Popivanov G, Ilcheva B, Konakchieva M, Kjossev K, Mutafchiyski V, Tabakov M
1Department of Surgery, Military Medical Academy, Sofia, Bulgaria.
2Department of Pathology, Military Medical Academy, Sofia, Bulgaria.
Georgian Med News. 2022 Nov(332):118-120.
Leiomyomatosis peritonei or disseminated peritoneal leiomyomatosis (DPL) is an extremely rare condition affecting mainly women of childbearing age with estrogen hypersecretion. It is characterized by the presence of multiple histologically benign smooth muscle nodules on the peritoneal surface and subperitoneal tissue mimicking peritoneal carcinomatosis. There are no clear guidelines for the primary management and follow-up o these patients. Case report: We represent a 44-years-old woman admitted due to two retroperitoneal tumors diagnosed on routine ultrasound screening. There was a past history of hysterectomy for uterine leiomyoma. The blood assay was normal. MRI revealed two well-circumcised tumors with a size of 15 cm and 6 cm, respectively, with a characteristic of leiomyoma. The tumors were removed completely. Macroscopically, they appeared as well-circumscribed masses with grey to white color, a rubbery texture, and a whorled cut surface. On the first postoperative day, due to a sudden drop of hemoglobin from 127 g/L to 6.8 g/L and clinical signs of acute intra-abdominal bleeding, the patient was reoperated. The site of bleeding was from the ileocolic vein located at the lower pole of the tumor. It was successfully controlled by ligation. The patient had an uneventful recovery. After five years she is free of recurrence. DPL is a rare disease, sometimes mimicking peritoneal carcinomatosis. The preoperative diagnosis should be suspected in women of childbearing age with estrogen hypersecretion and concurrent uterine leiomyomas. The management depends on the patient's age, reproductive plans, multiplicity, and symptomatology of DPL. Single tumors are best treated by surgery with synchronous hysterectomy or myomectomy in case of uterine myomatosis. In the case of symptomatic multiple DPL and fulfilled reproductive plan, bilateral salpingo-oophorectomy or treatment with Gonadotropin-releasing hormone analogs is indicated. In asymptomatic multiple DPL, the removal of all nodules is not mandatory because of the benign indolent course. The long-term prognosis of DPL is excellent.
腹膜平滑肌瘤病或播散性腹膜平滑肌瘤病(DPL)是一种极为罕见的疾病,主要影响雌激素分泌过多的育龄女性。其特征是在腹膜表面和腹膜下组织出现多个组织学上为良性的平滑肌结节,类似腹膜癌转移。目前对于这些患者的初始治疗和随访尚无明确指南。病例报告:我们报告一位44岁女性,因常规超声筛查发现两个腹膜后肿瘤入院。既往有因子宫平滑肌瘤行子宫切除术史。血液检查正常。磁共振成像(MRI)显示两个边界清晰的肿瘤,大小分别为15厘米和6厘米,具有平滑肌瘤的特征。肿瘤被完全切除。大体上,它们表现为边界清晰的肿块,颜色从灰色到白色,质地似橡胶,切面呈漩涡状。术后第一天,由于血红蛋白突然从127克/升降至6.8克/升以及急性腹腔内出血的临床症状,患者接受了再次手术。出血部位来自肿瘤下极的回结肠静脉。通过结扎成功控制了出血。患者恢复顺利。五年后未复发。DPL是一种罕见疾病,有时类似腹膜癌转移。对于雌激素分泌过多且并发子宫平滑肌瘤的育龄女性,应怀疑术前诊断。治疗方法取决于患者的年龄、生育计划、DPL的数量和症状。单个肿瘤最好通过手术治疗,如有子宫肌瘤则同步行子宫切除术或肌瘤切除术。对于有症状的多发性DPL且生育计划已完成的患者,建议行双侧输卵管卵巢切除术或用促性腺激素释放激素类似物治疗。对于无症状的多发性DPL,由于其良性惰性病程,不必切除所有结节。DPL的长期预后良好。