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[播散性腹膜平滑肌瘤病与交界性病变]

[Leiomyomatosis peritonealis disseminata and borderline lesion].

作者信息

Lubczyk V, du Bois A, Fisseler-Eckhoff A

机构信息

Institut für Pathologie, Dr.-Horst-Schmidt-Kliniken, Wiesbaden.

出版信息

Pathologe. 2005 Jul;26(4):291-5. doi: 10.1007/s00292-005-0768-z.

Abstract

Leiomyomatosis peritonealis disseminata (LPD) is a rare disease, which is most often found in women in reproductive age - seldom pre- and postmenaupausal. LPD has been described in women taking oral contraceptives or during pregnancy. LPD is mostly asymptomatic and discovered during operations like section caesarea, etc. Because of the macroscopic aspect (multiple small nodules <2 cm) a metastasing tumour is suspected during operation. These nodules consist of smooth muscle fibres and show benign histologic features. An increased level of cases with similar endometriosis (10%), myoms of uterus or functional ovarian tumours have been reported. Few cases of malignant transformation have also been described. In the pathogenesis of LPD a metaplastic change of submesothelial multipotential mesenchymal cells as an abnormal response on hormonal stimulation is debated. A regression of LPD after delivery or after stopping oral contraceptives is possible. Another therapeutic option is the application of GnRH-agonists. We present a case of a 35-year old female patient with sudden abdominal pain and suspicious findings in abominal ultrasound and CT-abdomen. The intraoperative aspect shows diffuse intraabdominal nodules consisting of smooth muscle fibres and a cystic left ovary. LPD, endometriosis and a borderline lesion of the left ovary were diagnosed by intraoperative histological examination. A bilateral salpingo-oophorectomy and omentectomy were performed.

摘要

播散性腹膜平滑肌瘤病(LPD)是一种罕见疾病,多见于育龄女性,绝经前和绝经后女性很少见。LPD在服用口服避孕药的女性或孕期女性中也有报道。LPD大多无症状,常在剖宫产等手术过程中被发现。由于其宏观表现(多个小于2厘米的小结节),手术中常怀疑是转移性肿瘤。这些结节由平滑肌纤维组成,具有良性组织学特征。据报道,LPD与子宫内膜异位症(10%)、子宫肌瘤或功能性卵巢肿瘤的病例数有所增加。也有少数恶性转化的病例报道。关于LPD的发病机制,有人认为是间皮下多能间充质细胞的化生改变,是对激素刺激的异常反应。分娩后或停用口服避孕药后LPD有可能消退。另一种治疗选择是应用促性腺激素释放激素激动剂(GnRH-agonists)。我们报告一例35岁女性患者,突发腹痛,腹部超声和腹部CT检查有可疑发现。术中可见弥漫性腹腔内结节,由平滑肌纤维组成,左侧卵巢有囊肿。术中组织学检查诊断为LPD、子宫内膜异位症和左侧卵巢交界性病变。实施了双侧输卵管卵巢切除术和大网膜切除术。

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