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Query: UMLS:C0020538 (hypertension)
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The lack of uniformity in the nomenclature of the uterine sarcomas, it have contributed to a variety and variability of classifications. Fortunately the sarcomas of uterus are rare. The incidence of this tumor is of 3-5% of all the uterine cancers or of 1.7/100,000 women of 20 years or more. The clinical presentation of these tumors is diverse could come bleed uterine abnormal, abdominal pain, pelvic mass, discharge or cervix prominent mass. Clinical discoveries associated with exist the sarcomas how they are the obesity and high blood pressure in a 30% of the patients it are also observed antecedents of pelvic radiation in a 5-10% of the cases. The genomic alterations that is reported the chromosomes in the literature is associated with 1, 7 11 playing an important paper in the initiation or progression of the sarcomas. We was carried out a retrospective analysis of 37 cases of uterine sarcoma managed in the National Institute of Cancerology at one time of 5 years. Being that the leiomiosarcomas comes in the 51.3% of the cases, followed by the stromal sarcoma, bleed uterine abnormal it was the clinical fact of high importance, detecting these patients in Ia and IIa stadiums predominantly. We observed an increment in the incidence of the uterine sarcoma in patients of 40 years or more. 17 patients were managed exclusively with surgery, 17 patients with surgery and radiotherapy and 5 patients with surgery and chemotherapy (2 patients were managed with surgery + radiotherapy + chemotherapy). The index of failure was from the 45.1% to two years in general form, coming metastasic illness in lung, liver and breast mainly. In conclusion, the adjuvant radiotherapy and chemotherapy to the hysterectomy doesn't increase the index of survivor in the several subtype of uterine sarcomas.
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PMID:[Uterine sarcoma. Analytic study of 37 cases]. 961 18

Two cases of calvarium sarcoma with intracranial extension, consecutive to Paget disease of the skull are presented. The neurosurgical implications of this rare complication of a common disease are reviewed. In both cases, the symptoms of malignant degeneration were unspecific and blurred by the presence of a well-known Paget disease. Increasing headaches, focal neurological deficits and signs of intracranial hypertension were observed. Skull X-rays and Computerised Tomography did not allow to make the difference between Pagetic bone and sarcoma. In the second case, Magnetic Resonance Imaging was the most accurate in determining the precise localisation of the tumour, and the subdural invasion. Extensive surgical resection was carried out, by craniectomy of the tumoural bone, followed by cranioplasty. Dural sinus involvement and tumour hypervascularisation caused important peroperative blood loss in both patients. In the second case, the tumour invaded the subdural space through a Pacchioni granulation. Survival of patients with Pagetic sarcoma seems shorter when compared to post-radiation or primitive osteosarcoma, despite adjuvant therapies, probably because of late diagnosis and incomplete surgical resection. The mean reported survival rate is 6 months. Although the first patient died within 4 months, in the second case, the authors obtained a more than two year survival with aggressive surgery alone. These cases also illustrate the polymorphism of sarcomatous degeneration of Pagetic calvarium, the interest of MRI, and the need for close surveillance of patients with known Pagets disease of the skull.
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PMID:Pagetic sarcoma of the calvarium: report of two cases. 992 24

The kidney is a rare site of hydatid disease. It remains clinically silent for a long time and only presents at the stage of complications. The diagnosis is essentially radiological. Renal hydatid cyst (RHC) raises therapeutic problems due to its complications, which sometimes make kidney-preserving cyst resection surgery difficult. The authors report a series of 45 cases of RHC treated in their department during a 20-year period. This series consisted of 27 men and 18 women with a mean age of 47 years (26-80 years). The clinical features were dominated by pain (80%), flank mass (42%), hydaturia (22%), haematuria (13%), urinary tract infection (6%), hypertension (3%). IVU, performed in all patients, showed calcifications in 35% of cases, renal tumour syndrome in 71% of cases and silent kidney in 22% of cases. Surgery was usually performed via a lumbar incision (75%) or a BARAYA incision (15%). Treatment consisted of 18 cyst roof resections (40%), 6 pericystectomies (13%), partial nephrectomy in 2 cases (4%) and total nephrectomy in 18 cases (40%). The postoperative course was marked by urinary fistula in two patients and one death at the 2nd postoperative month related to associated renal sarcoma. The objective of this study is to analyse the epidemiological, pathological and therapeutic features of this disease, which still constitutes a public health problem in Morocco.
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PMID:[Hydatid cyst of the kidney. Report of 45 cases]. 1009 9

Elevated tumor interstitial fluid pressure (IFP) is partly responsible for the poor penetration and distribution of therapeutic agents in solid tumors. The etiology of tumor interstitial hypertension is poorly understood. We have postulated that the solid stress generated by tumor cells growing in a confined space compresses blood vessels and increases tumor microvascular pressure and IFP. To test the hypothesis that neoplastic cell loss would decompress blood vessels and lower IFP, we induced apoptosis in tumors with paclitaxel and docetaxel. Taxanes inhibited the growth of the murine mammary carcinoma (MCa-IV) and of the human soft tissue sarcoma (HSTS-26T). Taxanes induced apoptosis and reduced the density of intact neoplastic cells in both MCa-IV and HSTS-26T. To determine whether neoplastic cell loss decompressed blood vessels, we measured the diameter of tumor vessels in HSTS-26T tumors implanted in transparent dorsal skin fold chambers. At 48 and 96 h after paclitaxel, the diameter of tumor vessels was significantly increased. The increase in vascular diameters was associated with reductions in microvascular pressure and IFP. The changes in neoplastic cell density and IFP were also correlated. In the human glioblastoma U87, which is resistant to paclitaxel, the IFP and cellular density were not modified by paclitaxel treatment. Collectively, these results support the hypothesis that solid stress generated by neoplastic cell proliferation increases vascular resistance and IFP. The increase in vessel diameter induced by paclitaxel and docetaxel suggests that taxanes could improve tumor response by increasing the vascular surface area for delivery of therapeutic agents.
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PMID:Taxane-induced apoptosis decompresses blood vessels and lowers interstitial fluid pressure in solid tumors: clinical implications. 1044 95

An 82-year-old woman was seen at our Dermatology Department for a plaque on the right parietal scalp that had recently increased in size, and bled. The lesion had been present for 3 months. The patient had a previous diagnosis of chronic bronchitis, noninsulin-dependent diabetes mellitus, and hypertension, but no previous history of cancer. Physical examination revealed a 7 x 10 cm plaque, composed of a central necrotic and bleeding surface, surrounded by small purple-red satellite nodules. A biopsy showed an ill-defined infiltrative intradermal mass with a pattern of hypercellular sheets of large cells alternating with areas of dilated, irregular, blood-filled channels, dissecting the collagen bundles. The endothelial cells lining these channels were plump and pleomorphic, surrounded by other spindle-shaped cells with pleomorphic and atypical nuclei. The diagnosis of angiosarcoma was made, and the patient was sent to an oncology center for further evaluation and treatment, where a computed tomography head scan was taken revealing no erosion of the skull. The patient refused surgery, so radiotherapy was proposed. One month later, she developed lymph node enlargement of the left anterior cervical nodes. A needle aspiration biopsy was consistent with sarcoma. Two weeks later, she was started on palliative radiotherapy: a programmed dose of 4500 cGy was proposed of which she only received 3000 cGy because of treatment withdrawal and loss to follow-up. During this time, she showed partial initial response, but despite treatment the disease relentlessly progressed, with hemorrhage and severe pain being the most striking features.
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PMID:Angiosarcoma of the scalp. 1051 88

A retrospective analysis of 77 children treated between 1974 and 1996 was undertaken to evaluate morbidity and the evolution of therapy. A Wilms' tumor (WT) was present in 73 children. 74% of patients (pats.) with WT survived (54 of 73 pats.). Histological specimens of 67 patients were re-evaluated, including 4 children with non-WT histology. Among patients with Wilms' tumors (WT), nephroblastoma (NB) of intermediate risk predominated (73%; 46 of 63 pats.). Low-risk tumors occurred in 5 of 63 children (8%; mesoblastic nephroma 3, cystic partially diff. NB 1, completely necrotic NB 1). High-risk WT were diagnosed in 12 of 63 patients (19%) (NB with anaplasia 10, clear cell sarcoma 1, malignant rhabdoid tumor 1). Nephrogenic rests were present in 14 cases. We observed 3 children of school age with renal carcinoma and one patient with an intrarenal neuroblastoma. WT histology was the most important factor determining prognosis (p = 0.018). The risk for relapses was 2.6-fold higher in patients with high-risk WT compared to the standard risk group. Stages were re-evaluated according to SIOP 93-01. Comparing relapse-free survival of stages I, II and III, respectively, there was a reduced survival rate for stage III (p=0.019). According to the SIOP/GPOH protocol in 1989, the regimen was switched from primary surgery to preoperative chemotherapy without biopsy in 1989 (11 pats.). Compared to earlier years, survival improved (n.s.). In 3 patients preoperative diagnosis by means of imaging failed. During preoperative chemotherapy a venous occlusive disease of the liver occurred in 2 patients. Preoperative chemotherapy led to an impressive tumor shrinkage in the majority of patients. 2 patients of the preoperative group died (focal anaplastic NB). Long-term morbidity was analysed in 49 patients and included radiation-induced scoliosis (35), chest-wall deformity (3), congestive cardiomyopathy after relapse (1) and arterial hypertension (2). Over the years there was a trend to reduce frequency and dose of irradiation. Prognosis of WT is excellent but unfavorable histology (high risk) predicts a poor prognosis. In our experience, reduction of tumor volume due to preoperative chemotherapy facilitates tumor removal by surgery and may prevent tumor spillage and the deleterious effects of radiation in young children. Surgery without delay is necessary if the diagnosis is unclear or the tumor fails to respond to preoperative chemotherapy.
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PMID:A 23-year experience with malignant renal tumors in infancy and childhood. 1137 Oct 43

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

Autosomal-dominant polycystic kidney disease (ADPKD) is a systemic disease with multiple extrarenal manifestations. It accounts for 7% to 11% of patients receiving dialysis or renal transplantation (RT) for end-stage renal disease (ESRD) in Europe. We analyzed retrospectively the causes of death, the prevalence of cardiovascular risk factors (CVRF) and the patient and graft survivals in 62 consecutive ADPKD patients who received 63 cadaveric grafts (29 men and 34 women), of the 600 RTs performed between 1980-2001. The diagnosis of ADPKD was established by family history and ultrasound techniques. At present, 50 patients (79.4%) have functioning grafts, with a mean follow-up of 84.7 months (range, 12-255), and 13 patients have lost their grafts. The main cause of failure was patient death with a functioning graft (9 cases). Malignancies occurred in 5 patients, including 2 lymphomas, 1 renal carcinoma, 1 pancreas sarcoma, and 1 lung cancer associated with infection. Three patients died of cardiocerebrovascular events, and 1 patient of pneumonia. One patient lost the graft after decreasing the immunosuppression for an obstructing colon cancer. Three additional patients now on dialysis lost their grafts due to chronic rejection in 2 cases and primary nonfunction in 1 case. The prevalence of cardiovascular risk factors among the 50 patients with functional grafts were: hypertension, 70%; hypercholesterolemia, 62%; hyperhomocysteinemia, 30%; hyperfibrinogenemia, 68%; increased lipoprotein (a), 18%; microalbuminuria, 22%; hyperuricemia, 48%; hyperparathyroidism, 24%; overweight status, 24%; and nonlethal myocardial infarction, 10%. We conclude that ADPKD patients have good graft and patient survivals, and that the presence of malignancy is the main cause of death and graft failure at our center.
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PMID:Autosomal-dominant polycystic kidney disease: high prevalence of graft loss for death-related malignancies and cardiovascular risk factors. 1296 69

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

Large vessel involvement by skeletal and soft tissue sarcomas of the extremities does not change the modern limb sparing surgery for those neoplasms. An arterial and, if the vein is open, a venous bypass should always be offered to any patient young or old, with high or low grade sarcoma, because preserving the limb permits quicker rehabilitation, which is particularly useful in the case of a short life expectancy. In 650 cases of skeletal sarcomas, 10 arterial (1.5%) and four venous bypasses were done, all with autologous veins but one in PTFE; we had no problems except a silent arterial occlusion. Of 1000 patients with soft tissue sarcomas, 32 (3%) had vessel involvement permitting limb sparing surgery. The arterial bypass, which is the limb-saving operation, was performed 16 times with a PTFE with one early occlusion and four cases of prosthesis infection, with two amputations despite redo operation with an autologous vein. The more recent 16 cases were, therefore, always done with biological vessel substitution--autologous vein or tissue bank vessel--with only one infection that healed without operation and one case of homograft rupture followed by amputation. Since 1999 in all 13 resected cases with an open vein, we did the arterial and the venous bypass (twice PTFE, six autologous vein, and five bank vessel) with the aim of avoiding postoperative venous hypertension, but only four of the venous bypasses remained open. Venous bypasses are a harmless, but still experimental, procedure.
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PMID:Vascular homografts for vessel substitution in skeletal and soft tissue sarcomas of the limbs. 1618 86


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