Incidentally discovered adrenal masses, or adrenal incidentalomas, have become a common clinical problem owing to wide application of radiologic imaging techniques. This definition encompasses a heterogeneous spectrum of pathologic entities, including primary adrenocortical and medullary tumors, benign or malignant lesions, hormonally active or inactive lesions, metastases, and infections. Once an adrenal mass is detected, the clinician needs to address two crucial questions: is the mass malignant, and is it hormonally active? This article provides an overview of the diagnostic clinical approach and management of the adrenal incidentaloma. Mass size is the most reliable variable to distinguish benign and malignant adrenal masses. Adrenalectomy should be recommended for masses greater than 4.0 cm because of the increased risk of malignancy. Adrenal scintigraphy has proved useful in discriminating between benign and malignant lesions. Finally, fine-needle aspiration biopsy is an important tool in the evaluation of oncological patients and it may be useful in establishing the presence of metastatic disease. The majority of adrenal incidentalomas are non-hypersecretory cortical adenomas but an endocrine evaluation can lead to the identification of a significant number of cases with subclinical Cushing's syndrome (5-15%), pheochromocytoma (1.5-13%) and aldosteronoma (0-7%). The first step of hormonal screening should include an overnight low dose dexamethasone suppression test, the measure of urinary catecholamines or metanephrines, serum potassium and, in hypertensive patients, upright plasma aldosterone/plasma renin activity ratio. Dehydroepiandrosterone sulfate measurement may show evidence of adrenal androgen excess. (+info)
A case of giant malignant phaeochromocytoma.
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Malignant phaeochromocytoma is defined as the presence of tumour deposits at sites that are normally devoid of chromaffin cells. We report on a 63-year-old man who had a giant malignant phaeochromocytoma of the right adrenal gland that encased the inferior vena cava. The urinary excretion rates of catecholamines and their metabolites were normal, except for normetanephrine, which was excreted at a higher rate than normal. The tumour was surgically unresectable by laparotomy. Postoperatively, the patient was given a 4-month trial of subcutaneous octreotide and intravenous meta-iodobenzylguanidine I 131. Occult lung secondary tumours were first detected by meta-iodobenzylguanidine scintigraphy after 2 years, and the patient died of bone and lung metastases 1 year later. Because phaeochromocytoma is rare, local experience in managing this disease is limited. This report alerts physicians of the methods of diagnosing and managing surgically unresectable malignant phaeochromocytoma. (+info)
A 35-year-old man with cerebral hemorrhage and pheochromocytoma: the second brain-dead organ donor in Japan.
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A 35-year-old man was brought into the emergency room of Keio University Hospital by ambulance because of a sudden onset of coma. His Glasgow Coma Scale was 3 and his blood pressure 150/100 mmHg. CT scanning revealed a subcortical hemorrhage 8 cm in diameter. His respiration deteriorated rapidly, and an emergency craniotomy was performed for hematoma removal and cerebral decompression. Postoperatively the patient remained in a deep coma (GCS = 3) requiring respiratory support. The family presented an organ donor card previously signed by the patient, and brain death was confirmed in accordance with Japan's transplant law. As a result of two tests conducted six hours apart brain death was confirmed on the 5th postoperative day. With the family's consent, the donor's heart, kidneys and skin were removed for organ transplantation to be performed in other institutions. An autopsy was performed after the removal of the organs and skin. An extensive subgaleal hemorrhage was found in the left cerebral hemisphere, and microscopic examination revealed extensive necrosis with karyolysis of neuronal cells, but no viable neuronal cells were found in the cerebrum. The brain stem was marked by edema, hemorrhage, infarction necrosis and neuronal cell loss. The cerebellum was swollen and congested and showed autolysis of the granular layer. These findings suggested brain death syndrome with respirator brain. Other autopsy findings included a huge pheochromocytoma in the right adrenal gland, bilateral bronchopneumonia, liver congestion and fatty metamorphosis with four cavernous hemangiomas, and mild chronic lymphocytic thyroiditis. This patient was the second brain-dead organ donor and the first brain-dead patient to undergo postmortem examination in Japan. (+info)
Malignant pheochromocytoma lacking clinical features of catecholamine excess until the late stage.
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A malignant pheochromocytoma is described in a 71-year-old man. Osseous metastases became manifest 12 years after successful removal of the primary tumor which originated in paraganglionic tissue near the right adrenal gland. Although the patient had no symptoms of catecholamine excess initially, hypertension, tachycardia and excessive sweating appeared several months before his death, concomitantly with a sharp increase in noradrenaline secretion due to an accelerated growth of metastatic tumors. Since there is no histologic criterion of malignancy in this neoplasm, it would be prudent to consider every case of pheochromocytoma as potentially malignant and to follow-up carefully for a long time after removal of the primary tumor. (+info)
Leiomyosarcomas of great vessels.
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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. (+info)
Dexamethasone-induced cardiogenic shock rescued by percutaneous cardiopulmonary support (PCPS) in a patient with pheochromocytoma.
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A 52-year-old man with pheochromocytoma had cardiogenic shock and was rescued using a percutaneous cardio pulmonary supporting system. After recovery, diagnostic tests including metaiodobenzylguanidine scintigraphy and computed tomography, revealed the pheochromocytoma which was confirmed by histology. It was postulated that the acute episode was induced by intra-joint dexamethasone, which increased the production of epinephrine and augmented the sensitivity of cardiomyocytes for catecholamine, thereby inducing the cardiomyopathy. (+info)
Phaeochromocytoma diagnosed during labour.
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The diagnosis of phaeochromocytoma during pregnancy is rare. We present the management of vaginal delivery in a woman diagnosed with the condition during labour. A Medline search and follow-up of references failed to find any similar report in the last 30 years. (+info)
Endoscopic retroperitoneal adrenalectomy: lessons learned from 111 consecutive cases.
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OBJECTIVE: To evaluate the effectiveness of endoscopic retroperitoneal adrenalectomy (ERA). SUMMARY BACKGROUND DATA: Minimally invasive adrenalectomy has become the procedure of choice for benign adrenal pathology. Although the adrenal glands are located in the retroperitoneum, most surgeons prefer the transperitoneal laparoscopic approach to adrenal tumors. METHODS: Clinical characteristics and outcomes of 111 ERAs from January 1994 to December 1999 were evaluated. RESULTS: Ninety-five patients underwent 111 ERAs (79 unilateral, 16 bilateral). Indications were Cushing syndrome (n = 22), Cushing disease (n = 8), ectopic adrenocorticotropic hormone syndrome (n = 6), Conn's adenoma (n = 25), pheochromocytoma (n = 19), incidentaloma (n = 11), and other (n = 4). Tumor size varied from 0.1 to 8 cm. Median age was 50 years. Unilateral ERA required 114 minutes, with median blood loss of 65 mL. Bilateral ERA lasted 214 minutes, with median blood loss of 121 mL. The conversion rate to open surgery was 4.5%. The complication rate was 11%. Median postoperative hospital stay was 2 days for unilateral ERA and 5 days for bilateral ERA. The death rate was 0.9%. At a median follow-up of 14 months, the recurrence rate of disease was 0.9%. CONCLUSION: For benign adrenal tumors less than 6 cm, ERA is recommended. (+info)