presented in the nasolabial fold of a 31-year-old female. An intraoral approach was utilized for complete surgical resection of the malignancy, resulting in continued remission after 11 months.
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