Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Following resection of a leiomyosarcoma, the superior vena cava (SVC) was successfully replaced by means of a spiral graft constructed from autogenous saphenous vein. This technique has previously been applied only as a bypass operation in patients with total occlusion of the SVC causing SVC syndrome. Our patient had mild SVC obstruction and absence of venous hypertension in the upper body before the operation in which segmental SVC replacement was employed. She has remained free from signs or symptoms of SVC obstruction, and graft patency was demonstrated ten months after operation. The literature concerning use of the spiral graft is reviewed, and our operative technique is described. Use of a spiral vein graft should be considered whenever large veins must be replaced.
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PMID:Segmental replacement of superior vena cava with spiral vein graft. 686 30

A 27-year-old woman with an unusual combination of three independent tumors, gastric epithelioid leiomyosarcoma, retroperitoneal paraganglioma and pulmonary chondroma is reported. There are 15 similar cases collected by Carney (1979) from the literature; ours is the first Japanese case. Patients with this tumor triad tend to be young females, all were of blood group A. The prognosis of these patients is generally good without associated systemic hypertension. Our patient is well 18 months after surgical removal of the three tumors.
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PMID:[Case of three peculiar tumors of gastric epithelioid leiomyosarcoma, retroperitoneal paraganglioma and pulmonary chondroma]. 687 95

Leiomyosarcoma of the inferior vena cava is a rare disease with protean manifestations related to the location of the tumor. Urological manifestations of the disease are rare and include renal vein thrombosis and renovascular hypertension. Approximately 63 cases have been reported in the literature, with a striking female predominance. Presentation as an isolated suprarenal mass has not been reported previously. We discuss this unusual tumor and report a case that presented as an asymptomatic suprarenal mass.
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PMID:Leiomyosarcoma of the inferior vena cava presenting as a suprarenal mass. 688 12

Primary, left-sided cardiac tumors are a rare cause of unexplained pulmonary hypertension. We describe herein two patients with leiomyosarcoma of the left atrium, who were initially seen with symptoms suggestive of primary pulmonary hypertension, venoocclusive disease, or multiple, small thromboemboli. Postmortem examination showed extension of the leiomyosarcoma into the pulmonary veins, which resulted in pulmonary venous hypertension. Although unusual, the occurrence of left-sided cardiac neoplasms should be included in the differential diagnosis of patients who are initially seen with unexplained pulmonary hypertension. An open lung biopsy should be considered and may indicate a venous origin for the hypertension.
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PMID:Left atrial leiomyosarcoma: manifestation as unexplained pulmonary vascular disease. 712 74

We report a case of renin-producing leiomyosarcoma associated with the hyponatremic hypertensive syndrome and nephrotic-range proteinuria. Extremely high levels of active renin and, to a greater extent, of prorenin were found in plasma and tumor tissue. Immunohistochemical and in situ hybridization studies demonstrated that the neoplastic cells were the source of renin production. The hyponatremic hypertensive syndrome and proteinuria promptly responded to treatment with angiotensin-converting enzyme inhibitors, suggesting an angiotensin II dependency of these disorders. After removal of the leiomyosarcoma, plasma concentration of active renin, but not of prorenin, normalized and the hypertension, proteinuria, and electrolyte abnormalities disappeared. However, 5 months after operation, the patient presented once again with hypertension, hypokalemia, proteinuria, and markedly increased plasma levels of both active renin and prorenin that heralded the relapse of neoplastic disease.
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PMID:Hyponatremic hypertensive syndrome and massive proteinuria in a patient with renin-producing leiomyosarcoma. 802 29

Leiomyosarcoma are malignant tumors showing smooth muscle differentiation, they are rare, representing only about 25 percent of all uterine sarcomas and mixed malignant tumors and slightly more than 1 percent of all corporeal malignant tumors. Usually they arise in postmenopausal women and are not known to be related to the known risk factors for endometrial carcinoma (nulliparity, obesity, diabetes mellitus, hypertension, etc.) or carcinosarcoma (prior radiation therapy). They may occur in uteri that also bear typical benign leiomyomas, but leiomyosarcoma rarely can be proven to have arisen in or from benign leiomyomas. We report on two premenopausal women with leiomyosarcoma--out of totally 31 cases in our clinic from 1975-1995 who got pregnant after surgical treatment preserving the uterus. The patients are alive without evidence of disease 3 and 6 years after surgical resection.
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PMID:[Uterine leiomyosarcoma with subsequent pregnancy]. 970 57

This report encourages clinicians to consider a diagnosis of Carney's triad in patients with multifocal gastric stromal sarcoma, extraadrenal paraganglioma (predominantly mediastinal), or pulmonary chondroma. The authors conducted a retrospective 20-year survey at the Hospital for Sick Children and identified two children with Carney's triad. One child, presenting atypically with papilledema and fundal hemorrhages from malignant hypertension and benign intracranial hypertension from chronic iron-deficiency anemia, is the second patient ever to date be described with the complete Carney's triad of neoplasms at diagnosis. Another child presented more typically with gastric stromal sarcoma and pulmonary chondroma without paraganglioma. Carney's triad is a rare differential diagnosis for "idiopathic" hypertension or iron-deficiency anemia from chronic gastrointestinal bleeding. If missed, patients with Carney's triad may have the debilitating physical and mental consequences of chronic iron deficiency and may die of untreated prolonged hypertension and metastatic leiomyosarcoma.
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PMID:Typical and atypical Carney's triad presenting with malignant hypertension and papilledema. 1187 80

The objectives of this study were to describe five cases involving primary tumors of the thoracoabdominal aorta and to review the pertinent literature. Between April 1990 and April 2000, we performed surgery on five patients with primary tumors of the aorta (PTA). There were three men and two women ranging in age from 37 to 65 years (mean, 49.8 years). The presenting manifestations were renovascular hypertension in four cases, including three associated with abdominal angina and lower extremity embolism in one case. In all patients aortograms identified atherosclerotic-like occlusive lesions in the thoracoabdominal aorta extending to the descending thoracic aorta in three cases, visceral arteries in four cases, and infrarenal aorta in one case. Preoperative histological diagnosis of PTA was achieved in two patients following open repair with placement of an aortoaortic graft in one case and peripheral embolectomy in one case. In two cases, diagnosis of PTA was strongly suspected before or during the procedure. In the remaining case, diagnosis was not achieved until the definitive histological report. In two patients surgical treatment was carried out with curative intent (aortic resection with graft replacement). In two cases surgical treatment was incomplete (endarterectomy of the aorta and visceral arteries). In the remaining case surgical treatment was purely palliative (aortic and superior mesenteric artery bypass). Histological findings demonstrated intimal-type sarcoma in two cases, leiomyosarcoma in one case, and angiosarcoma in one case. In the remaining case, histological analysis was unfeasible for technical reasons. One patient died due to massive cerebral embolism 2 days after surgical treatment involving revascularization of the aortic arch carried out with hypothermic circulatory arrest. One patient developed secondary paraplegia. All four patients who survived the immediate postoperative period died of tumor-related complications and cachexia at 5, 7, 16, and 24 months after the initial surgical procedure. The results of this small series as well as those of 130 previously reported cases confirm the extremely dismal prognosis of PTA. Mean overall survival for patients presenting PTA was less than 16 months. Survival at 5 years was 8%. Survival rates appear to be higher after surgical treatment and were significantly improved by adjuvant chemotherapy. The main factors correlated with poor prognosis were intimal type, involvement of the ascending aorta, aortic arch, or visceral aorta, and incomplete resection.
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PMID:Primary tumors of the thoracoabdominal aorta: surgical treatment of 5 patients and review of the literature. 1467 13

Adrenal myelolipoma is a rare, benign and biochemically inactive tumor. It is usually diagnosed incidentally by radiological methods and is known to be associated with obesity, hypertension, endocrinological disorders and some malignancies. We report herein the association of a myelolipoma with a gastrointestinal stromal tumor. To our knowledge this is the first report of such an association to date. A 67-year-old male patient admitted to our clinic with abdomimal pain and fever; he had a history of hypertension and diabetes mellitus. In physical examination, a mass involving the right quadrants was palpated. Computerized tomography revealed a right retroperitoneal mass, probably originating from the kidney or cecum. In laparotomy, the tumor (12 cm radius and 1500 g) localized on the superior of right kidney was excised. Abdomen exploration revealed another mass with 10 cm radius 100 cm distal to the ligamentum of Treitz and segmental jejunal resection and anastomosis were applied. The pathological diagnosis was reported as myelolipoma for the retroperitoneal mass and leiomyosarcoma for the jejunal mass. Myelolipoma is a benign tumor, involving mature fat and hematopoietic stem cells. Pathogenesis is still not clear and the microscopical characteristics are hematopoietic, lipoid, and reticuloid cells and megakaryocytes. Myelolipomas are reported to be associated with some other malignancies (especially renal), but this is the first report showing the association with a leiomyosarcoma. Therefore, leiomyosarcoma should also be one of the possible associations kept in mind by the physician in the diagnosis and treatment of myelolipomas.
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PMID:Giant adrenal myelolipoma associated with small bowel leiomyosarcoma: a case report. 1683 Feb 97

We describe the case of a 44-year-old woman who presented with renovascular hypertension caused by primary leiomyosarcoma of the abdominal aorta that had metastasized into the renal arteries. Despite an extensive radiological evaluation, the diagnosis was mistaken first for Takayasu's arteritis and then for retroperitoneal hematoma or neoplasm. The patient developed renal failure due to bilateral renal infarction, and died 3 months after her initial presentation with ischemic colitis. Postmortem examination confirmed the diagnosis.
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PMID:Leiomyosarcoma of the abdominal aorta: a rare cause of renovascular hypertension. 1751 May 10


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