腹膜播散性平滑肌瘤病的诊治进展

doi:10.12280/gjfckx.20230036

摘要/Abstract

摘要：

腹膜播散性平滑肌瘤病（leiomyomatosis peritonealis disseminate，LPD）是一种罕见的、特殊类型的平滑肌瘤病，表现为腹膜、盆腹腔脏器表面多发的平滑肌瘤结节。其发病机制目前尚不明确，主要有腹膜下间充质干细胞化生学说、医源性学说、性激素学说和遗传学说。LPD好发于绝经前女性，临床表现多无明显症状，常通过影像学检查或术中探查偶然发现。其明确诊断需依靠术中探查及术后病理结果，根据病史、术中平滑肌瘤播散的范围、病理等与良性转移性平滑肌瘤病、子宫静脉内平滑肌瘤病及转移性平滑肌肉瘤等鉴别。手术切除是目前首选的治疗方法，手术方式需综合考虑患者的年龄、生育要求、症状等因素，内分泌治疗可作为辅助治疗。该病虽为良性疾病，但有复发和恶变的可能，需长期严密随访。

关键词: 平滑肌瘤病, 腹膜肿瘤, 诊断, 治疗, 预后

Abstract:

Leiomyomatosis peritonealis disseminate (LPD) is a rare and specific type of smooth muscle tumor disease that presents as multiple smooth muscle myoma nodules on the surface of peritoneal, pelvic and abdominal organs. The pathogenesis of the disease is still unclear, but there are mainly theories, including subperitoneal mesenchymal stem cells theory, the iatrogenic theory, the sex hormone theory and the genetic theory. It occurs in pre-menopausal women and has an asymptomatic clinical presentation, with incidental findings on imaging or during surgery. Diagnosis depends on intraoperative exploration and postoperative pathology, and differentiation from benign metastasizing leiomyomatosis, intravenous leiomyomatosis and metastatic smooth muscle sarcoma based on history, the extent of intraoperative smooth muscle tumour spread and pathology, etc. Surgery is currently the preferred treatment method. The surgical approach needs to be considered in relation to the patient′s age, fertility requirements and symptoms, and endocrine therapy can be used as adjuvant treatment. Although the disease is benign, it has the potential for recurrence and malignancy and requires close long-term follow-up.

Key words: Leiomyomatosis, Peritoneal neoplasms, Diagnosis, Therapy, Prognosis

许阡, 袁静. 腹膜播散性平滑肌瘤病的诊治进展[J]. 国际妇产科学杂志, 2023, 50(4): 361-365.

XU Qian, YUAN Jing. Progress in Diagnosis and Treatment of Leiomyomatosis Peritonealis Disseminate[J]. Journal of International Obstetrics and Gynecology, 2023, 50(4): 361-365.

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