Cases reported "Pheochromocytoma"

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1/18. Combined surgery for coronary artery disease and pheochromocytoma.

    PURPOSE: To report a case of severe coronary artery disease complicating pheochromocytoma, managed with combined coronary artery bypass grafting (CABG) and adrenalectomy. CLINICAL FEATURES: A 55-yr-old woman presented with poorly controlled hypertension and investigation revealed an active pheochromocytoma of her left adrenal gland. During medical preparation for adrenalectomy, she developed an acute myocardial infarct complicated with unstable angina. This required urgent CABG, and combined surgery for the triple vessels coronary artery disease and the pheochromocytoma was planned. We explain the details of medical preparation before surgery and the anesthetic considerations during the surgical procedure. Postoperative recovery was normal and no complication occurred. Even if the pheochromocytoma was malignant, her urinary catecholamines two months after the surgery were normal and remain normal after more than two years of follow-up. CONCLUSION: We report a patient who underwent combined CABG and adrenalectomy for pheochromocytoma. The CABG was done first, followed by the adrenalectomy with invasive monitoring. The procedure was well tolerated with cure of the two underlying conditions. So we propose that combined procedure should be considered in this clinical setting.
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2/18. pheochromocytoma with renal artery stenosis and high plasma renin activity.

    An unusual case of pheochromocytoma is described in this communication. Besides a chain of typical clinical pictures and laboratory findings which suggested a catecholamine-producing tumor, the left renal artery stenosis was demonstrated by an aortography and the plasma renin activity was consistently elevated. Surgery revealed the left renal artery was embedded in the tumor mass, originated from the left adrenal gland, resulting in a high degree of constricture of the vessel. Following the removal of the tumor, blood pressure immediately returned to normal, however, plasma renin activity remained elevated as long as 9 months of the follow-up study. The second aortography performed 14 months after the operation failed to demonstrate the left renal artery stenosis and subsequent studies revealed that plasma renin activity was gradually declining to upper normal levels. It is suggested that an excess of catecholamines secreted by the tumor was responsible for hypertension in this case, and that another factor, probably renal artery stenosis, was involved in the elevation of plasma renin activity, although this high renin activity was maintained for more than 9 months following the tumor extirpation.
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3/18. Mandibular metastasis presenting as the initial maifestation of malignant pheochromocytoma.

    A case of 59-year-old male patient presenting with metastasis to the mandible from malignant pheochromocytoma is described. The conventional radiographs and CT images suggested that the lesion was malignant osteogenic tumors or metastatic tumors due to the existence of calcification and widespread periosteal sunburst spiculation. On MRI, an expansive mass was clearly depicted and the signal intensities of the lesion were low to intermediate on T1 weighted image with intermediate to high signal intensity on T2 weighted image. A strong enhancement of the lesion was also observed on contrast enhanced T1 weighted image. On maximum intensity projection image in the arterial phase, the mass showed exceedingly early enhancement and excessively dislocated adjacent vessels. The diagnosis of a pheochromcytoma was difficult on the basis of these imagings. The final diagnosis was based on a biopsy of the mandible and I-131 Meta-iodobenzylguanidine scintigraphy (MIBG) scintigraphy. A primary lesion of the right adrenal showed low uptake due to wide centric necrosis and metastatic lesions of liver, lumber vertebrae, ribs and sacroiliac joint showed high uptake on the I-131 MIBG scintigraphy. The final diagnosis was nonfunctioning malignant pheochromocytoma due to the absence of elevation of catecholamine or its metabolite.
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4/18. A case of a ruptured pheochromocytoma with an intratumoral aneurysm managed by coil embolization.

    Although the spontaneous rupture of adrenal pheochromocytoma is rare, it can be lethal because it can induce serious changes in the circulation. We describe a 32 year old man with bilateral pheochromocyroma presenting as abdominal pain. In the emergency room, an abdominal MRI showed an aneurysmal vessel in the right adrenal mass and accompanying hemorrhage around the tumor capsule. The bleeding site was found by transfemoral abdominal angiography. Coil embolization was done in the bleeding vessels, specifically branches of the right adrenal artery. The hemorrhage was successfully controlled and vital signs of the patient were restored. Following emergency care, biochemical and imaging studies showed compatible findings of a bilateral adrenal pheochromocytoma. Postoperative histologic findings confirmed these observations. A ruptured pheochromocytoma should be considered as a cause of acute abdomen in cases of a concomitant adrenal mass. Intratumoral aneurysmal bleeding may be a cause of ruptured tumor, and careful angiographic intervention will help to ensure safe control of bleeding in such an emergency situation, even in cases of bilateral tumor.
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5/18. Retroperitoneal malignant fibrous histiocytoma mimicking pheochromocytoma.

    We report the case of a 52-yr-old man with a mass in the area of the left adrenal. The clinical features, MIBG uptake, and elevated urinary dopamine levels suggested the diagnosis of pheochromocytoma. He presented with unstable hypertension, tachycardia, weight loss, and the "inflammatory syndrome" (fever, leukocytosis, and high sedimentation rate). Clinical findings, preoperative radiographic (sonography, CT scan, [131I]MIBG scintigraphy), and endocrine evaluations (elevated 24-h urinary dopamine) were suggestive of a dopamine-secreting adrenal tumor. The mass was resected and on histologic examination showed the characteristic features of a malignant fibrous histiocytoma (MFH). The tumor cells were immunopositive for neuron-specific enolase (NSE), vimentin, CD-68, S-100, desmin, and immunonegative for chromogranin a, synaptophysin, neurofilament protein, and low-molecular-weight keratin, indicating that this tumor was not able to synthesize catecholamines. The prolonged retention of the tracer (MIBG) was interpreted as a consequence of obstructive hydronephrosis, while elevated urinary dopamine levels were assumed to be due to compression of the renal vessels by the large retroperitoneal mass.
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6/18. pheochromocytoma and ischemic enterocolitis.

    In the nine year period, 1978-1987, two patients with pheochromocytoma presented to Sacred heart Hospital, Yankton, south dakota. Both cases were complicated pre-operatively by ischemic enterocolitis--a rarely reported, usually fatal complication of pheochromocytoma. It is thought that catecholamine-induced vasoconstriction of the mesenteric vessels resulted in the wide-spread hemorrhagic necrosis of the gut. Both patients survived not only the initial ischemic mesenteric insult, but also excision of the tumor.
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7/18. Removal of pheochromocytoma by enucleation.

    In 6 cases pheochromocytomas were removed by enucleation. A typical case is reported in detail illustrating that it might be a procedure of choice in avoiding the risk of injuring the great vessels during operation.
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8/18. ultrasonography of adrenal masses: unusual manifestations.

    Forty patients with pathologically-proved adrenal masses, and two patients with extra-adrenal pheochromocytomas, were examined with gray scale B-scan. The authors describe unusual echo patterns of adrenal masses due to necrosis or hemorrhage, variations in the effects of large adrenal masses on surrounding organs and vessels, and the differential diagnosis. The complementary role of computed tomography is also discussed.
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9/18. role of intraoperative manometry in renal artery compression by a pheochromocytoma.

    A case of renal artery stenosis due to compression by a neurofibrous cord (histologically a pheochromocytoma) and resolved by the debridment of the vessel and angioplasty with a patch in the saphenous vein, is reported. After the compression has been relieved, the vessel appeared normal at inspection and palpation (pulsation); the manometric control, recorded distally and proximally to the area of compression by the pheochromocytoma showed a significant gradient of pressure that led to the exploration of the vessel, partially obstructed by a recent thrombus. These lesions were not detectable solely on inspection and palpation. It is stressed that a restored normal aspect after removal of the extrinsic pressure on the renal artery may not represent an equivalent optimal restoration of function. Only manometric control or rate of flow measurements are able to assess the efficacy of surgical reconstruction.
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10/18. Computed tomography of juxtacardiac pheochromocytoma.

    The diagnosis of juxtacardiac pheochromocytoma was made with the help of three-dimensional computed tomography, angiography, and radionuclide scintigraphy, and was proved at surgery. If findings on adrenal computed tomography are normal and the clinical and laboratory findings support the diagnosis of pheochromocytoma, the patient should be further investigated by computed tomography or radionuclide-guided computed tomography and angiography to determine the extraadrenal location of this tumor. If the abnormal area is in the mediastinum, it is essential to do either direct coronal computed tomography imaging of the suggestive area or to obtain coronal and sagittal reconstruction images to investigate the relationship of the lesion to the great vessels and cardiac chambers.
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