Introduction: We present the case of coincidence of pheochromocytoma and aderenocortical carcinoma at the same adrenal gland in patient with neurofibromatosis. That kind of tumors coincidence origin from different blastodermic layers is extremely rare.
It is broadly known that pheochromocytoma is rare neoplasm, occurs in 0.1% of hypertensive individuals. Pheochromocytoma may occur sporadically or in certain familial syndromes, including multiple endocrine neoplasia (MEN) 2A and 2B, neurofibromatosis, and von HippelLindau (VHL) disease. Sporadic pheochromocytomas are usually unilateral, however in 10% of familiar tumors are located bilateral. The clinical manifestations of a pheochromocytoma result from excessive catecholamine secretion by the tumor.
Adrenocortical carcinoma is also very rare tumor which reveals in 12 cases per million. Etiology is still non-elucidated, but the role of genetic and environmental factors are mainly considerate. Most of the tumors are functional and usually its first manifestation is Cushing syndrome with virylization.
Case report: The female 45-year-old patient with neurofibromatosis and bilateral tumors of adrenal glands was admitted to Endocrinology Department in order to confirm pheochromocytoma. The suspicion resulted from paroxysmal blood hypertension lasting for 1 year and arrhythmia. The hormonal examinations (metanephrine and vanillin-mandelic acid in the urine collection) confirmed pheochromocytoma. Remaining hormonal examinations were normal. After proper medical preparation the right adrenalectomy was performed. The histopatological examination revealed coincidence of pheochromocytoma and adrenocortical cancer at right adrenal gland. Controlled metanephrine urine collection again revealed increased level of normethanefrin and methanefrin. Two weeks after first operation the left adrenalectomy was preformed. Histopatological examination is in progress.
Conclusion: The coincidence of pheochromocytoma and adrenocortical carcinoma is unique. Finding of adrenocortical carcinoma cells in adrenal gland tissue during histopathological exam changes patients prognosis and planned follow up. Patients and patients family members genetic exam should be considerated.
03 - 07 May 2008
European Society of Endocrinology