Lamarca M, Rubio P, Andrés P, Rodrigo C
Department of Gynecology, Miguel Servet University Hospital, Zaragoza, Spain.
Eur J Gynaecol Oncol. 2011;32(6):702-4.
Leiomyomatosis peritonealis disseminata (LPD) is an uncommon condition characterized by multiple nodules of smooth muscle within the peritoneal cavity. It usually occurs during reproductive age, and is especially associated to exogenous and endogenous exposure to female gonadal steroids. A limited number of cases of malignant transformation have been reported in the literature. We report a case of leiomyomatosis peritoneais disseminata with sarcomatous degeneration in a 37-year-old nulligravid patient with no exposure to exogenous estrogen or progesterone, revealed by increased abdominal perimeter. The imaging techniques showed occupation of the entire peritoneal cavity by bulky solid masses. The patient underwent a total hysterectomy with bilateral salpingo-oophorectomy and tumoral mass resection. The histopathologic diagnosis was leiomiomatosis peritonealis disseminata with leiomyosarcomatous degeneration. The patient was given systemic chemotherapy with tumoral progression, and died 24 months after the initial diagnosis.
播散性腹膜平滑肌瘤病（LPD）是一种罕见的疾病，其特征是腹腔内出现多个平滑肌结节。它通常发生在育龄期，尤其与外源性和内源性女性性腺类固醇暴露有关。文献中报道了少数恶性转化病例。我们报告一例37岁未孕患者的播散性腹膜平滑肌瘤病伴肉瘤样变性，该患者未接触外源性雌激素或孕激素，因腹围增加而被发现。影像学检查显示大量实性肿块占据整个腹腔。患者接受了全子宫切除术、双侧输卵管卵巢切除术和肿瘤肿块切除术。组织病理学诊断为播散性腹膜平滑肌瘤病伴平滑肌肉瘤样变性。患者出现肿瘤进展后接受了全身化疗，初诊后24个月死亡。
