腹膜播散性平滑肌瘤病临床分析

目的探讨腹膜播散性平滑肌瘤病(LPD)的病因、病理、临床表现和治疗。方法结合我院一例LPD,回顾分析国内外报道LPD的临床表现、组织发生、诊断方法和预后。结果LPD的发生与性激素有关,多见于育龄期妇女,缺乏特殊的临床症状与体征,其特点是肿瘤呈结节状弥漫分布于腹膜的壁层及脏层,显微镜下观察肿瘤由交错成束的平滑肌细胞组成,术前误诊率达100%。结论LPD是一种发生在腹膜的非转移性、同源性、多中心的良性平滑肌肿瘤,性激素对LPD发生有促进作用,通常在术中经病理诊断,可行全子宫切除或加上双附件切除,不需化疗,预后良好。

The Clinical Analysis of The Leiomyomatosis Peritonealis Dissemlnate

Objective To discuss the cause, pathology, clinical manifestation and treatment of Leiomyomatosis peritonealis disseminate (LPD). Methods Refer to a case of a patient with LPD in our hospital and retrospectively analyze the clinical manifestation, histogenesis, diagnostic method and prognosis of cases with LPD in international and internal reports. Results The occurrence of LPD is associated with gonadal hormone. Patients with LPD are mainly females of reproductive age. They present with non-specific clinical symptoms and physical signs. Leiomyomas distributes with widespread nodules in the parietal layer of peritoneum and visceral layer and it contains spindle cell of smooth muscle observed under microscope. Misdiagnosis rate reaches 100% preoperatively. Conclusions LPD is benign leiomyomas on peritoneum with non-metastatic and multicentric origin features. Gonadal hormone promotes the occurrence of LPD. Usually, hysterectomy or plus bilateral salpingo-oophorectony are performed during surgery after pathologic diagnosis and there is no need for chemotherapy. The disease usually regresses after removing leiomyomas.