Cases reported "Leiomyomatosis"

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1/9. Intracardiac leiomyomatosis: echocardiographic features.

    Intravenous leiomyomatosis is a histologically benign smooth-muscle tumor arising from either a uterine myoma or the walls of a uterine vessel with extension into veins. Echocardiographic features of two cases of intravenous leiomyomatosis with extensive spread into the right-sided cardiac chambers and pulmonary arteries are described. Both patients were middle-aged women, with prior history of hysterectomy 12 and 10 years earlier who presented with cardiac symptoms and signs. Distinctive echocardiographic features include 1) elongated mobile masses extending from the veins of the lower body, including inferior vena cava and azygos vein; 2) multiple venous attachments or metastases; and 3) filling of venous vessels and right-heart chambers. Intracardiac leiomyomatosis should be considered in a female patient presenting with an extensive mass in the right-sided cardiac chambers.
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2/9. leiomyosarcoma of the uterus with a florid intravascular component ("intravenous leiomyosarcomatosis").

    A leiomyosarcoma of the uterus in a 54-year-old woman exhibited striking involvement of large vessels of the myometrium and broad ligament on both gross and microscopic examination. The pattern of vascular involvement resembled that seen in intravenous leiomyomatosis. Imaging studies showed recurrent tumor within the inferior vena cava 3 months after hysterectomy. To our knowledge, this is the first reported such case in the literature, for which we propose the designation intravenous leiomyosarcomatosis of the uterus.
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3/9. Intravenous leiomyomatosis of the uterus.

    BACKGROUND: Intravenous leiomyomatosis is a rare uterine tumor defined as an intraluminal growth of benign smooth muscle cells in either venous or lymphatic vessels outside the confines of, or even in absence of leiomyomas. We report a case of intravenous leiomyomatosis of the uterus managed at our institution. CASE REPORT: The patient, a 47-year-old woman; gravida 2 para 2, presented without complaints for a routine gynecological examination. An irregular-shaped enlarged uterus with a nodular pelvic mass extending into the broad ligament was found laparotomy revealed a large retroperitoneal, moderately firm tumor which demonstrated fingerlike projections into the pelvic veins. Due to great blood loss, surgery had to be discontinued with substantial residual disease and our patient was set on a regimen of the GnRH analogue leuprolide, which was applied over 5 months. This led to a reduction of the tumor which facilitated successful surgery. The primary tumor as well as the residual disease were classified as a leiomyoma with intravenous extension consisting of uniform, spindle-shaped smooth muscle cells in a whorled arrangement. Mitotic activity was low and pleomorphism was missing. Areas with degenerative changes were found, as well as prominent vascularity. At the right parametrium, the lumina of the dilated veins were filled with tumor. Immunhistochemically, the intravascular parts of the tumor revealed a positive staining reaction for vimentin, desmin and alpha-smooth muscle actin. The tumor also reacted for antibodies against estrogen- and progesterone-receptors. CONCLUSION: knowledge about this rare uterine tumor is important for adequate treatment and exact differential diagnosis. Though intravenous leiomyomatosis imitates a malignant neoplasm regarding its pattern of growth and extension, it must be differentiated histologically from malignant tumors to prevent overtreatment. Since intravenous leiomyomatosis demonstrates a tendency to recur, long-term follow-up of the patient is recommended.
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4/9. Intravenous leiomyomatosis with atypical histologic features: a case report.

    Intravenous leiomyomatosis (IVL) is a rare smooth muscle tumor. We report a case of IVL with atypical histologic features, which did not respond to gonadotropin-releasing hormone agonists and could be only partially resected due to adherence to the vessel wall. Atypical histology may signify more aggressive behavior. IVL should always be considered when a patient presents with both uterine leiomyoma and venous thrombosis, and a high index of suspicion is crucial for early diagnosis. An adequate surgical preparation including venous graft or prosthetic reconstruction is essential as difficulty in removal may arise if the intravascular tumor adheres to the vessel wall.
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5/9. Value of magnetic resonance imaging in the depiction of intravenous leiomyomatosis extending to the heart.

    Intravenous leiomyomatosis is a seldom neoplasia characterized by invasion of venous channels by a benign smooth muscle tumor originating either from a uterine myoma or from vessel wall. Extension to the heart may cause mechanical obstruction and is frequently misdiagnosed as a right-atrial myxoma. We present a case of recurrent intravenous leiomyomatosis with previous hysterectomy because of uterine leiomyoma which have different magnetic resonance characteristics than that of the former reports.
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6/9. Successful one-stage radical removal of intravenous leiomyomatosis extending to the right ventricle.

    BACKGROUND: Intravenous leiomyomatosis is a rare tumor originating from the uterus that spreads through vessels. This tumor, while histologically benign, can cause fatal cardiovascular symptoms resulting from growth within the heart cavity. CASE: A 51-year-old woman with chronic hypertension was found to have a murmur. Echocardiogram, magnetic resonance imaging, and clinical evaluation revealed an intravenous leiomyomatosis extending to the right ventricle. The appropriate therapy for intravenous leiomyomatosis is complete resection. In this patient, one-stage cardiotomy with laparotomy resulted in successful radical removal of the tumor. CONCLUSION: Our patient underwent successful radical therapy for an intravenous leiomyomatosis extending to the heart, which involved complete resection of the tumor by one-stage cardiotomy with laparotomy.
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7/9. CD10 in diffuse uterine leiomyomatosis: a case report along with a few comments on histogenesis.

    Diffuse uterine leiomyomatosis (DUL) is a rare entity with an unknown etiopathogenesis. A 24 years old female presented with abdominal discomfort and menorrhagia. Clinical and ultrasonographic examination revealed an enlarged uterus. The hysterectomy specimen showed a symmetrically enlarged uterus with a bosselated external surface. The cut surface showed multiple nodules of varying sizes diffusely involving the myometrium. Microscopically, the nodules were leiomyomas of varying degrees of cellularity. Some of the leiomyomas showed an increased vascularity either in the form of congeries of blood vessels with a lobular arrangement or occasionally as foci of 2-3 vessels. The vessels were surrounded by whorls of spindle cells. On immunohistochemistry the leiomyomas expressed vimentin, smooth muscle actin (SMA), desmin and CD10: the cells whorling around the blood vessels expressed vimentin, SMA and focally desmin and were negative for CD10 and HMB-45. The aim of this paper is to document that CD10 is expressed in diffuse uterine leiomyomatosis and discuss the histogenesis of DUL.
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8/9. Familial multiple cutaneous leiomyomas.

    BACKGROUND: Leiomyomas are rare, benign tumors that originate from the arrector muscle of hair follicles (cutaneous leiomyoma, piloleiomyoma), the tunica dartos of the scrotum and the mammillary muscle of the nipple (genital leiomyoma), and the smooth muscle of blood vessels (angioleiomyoma). OBSERVATIONS: We describe 6 members of a single family with mostly painful papulonodular cutaneous lesions on the limbs, trunk and face. Histological examination of sections stained with hematoxylin-eosin and Masson trichromic stain confirmed the diagnosis of leiomyoma. All affected women had also uterine myoma. CONCLUSION: Our observations support the concept that this entity is an autosomally inherited disorder.
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9/9. 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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