Cases reported "Adrenal Gland Neoplasms"

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1/35. jaw and pulpal metastasis of an adrenal neuroblastoma.

    Hematogenous spread of malignant tumors to the dental pulp is very rare. A case of adrenal neuroblastoma in a 71/2-year-old boy which metastasized to the mandible and dental pulp is described. Tumor cells were found within the pulpal blood vessels of a deciduous molar tooth.
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2/35. 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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3/35. Leiomyosarcomas of great vessels.

    Sarcomas of the great vessels are rare. Altogether 400 such cases have been described in the aorta, the pulmonary artery, and inferior vena cava. The clinical symptoms are generally related to embolic phenomena, aneurysm formation, and widespread metastases, especially to bones. With improved diagnostic modalities more cases are diagnosed and treated surgically. Resection of the tumor may prolong the patient s life. In this paper authors present two cases of such rare sarcomas. In our first case a tumor has developed in the thoracic aorta with symptoms of imminent aortic dissection. The tumorous nature of the lesion was revealed only histologically, since neither the operation, nor macroscopic picture gave any clue to its tumorous nature. The second case was a male patient with a huge retroperitoneal tumor arising from the inferior vena cava, which was clinically suspected to be a carcinomaarising in the adrenal gland.
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4/35. 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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5/35. Selective adrenal venography in an infant with virilizing adrenal cortical carcinoma.

    Selective adrenal venography in a 10 month old infant with adrenal cortical carcinoma and virilizing symptoms demonstrated grossly dilated, irregular tumor veins. This was an unexpected finding as at aortography no adrenal arteries which would allow selective catheterization, and only very small neoplastic vessels were seen.
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6/35. 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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7/35. 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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8/35. 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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9/35. The da Vinci robot in right adrenalectomy: considerations on technique.

    The da Vinci Robotic System (Intuitive Surgical, Mountain View, CA) became available at the general surgery Department of Camposampiero Hospital in May 2001. From May 2001 to October 2002, 139 robotic operations were performed, one of which was a right adrenalectomy for a right adrenal mass. The progressive growth of the mass was the indication for surgical excision. Surgical adrenalectomy was successfully completed with da Vinci Robotic System using 5 ports (3 for the robotic system, 2 as service trocars). The wrist-like movements of the instrument's tip easily enabled the detachment of the right hepatic lobe from the gland and vessel isolation, while the 3-dimensional vision facilitated dissection of the veins from the vena cava.
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10/35. Studies of 16-androstenes in an infant with virilizing adrenal carcinoma.

    An 18-month-old girl with virilization was found to have an encapsulated right adrenal carcinoma (2 x3 cm) with great variation in nuclear size, frequent mitoses, and possible blood vessel invasion. Preoperative urinary excretions of 17-ketosteroids, androsterone, etiocholanolone, dehydroepiandrosterone, testosterone, pregnanetriol, 3alpha-androstenol, and 3 beta-androstadienol were elevated; all showed a noticeable decrease postoperatively. Cortisol acetate, given preoperatively, produced a definite decrease in the urinary excretion of 17-ketosteroids and dehydroepiandrosterone; administration of corticotropin resulted in an increase in levels of urinary 17-ketosteroids, 17-hydroxycorticosteroids, and pregnanetriol. Urinary testosterone and 3beta-androstadienol may have diagnostic value since neither was suppressed by cortisol therapy. The behavior of both 3alpha-androstenol and 3beta-androstadienol in this study suggests that they are of adrenal origin.
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