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1/4. Postmenopausal intravenous leiomyomatosis with high levels of estradiol and estrogen receptor.

    BACKGROUND: Intravenous leiomyomatosis is a rare variant of leiomyoma. CASE: The patient was a 49-year-old gravida 3, para 3 woman with menopause at age 46. She presented with a history of syncope. Vaginal examination revealed an enlarged and elastic-soft mass of the uterus. A pelvic ultrasound, computed tomography scan, and magnetic resonance imaging showed a heterogeneous, irregularly shaped 8- to 10-cm tumor. In addition, the inferior vena cava was almost completely occluded. Cardiac ultrasound demonstrated a mobile mass in the right atrium. The serum estradiol was 208 pg/mL (normal 0-59). Intravenous leiomyomatosis with cardiac extension was diagnosed preoperatively. A resection of the intracardiac and intracaval mass and a subtotal hysterectomy with bilateral salpingo-oophorectomy were performed. The uterine tumor weighed 600 g, and the cordlike intravascular tumor extending from the internal iliac vein into the right ventricle was 40 cm long and weighed 60 g. Pathologic examination confirmed intravenous leiomyomatosis with no evidence of atypia. The level of estrogen receptor in the tissue was 140 fmol/mg protein. The postoperative course was uneventful, and she has been in good health for 17 months after the operation. CONCLUSION: We report a case of intravenous leiomyomatosis extending into the right ventricle treated with a one-stage operation. It is possible that a high concentration of serum estradiol and high level of tissue estrogen receptor are related to the intravenous leiomyomatosis.
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keywords = leiomyomatosis
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2/4. Clinicopathological features of unusual vascular lesions of the pelvis, retroperitoneum and colon in females: a report of five cases and review of the literature.

    Vascular lesions comprise benign and malignant neoplasms as well as non-neoplastic conditions that may be located in various sites but only rarely in the pelvis or the retroperitoneum. In our study we describe five diverse and unusual cases of vascular lesions of the pelvis, retroperitoneum and colon in female patients: a case of retroperitoneal angiosarcoma, pelvic hemangioendothelioma, pelvic angiomyxoma, retroperitoneal lymphangioleiomyomatosis and a case of diffuse cavernous hemangiomatosis of the colon, with emphasis on their clinicopathological features and differential diagnosis. The recent literature on the subject is also briefly reviewed.
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keywords = leiomyomatosis
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3/4. Intravenous leiomyomatosis with cardiac extension: successful surgical management and histopathologic study.

    A case of intravenous leiomyomatosis with cardiac extension was treated successfully with a two-stage operation: resection of the intracardiac and intracaval mass for the first stage and total hysterectomy with bilateral salpingo-oophorectomy for the second. Tumors were located either within or in continuity to dilated uterine veins. A leiomyomatous protrusion of the wall of a small uterine vein was found in two remote sections. The involved veins were located alone in the layer of apparently normal uterine smooth muscles. The findings suggest that intravenous leiomyomatosis originates from the smooth-muscle component of uterine veins.
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keywords = leiomyomatosis
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4/4. The clear cell variant of epithelioid intravenous leiomyomatosis of the uterus: report of a case.

    A case of very rare uterine clear cell epithelioid leiomyomatosis is reported. The patient presented with a recurrent pelvic mass after hysterectomy 2 years earlier. The recurrent tumor was located mainly in the vessels of the broad ligament as worm-like plugs. The reviewed hysterectomy specimen showed a typical multi-lobulated intramural mass together with worm-like plugs within the myometrial vessels. Computer tomography exhibited metastatic nodules in both lungs. According to an histological examination, all the tumor cells were of epithelioid type and contained abundant clear cytoplasm. Characteristic large, thick-walled blood vessels were observed. Immunohistochemical staining and ultrastructural examination supported smooth-muscle origin in this case. Electron microscopic study revealed that the clear cytoplasm was attributed to the presence of numerous dilated mitochondria that had lost their cristae. The patient is still alive with the disease 28 months after surgery.
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keywords = leiomyomatosis
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