Childhood Familial Pheochromocytoma
نویسنده
چکیده
Childhood familial pheochromocytoma was investigated in four patients by abdominal computed tomographic scan, [ IJmetaiodobenzylguanidine scan, and vena caval catecholamine sampling. Results conflicted with surgical findings. Computed tomographic scan identified all four adrenal tumors but missed two mid line tumors in one patient. [I]metaiodobenzylguanidine scan identified two of three adrenal tumors but also suggested extra-adrenal tumors not confirmed at operation in two of three patients. Vena caval sampling for catecholamines confirmed all adrenal tumors but suggested additional tumors not verified at operation in two of three patients. All patients are asymptomatic and have normal urinary catecholamines 15 to 51 months after operation. Because of the frequency of multiple tumors in familial pheochromocytoma, different diagnostic techniques were employed. False-positive results were more frequent with [I]metaiodobenzylguanidine and vena caval sampling. Reinterpretation of the [I]metaiodobenzylguanidine scans at a later date led to less false-positive interpretation, although the false-negative rate remained unchanged. More pediatric experience with [ Ijmetaiodobenzylguanidine scans and vena caval sampling in familial pheochromocytoma is needed. Confirmation of tumor and its localization rest with meticulous surgical exploration. (Hypertension 8: 851-858, 1986)
منابع مشابه
فئوکروموسیتوم راجعه و فئوکرموسیتوم فامیلیال RECURRENT PHEOCHROMOCYTOMA AND FAMILIAL PHEICHROMOCYTOMA
متن کامل
Childhood familial pheochromocytoma. Conflicting results of localization techniques.
Childhood familial pheochromocytoma was investigated in four patients by abdominal computed tomographic scan, [131I]metaiodobenzylguanidine scan, and vena caval catecholamine sampling. Results conflicted with surgical findings. Computed tomographic scan identified all four adrenal tumors but missed two midline tumors in one patient. [131I]metaiodobenzylguanidine scan identified two of three adr...
متن کاملPediatric and adolescent pheochromocytoma: clinical presentation and outcome of surgery.
OBJECTIVES To describe the clinical presentation and outcome of surgery in children with pheochromocytoma in a tertiary care hospital in India. METHODS Clinical records of 24 children who were operated between January 1990 and January 2011 were reviewed. The diagnosis of familial disease was established based on clinical examination and follow-up events. RESULTS Familial, bilateral, extra-a...
متن کاملRecent advances in genetics, diagnosis, localization, and treatment of pheochromocytoma.
Pheochromocytoma is a rare but important tumor of chromaffin cells that is frequently considered in the evaluation of hypertension, arrhythmias, or panic disorder and in the follow-up of patients with particular genetic diseases. This report provides an update about the genetics, neurochemical diagnosis, localization by imaging, and surgical management of pheochromocytoma. Specific mutations of...
متن کاملCharacteristic genomic imbalances in pediatric pheochromocytoma.
Pheochromocytoma (PCC) in children is rare, genetically not well described, and often related to a poor prognosis. We detected genomic imbalances in all 14 tumors from children analyzed by comparative genomic hybridization. A combinatorial loss of chromatin from 3p and 11p was a common feature in 10 of 14 (72%) patients, which was a result of either a loss of a total chromosome 3 and a total ch...
متن کامل