DISSEMINATED PERITONEAL LEIOMYOMATOSIS - A RARE ENTITY, COMPLICATED BY LATE BLEEDING FROM THE ILEOCOLIC VEIN

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.