Cases reported "Heart Neoplasms"

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1/50. 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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ranking = 1
keywords = leiomyomatosis
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2/50. Combined surgical and medical approach to intravenous leiomyomatosis with cardiac extension.

    Intravenous leiomyomatosis with cardiac extension is a rare entity. The case of a 49-year-old patient is described: she was operated on for intracaval intra-atrial leiomyomatosis. After an incomplete procedure (the tumour appeared not totally resectable), the patient was treated for a period of three years with a GnRH-analogue, whereafter the patient was doing clinically well and the tumour, although it regained some growth, was in a stable situation. This new strategy seems of certain importance to the surgeon, as it carries an alternative to a high-risk reoperation. To our knowledge, this is the first description of such a combined therapeutical approach.
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ranking = 0.85714285714286
keywords = leiomyomatosis
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3/50. Low-grade endometrial stromal sarcoma with intracardiac extension. Evolution of extensive smooth muscle differentiation and usefulness of immunohistochemistry for its recognition and distinction from intravenous leiomyomatosis.

    This case, a rare example of low-grade endometrial stroma sarcoma with extensive smooth muscle differentiation which extended to the inferior vena cava and cardiac chambers closely resembling intravenous leiomyomatosis grossly and microscopically, illustrates the importance of extensive sectioning and the usefulness of immunohistochemistry. Although spindle cell components arranged in interlacing bundles consistent with smooth muscle differentiation were recognizable in the primary tumor (on retrospective review), extensive smooth muscle differentiation in the recurrent tumors masked prototypical morphologic features of stromal sarcoma and only small neoplastic stromal components were preserved in limited areas, leading to initial failure to distinguish the lesion from intravenous leiomyomatosis. The immunophenotyping disclosed two distinct cell populations in the tumor: i.e. vimentin-positive and smooth muscle marker negative stromal cells, and vimentin-negative spindle-shaped desmin-positive smooth muscle cells. Our observation suggests that the predominance of a smooth muscle component in such a tumor can be misleading and does not always warrant a diagnosis of intravenous leiomyomatosis, nor does it predict a benign clinical course. This case also provides an insight into the relationship of the endometrial stroma and myometrium, and their cell of origin and the histogenesis of endometrial stromal sarcoma.
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ranking = 1
keywords = leiomyomatosis
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4/50. Successful one-stage resection of intravenous leiomyomatosis of the uterus with extension into the heart.

    Intravenous leiomyomatosis of the uterus is a rare neoplasm characterized by nodular masses of benign smooth muscles with intraluminal growth to the inferior vena cava and, in some cases, to the heart. It may cause abdominal and cardiovascular symptoms and is a serious risk of death when it reaches the tricuspid valve. Surgery is the best treatment and must be applied as soon as possible using cardiopulmonary bypass. The authors report a new case that had cardiac involvement and was successfully resected. The symptoms, imaging diagnosis, pathological and histopathological findings, tumors that mismatched the intravenous leiomyomatosis (IVL), and the use of circulatory arrest and deep hypothermia are discussed. A review of the literature is included.
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ranking = 0.85714285714286
keywords = leiomyomatosis
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5/50. GnRH agonist for intravenous leiomyomatosis with cardiac extension. A case report.

    BACKGROUND: Intravenous leiomyomatosis with cardiac extension is an extremely rare disease. CASE: We recently treated a case of intravenous leiomyomatosis with extension from the inferior vena cava into the right atrium. Three operations--exploratory laparotomy, debulking of the pelvic mass and resection of the intracardiac leiomyoma--were performed. Since cells of the resected leiomyomatosis were estrogen receptor positive, we postoperatively administered GnRH agonist (leuprorelin acetate) for six months to prevent regrowth of the residual mass in the pelvis. The residual mass began to enlarge immediately after cessation of leuprorelin acetate. The same medication was readministered, and regrowth of the residual mass was completely inhibited for 15 months, until this writing. CONCLUSION: Intravenous leiomyomatosis seems to be hormone dependent, as in the case of uterine leiomyomas. In the absence of total resection, functioning ovarian tissue may remain. Therefore, long-term treatment with GnRH agonist may be useful in preventing recurrence of this disease.
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ranking = 1.1428571428571
keywords = leiomyomatosis
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6/50. Intracardiac extension of intravenous leiomyomatosis in a pregnant woman: A case report and review of the literature.

    Intravenous leiomyomatosis is an uncommon clinical entity characterized by the growth of a benign, smooth muscle tumour within the venous system. Intracardiac extension of this tumour is rare, and approximately 35 cases have been reported in the literature. The second case of massive intracardiac extension of intravenous leiomyomatosis is reported in a pregnant patient diagnosed after the unusual presentation of seizure activity. Tumours were successfully removed from the right internal iliac vein, inferior vena cava, and right atrium and ventricle in a single-stage operation using cardiopulmonary bypass and circulatory arrest. Concomitant total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed. Nine months after diagnosis, the patient was evaluated for recurrent disease. A review of the literature from 1994 to 1998 is presented.
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ranking = 0.85714285714286
keywords = leiomyomatosis
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7/50. Intravenous leiomyomatosis with cardiac extension: tumor thrombectomy through an abdominal approach.

    Intravenous leiomyomatosis is an uncommon vascular tumor that may be seen with potentially life-threatening symptoms resulting from intracardiac extension. This tumor is frequently misdiagnosed and treated without appropriate preoperative imaging and planning, which at times leads to inadequate treatment and incomplete resections. The appropriate therapy is complete excision of the tumor. We describe a patient who was treated with a new approach involving a single-stage operation without the need for median sternotomy, cardiopulmonary bypass graft, or hypothermic arrest by resection of the tumor from the point of attachment in the abdominal portion of the inferior vena cava.
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ranking = 0.71428571428571
keywords = leiomyomatosis
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8/50. Intravenous leiomyomatosis extending into the right ventricular cavity: one-stage radical operation using cardiopulmonary bypass--a case report.

    The authors describe a 47-year-old woman with intravenous leiomyomatosis (IVL) extending into the right ventricular cavity. This rare entity is a neoplasm originating from smooth muscle of the uterus, with vermiform extensions into the inferior vena cava. The patient underwent a one-stage operation under simultaneous sternotomy and laparotomy, and radical excision of the tumor was successfully achieved with use of normothermic cardiopulmonary bypass. Although this tumor is histologically benign, it sometimes extends into the cardiac cavity and causes sudden death due to incarceration into the atrioventricular orifice. Moreover, recurrence or lung metastasis of IVL has been reported. The authors recommend a one stage-radical resection of the tumor or a two-staged operation within a short interval. In the literature, 24 surgical cases of the intravenous leiomyomatosis with intracardiac extension have been reported. The diagnosis and surgical treatment of this tumor are reviewed and discussed.
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ranking = 0.85714285714286
keywords = leiomyomatosis
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9/50. Intracardiac leiomyomatosis. Case report and literature review.

    We describe a case of intracardiac leiomyomatosis originating from the uterus, growing up in the inferior vena cava, and extending into the right ventricle. She also found to have lung metastasis. As she declined for further operative intervention, tamoxifen was given for the control of disease. This case represented an unusual rapid recurrence of intravenous leiomyomatosis which was potentially preventable. To investigate for the appropriate management, a review of the literature concerning this rare condition was made.
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ranking = 0.85714285714286
keywords = leiomyomatosis
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10/50. Late intracaval and intracardiac leiomyomatosis following hysterectomy for benign myomas treated by surgery and GnRH agonist.

    BACKGROUND: The aim of this study was to report an exceptional case of a patient presenting with intracaval and intracardiac leiomyomatosis treated by combined surgical and medical treatment. CASE: A 48-year-old presented with intracaval and intracardiac leiomyomatosis (IL) discovered 6 years following a total hysterectomy with ovarian conservation for myomas. Surgical resection of the pelvic myomas and intracaval leiomyomatosis was performed during the same surgical procedure. Given the presence of a small tumor residuum in the pelvic cavity, postoperative medical treatment based on a gonadotropin-releasing hormone (GnRH) agonist was delivered for 1 year. The patient was followed-up using clinical examination and systematic CT scan. Ten months following the end of medical treatment, she is still in good health and the pelvic residuum has stabilized. CONCLUSIONS: patients with pelvic tumor combined with IL could be treated using a one-stage surgical procedure. In cases of incomplete surgical resection, medical treatment based on GnRH agonist could be successfully delivered.
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ranking = 1
keywords = leiomyomatosis
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