Cases reported "Pheochromocytoma"

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1/49. A case of extraadrenal pheochromocytoma associated with adrenal cortical nodular hyperplasia and papillary thyroid carcinoma.

    A 64-year-old woman was admitted in November, 1996 for fluctuating blood pressure. There was multinodular goiter in her neck. High urine VMA and serum aldosterone were noted. Computed tomography showed an oval lesion in the left adrenal gland. Left adrenalectomy was performed and the pathology was proved to be adrenal cortical nodular hyperplasia. Fluctuating blood pressure and high urine VMA persisted after the operation. CT scan of the abdomen revealed a soft tissue mass in lower abdomen. The patient was admitted again in September, 1997. Laboratory examinations showed normal serum aldosterone, normal plasma renin activity and high urine VMA. Aspiration cytology of the thyroid gland disclosed papillary thyroid carcinoma. [131I]-metaiodobenzylguanidine image revealed a high uptake lesion in the right L-3 paravertebral area. Tumor excision and thyroidectomy were performed. The pathology was reported as extraadrenal pheochromocytoma and papillary thyroid carcinoma. Papillary thyroid carcinoma is rarely associated with pheochromocytoma. To our knowledge, this paper is the first report of a patient with extraadrenal pheochromocytoma associated with papillary thyroid carcinoma and adrenal cortical nodular hyperplasia.
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2/49. renal artery stenosis and pheochromocytoma: coexistence and treatment.

    PURPOSE: The aim of this study was to develop an optimal treatment plan for coexisting pheochromocytoma and renal artery stenosis. methods: A retrospective analysis of the evaluation and management of a hypertensive 14-year-old boy was conducted. RESULTS: Secondary causes of hypertension were excluded initially including intracranial, cardiac, and endocrine abnormalities. A renal scan showed right renal function at 39%. angiography confirmed a 90% subsegmental stenosis, as well as noting a blush suggesting a tumor. A computerized tomography (CT) scan of the abdomen showed a right adrenal mass. serum epinephrine was 3,006 pg/mL (normal, <1,009 pg/mL) and 24-hour urinary norepinephrine was 2,001 microg (normal, <105 microg/24 h), suggesting the presence of a pheochromocytoma. During the operation for resection, a right subsegmental renal artery was found to be draped around a renal hilar mass; the adrenal gland was normal. The tumor was resected, and pathology confirmed a pheochromocytoma. Postoperatively, the patient had some mild hypertension that gradually resolved. CONCLUSIONS: Extraadrenal pheochromocytomas (paragangliomas) occur more frequently in children and are most commonly located in the renal hilum. In this location, they may present initially as renal artery stenosis as a result of direct arterial compression, fibrous bands, or catecholamine-induced vasospasm. Our experience supports tumor resection of extraadrenal pheochromocytomas as the definitive treatment for both conditions.
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3/49. Primary pheochromocytoma extending into the right atrium: report of a case and review of the literature.

    pheochromocytoma rarely extends locally into the vena cava or the right atrium. We report a case of malignant pheochromocytoma with growth into the inferior vena cava, extending into the right atrium, address clinical aspects of this tumour and review the literature on this malignancy. Pre-operative work-up of this tumour should include measurements of urinary vanillyl mandelic acid and cathecholamine excretion, MRI and spiral CT of the abdomen and thorax. After the diagnosis is made the patient should be treated with catecholamine alpha-receptor blockade and if necessary with subsequent beta-receptor blockade. An aggressive surgical approach is always warranted, even in cases with very large localized tumours, because surgery has been shown to lead to relief of symptoms and to prolong survival in cases otherwise deemed irresectable. The optimal surgical exposure is obtained via a transsternal midline thoraco-laparotomy. If feasible, a combination of cardiopulmonary bypass, hypothermia, cardiac arrest and exsanguination procedures should be used. In case of local of tumour remnants after surgery or distant metastases treatment options are secondary surgery, tumour embolization, or treatment with radioactive labelled drugs, including(131)I-MIBG. copyright Harcourt Publishers Limited.
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4/49. A case of "silent" pheochromocytoma presenting as spontaneous retroperitoneal hematoma.

    pheochromocytoma of the adrenal gland can be the cause of massive and lethal retroperitoneal haemorrhage presenting as acute abdomen. Here we report a case of retroperitoneal hematoma, with concomitant peritoneal spillage, due to the spontaneous rupture of a silent pheochromocytoma. The main clinical findings of this disease will be described. Therapy and prognosis will be also discussed.
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5/49. Cardiac phaeochromocytoma presenting with severe hypertension and chest pain.

    Cardiac phaeochromocytoma is a rare cause of endocrine hypertension. We report a case of a 25-year-old woman, who presented with severe hypertension and intermittent chest pain. The patient denied typical phaeochromocytoma spells of palpitation, headache, and diaphoresis. The 24-hr urinary excretion of norepinephrine was increased sevenfold above the upper limit of normal; however, the excretion of total metanephrines, epinephrine, and dopamine were normal. Computed tomography (CT) scan of the abdomen was normal. An 131I-labelled metaiodobenzylguanidine (MIBG) scan was falsely negative while the patient was taking labetalol. The cardiac phaeochromocytoma was localized with indium-111-pentetreotide scintigraphy and chest magnetic resonance imaging scan. Repeat 123I-MIBG scintigraphy was positive after discontinuing labetalol. The cardiac phaeochromocytoma was located in the right atrial groove, adjacent to the tricuspid valve, and contained multiple feeder arteries from the right coronary artery. After treatment with volume expansion, alpha-methyl-p-tyrosine, and alpha- and beta-adrenergic blockade, surgical resection was performed. While under cardiopulmonary bypass, coronary bypass grafting and tricuspid annuloplasty were performed to facilitate the complete surgical resection of the 4.5-cm tumour. The surgical course was uncomplicated, with complete cure of hypertension and normalization of catecholamine excretion. Post-operative cardiac function, as measured by echocardiogram, was normal. Although cardiac phaeochromocytoma may be highly vascular, invasive and difficult to resect, it can be cured.
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6/49. Twenty-six-years' survival with multiple bone metastasis of malignant pheochromocytoma.

    The prognosis of metastatic pheochromocytoma is poor in general. There have been few instances of long-term survival reported. We report a case of a 44-year-old woman who has survived for 26 years after bone metastasis. She was diagnosed as having pheochromocytoma arising in the left adrenal medulla in 1974. Metastasis of pheochromocytoma in the first and third lumbar vertebrae and the right ilium was observed at the same time. The primary lesion was removed, and posterior lumbar spinal fusion was performed for immobilization. The metastatic lesion in the ilium was left untouched. After 26 years, she is well despite a recurrence of the tumors in the skull and a new metastasis in the left abdomen.
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7/49. I-123 MIBG imaging and intraoperative localization of metastatic pheochromocytoma: a case report.

    The authors describe the diagnostic use of I-123 MIBG scintigraphy in a 61-year-old man who was thought to have a recurrence 25 years after a left adrenalectomy for a pheochromocytoma. Preoperative I-123 MIBG scintigraphy was performed twice along with intraoperative gamma probe localization of the lesions. The preoperative MIBG scintigraphy revealed three pathologic processes in the upper left abdomen, whereas computed tomographic scanning identified only one site of involvement. All three metastatic lesions were removed successfully with the aid of a gamma probe. Preoperative I-123 MIBG scintigraphy, combined with intraoperative gamma probe identification of I-123 MIBG foci, is feasible and a valuable tool to detect malignant masses possibly overlooked by other imaging techniques.
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8/49. Laparoscopic resection of a large periadrenal nonmalignant pheochromocytoma.

    This is, to our knowledge, the first case description of an extraadrenal pheochromocytoma located in the periadrenal fat. pheochromocytoma is a tumor that originates in the chromaffin tissue. Extraadrenal pheochromocytomas have been described commonly in locations such as the organ of Zuckerkandle (29%); the bladder (12%); the sacrum, testis, rectum, and pelvic floor (2%); the upper abdomen in association with celiac, superior mesenteric, and inferior mesenteric ganglia (43%); the thorax (12%); and the neck (2%), most commonly in association with the ninth or tenth cranial nerve ganglion. Our patient was a 40-year-old woman known to have had an adrenal mass for the last 4 years. She was referred for surgery because of an increase in the size of the mass to 11 cm. Laparoscopic adrenalectomy was performed via a posterior flank approach. The pathology report was of periadrenal fat pheochromocytoma, with positive staining for synaptophysin, chromogranin, and vimentin. The patient was discharged on postoperative day 3. The unique feature in this case was the uncommon location of the extraadrenal tumor: the supraadrenal fat. The other unique finding in this case was that the pheochromocytoma was neither symptomatic nor malignant, common features of extraadrenal masses.
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9/49. Paroxysmal hypertension, pheochromocytoma, and pregnancy.

    BACKGROUND: hypertension is the most common medical complication of pregnancy. pheochromocytoma in pregnancy is rare, and if unrecognized, can cause serious perinatal morbidity and mortality. methods: A patient with severe hypertension, postpartum pulmonary edema, and a recognized pheochromocytoma is described. RESULTS: Abdominal palpation after vaginal childbirth reproduced the diagnostic triad of hypertension, headaches, and palpitations. magnetic resonance imaging established the correct diagnosis before biochemical confirmation of excess catecholamine production. The patient responded to alpha-adrenergic receptor blockade with control of her severe hypertension and clearing of pulmonary edema. The best time to diagnose a pheochromocytoma is before delivery because vaginal childbirth stimulates the release of lethal amounts of catecholamines. CONCLUSIONS: The physician who delivers babies must distinguish between labile hypertension and paroxysmal hypertension. Most experts believe that a spontaneous vaginal delivery is contraindicated when the patient has a pheochromocytoma. Postpartum pulmonary edema associated with a pheochromocytoma is unusual. The profound pressor response elicited by palpation of the postpartum abdomen, the failure of medications usually effective in the treatment of a hypertensive crisis, and the use of magnetic resonance imaging to confirm a functioning adrenal adenoma are the features unique to this case.
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10/49. New onset systemic lupus erythematosus with pheochromocytoma.

    We describe a patient with positive antinuclear and anti-Smith antibodies, proteinuria, and thrombocytopenia suggesting systemic lupus erythematosus (SLE). During hospitalization, the patient developed labile hypertension, tachycardia, and intermittent fever. A computer tomography scan of the abdomen showed an extraadrenal mass, which was confirmed as a pheochromocytoma. After removal of the pheochromocytoma, the patient's symptoms resolved and her serology normalized. Previous case reports describe SLE patients with adrenal pheochromocytomas that presented many years after the diagnosis of lupus. This is a novel case of pheochromocytoma discovered at the onset of SLE, with resolution of SLE manifestations shortly after its removal.
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