1/26. Extra-adrenal pheochromocytoma (paraganglioma).The authors describe the case of a 19-year-old female patient with an abdominal paraaortic extra-adrenal pheochromocytoma (paraganglioma), presenting arterial hypertension. The predominant catecholamine produced by the tumor was norepinephrine (4110 pg/ml; normal < 450 pg/ml). 131I metaiodobenzylguanidine (131I-MIBG), computed tomography and magnetic resonance imaging allowed location and characterization of the tumor. Histologically the tumor (weight = 34.2 g; 5.8 x 4 x 3 cm) was a typical pheochromocytoma.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
2/26. paraganglioma manifesting as shock: a case report.paraganglioma is a rare neuroendocrine tumor in children that rarely manifests as shock. We describe the case of a 12-year-old girl with paraganglioma who developed impaired cardiac function, pulmonary edema, and shock at the time of admission. Her blood pressure stabilized after intravenous normal saline rescue and dopamine treatment. However, hypertension was noted thereafter. After a series of examinations, paraganglioma was diagnosed and excision of the tumor was performed. After surgery, blood pressure stabilized and her cardiac function had fully recovered at 4 months' follow-up.- - - - - - - - - - ranking = 8.1931786560244keywords = blood pressure, hypertension, pressure (Clic here for more details about this article) |
3/26. Typical and atypical Carney's triad presenting with malignant hypertension and papilledema.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.- - - - - - - - - - ranking = 8keywords = hypertension (Clic here for more details about this article) |
4/26. A case of catecholamine induced heart failure with left ventricular hypertrophy accompanied by mid-ventricular obstruction.A 36 year old Turkish female patient complaining of widespread redness of the skin, shortness of breath, palpitations, nausea, hum and reverberation in the head was examined. The patient was diagnosed with catecholamine induced hypertension, which was caused by paraganglionoma. In addition, left ventricular concentric hypertrophy accompanied by systolic gradient in mid-ventricle, which is rarely observed, was determined by echocardiography. Hypertensive attacks and mid-ventricular systolic gradient disappeared after surgery. This case shows that one of the causes of the heart failure due to catecholamine releasing tumors can be left ventricular obstruction.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
5/26. A painful pulsatile abdominal mass in a young man with elevated blood pressures: an unusual presentation of phaeochromocytoma.We report an unusual presentation of phaeochromocytoma in a young man with a painful, pulsatile abdominal mass and elevated blood pressures. This led to a delay in diagnosis and resulted in the administration of triggers of catecholamine release, possibly causing a catecholamine surge. This caused the development of catecholamine-induced cardiomyopathy and multiple organ failure, requiring inotropic and ventilatory support, intra-aortic balloon pump and dialysis. Fortunately, his condition reversed with supportive treatment and alpha-adrenergic blockade. This illustrates the importance of having a high index of suspicion of phaeochromocytoma, especially in young patients with elevated blood pressures.- - - - - - - - - - ranking = 21.579535968073keywords = blood pressure, pressure (Clic here for more details about this article) |
6/26. Variable hemodynamic fluctuations during resection of multicentric extraadrenal pheochromocytomas.PURPOSE: To report the perioperative management and the serious hemodynamic fluctuations during manipulation of an organ of Zuckerkandl tumour in a patient undergoing resection of multicentric extraadrenal pheochromocytomas. CLINICAL FINDINGS: A 28-yr-old man who had undergone at age 12 a laparotomy for excision of an extraadrenal pheochromocytoma complained of paroxysmal headache, occasional sweating and palpitations. The arterial blood pressure (BP) was 200/100 mmHg. A 24-hr-urine collection showed catecholamines 5076 microg x 24 hr(-1) (normal < 25 microg x 24 hr(-1)). Computed tomography of the abdomen revealed two retroperitoneal masses, one adjacent to the lower pole of the right kidney and a second larger mass located at the aortic bifurcation in the region of the organ of Zuckerkandl. The patient was scheduled for excision of multiple extraadrenal pheochromocytomas. He was prepared preoperatively for two weeks with prazosin 1 mg po q six hours and propranolol 10 mg tid. Manipulation of the infrarenal tumour was uneventful but manipulation of the Zuckerkandl tumour resulted in severe hypertensive episodes with BP ranging from 200/100 to 320/120 mmHg. Surgery was interrupted temporarily; the hypertensive crisis was controlled by the infusion of sodium nitroprusside and by iv phentolamine and esmolol. CONCLUSION: In a patient undergoing resection of recurrent multicentric extraadrenal pheochromoctyomas, severe hypertensive episodes occurred during manipulation of one tumour but not during manipulation of the other. This may be attributed to inadequate preparation of the patient, difficult surgical dissection of the large Zuckerkandl pheochromocytoma, and/or secondary to an excessive and different pattern of release of catecholamines during manipulation of the Zuckerkandl tumour.- - - - - - - - - - ranking = 3.5965893280122keywords = blood pressure, pressure (Clic here for more details about this article) |
7/26. Abdominal and pelvic extra-adrenal paraganglioma: a review of literature and a report on 7 cases.INTRODUCTION: Extra-adrenal paraganglioma (pheochromocytoma) is a rare tumor. Herein we describe the clinical and pathological findings in patients with paragangliomas of the urinary bladder, seminal vesicle and retroperitoneum. methods: Between January 1994 and January 2001, extra-adrenal paragangliomas were diagnosed in 7 patients: 3 males and 4 females. The mean age of our patients was 32 /- 15.9 years. We reviewed the clinical data. Urinary metanephrines and vanillyl mandelic acid and blood catecholamine levels were estimated in 4 cases. CT scan and/or MRI were used in the imaging of all cases. 123I-MIBG was used in only 1 patient, who harbored multiple tumors. All the patients but one underwent surgical treatment. RESULTS: The definitive diagnosis was made by histopathological examination of the removed tumors and was confirmed in all cases by the immunohistochemical stains of chromogranin a and S100 protein. There was metastasis in the pelvic lymph nodes in 1 patient. Follow-up ranged from 3 to 82 months (mean = 37.9 /- 25.8). The catecholamine level was elevated in 3 patients under basal conditions and during endoscopic resection of the tumor in a fourth patient. In all cases, the catecholamine level was normalized after surgery. There was no recurrence or metastasis in any case following surgery. CONCLUSION: Pre-operative diagnosis of nonfunctioning bladder paraganglioma is difficult, but the tumors should be suspected in patients who have hypertension, hematuria or mass effects due to the tumor growth in the pelvis and/or retroperitoneum. Six of the seven cases reported here were found in the usual locations: 3 in the urinary bladder, 2 in the renal hilum and 1 in the organ of Zuckerkandl. One patient had multiple tumors, including a paraganglioma of the seminal vesicles. Resection is the treatment of choice, and in the case of urinary bladder paraganglioma should include total cystectomy. In patients with unresectable multiple tumors, medical therapy may be used to control hypertension.- - - - - - - - - - ranking = 2keywords = hypertension (Clic here for more details about this article) |
8/26. Extra-adrenal pheochromocytoma (paraganglioma) of the urinary bladder: a case report.The case of a 49-year-old male patient with paraganglioma of the urinary bladder is presented here. The patient's only complaint was of gross hematuria: sustained hypertension and post-micturitional hypertension were not presented. Transurethral resection was performed to diagnose the bladder tumor. Pathological examination resulted in the diagnosis that the resected tissue was a paraganglioma. Computed tomography, magnetic resonance imaging and iodine-131-labeled metaiodbenzylguanidine scintigraphy revealed that the tumor was a primary paraganglioma in the urinary bladder. plasma concentrations of the catecholamines were virtually within the normal limits. Hypertensive crisis was not revealed during the transurethral resection. The tumor was non-functional. Partial cystectomy was performed. The patient has remained disease-free for five months after surgery.- - - - - - - - - - ranking = 2keywords = hypertension (Clic here for more details about this article) |
9/26. Metastatic renal cell carcinoma to the temporal bone: case report.We report an extremely rare case of metastasic renal cell carcinoma to the temporal bone which presented initially as a jugulotympanic paraganglioma. The clinical and radiological appearances were misleading.Investigations of concomitant high blood pressure revealed a tumour of the right kidney. biopsy of the mastoid mass was histologically compatible with a metastasis from a clear cell renal carcinoma.The patient underwent a radical nephrectomy and local external radiotherapy to the head. He also received adjuvant treatment with interferon-_ and interleukin 2. The clinical presentation, the radiological and histological features, the patterns of spread, the treatment options and the prognosis of these tumours are discussed. A review of the literature confirms the extremely unusual occurrence of this localisation.- - - - - - - - - - ranking = 3.5965893280122keywords = blood pressure, pressure (Clic here for more details about this article) |
10/26. Familial paraganglioma.Paragangliomas are unusual tumors that are sometimes familial. We treated a family who exhibited multiple head and neck paragangliomas (HNPGs) and pheochromocytomas. The purpose was to determine the clinical characteristics of paragangliomas with familial history and to define a better standardized proceeding in the management of these tumors. patients diagnosed with head and neck paragangliomas and identified retrospectively through clinical otolaryngology practices were given a medical and family history questionnaire. We studied a family who exhibited familial paragangliomas. This relationship was examined by reviewing the medical records of family members with verified tumors, carrying out neck computed tomography or magnetic resonance imaging on their relatives to look for tumors that had been unrecognized in the past. All patients underwent a complete head and neck examination. The initial evaluation usually included CT and/or MRI. Computed tomography and magnetic resonance imaging contributed additional information about tumor extension. angiography was performed in every patient with carotid body tumor, with one undergoing therapeutic embolization to reduce the tumor size. Eleven tumors were identified in four patients with a familial history. Familial disease was initially determined by pedigree analysis. Four patients with a median age of 31 years (range: 25-42) underwent surgery. Median follow-up was 5 years (range 2-14); carotid angiography provided essential mainstays for the definite diagnosis. All patients underwent successful surgical resection of the tumor after the appropriate preoperative preparation. There were no perioperative deaths or hemiplegia. Three patients had bilaterality carotid body paragangliomas. One patient had three paragangliomas, and two patients had bilateral carotid body paragangliomas associated with pheochromocytoma. Clinically functioning tumors and malignant tumors were not identified, and none of the patients died after surgery. During follow-up, none of the patients developed recurrence or metastatic disease. The carotid body paraganglioma (CBPG) and glomus vagale manifested as asymptomatic neck masses. The clinical pheochromocytomas typically present with uncontrolled hypertension. In conclusion, paragangliomas are rare, with multicentricity being more common in patients with a familial history. In patients with familial paragangliomas, high-resolution computed tomography and magnetic resonance imaging are recommended for early screening and contributed additional information about the tumor extension and definitive treatment. Early surgery is recommended to minimize major risks.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
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