Background: Leiomyomatosis peritonealis disseminata is a benign disease of women of reproductive age. Female gonadal steroids play the major role in pathogenesis, but reported cases in postmenopausal women suggests other factors. A few cases in the literature with coexisting endometriosis confirm the hypothesis that both originate in submesothelial multipotential mesenchymal cells. The clinical behavior can be life threatening due to extension through the pelvic veins.

Case: A 46-year-old woman with endometriosis diagnosed at laparotomy 3 years earlier presented with a huge pelvic mass. Postoperative histologic examination of the peritoneal nodules confirmed the diagnosis. Magnetic resonance angiography performed postoperatively revealed intravenous leiomyomatosis. Surgical castration was satisfactory after 2 years of follow-up.

Conclusion: Although malignant transformation of the disease is known, intravascular extension of the tumor increases the risk of mortality. Close follow-up by clinical examination and radiologic evaluations is required.

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