Familial Pheochromocytoma: A Report of 2 Cases in a Kindred

Abstract

A kindred with 2 current cases of pheochromocytoma is reported. The proband had classical features of adrenal medullary hyperfunction, in addition to Raynaud's phenomenon. After surgical removal of the right adrenal gland, containing a pheochromocytoma and a small paraganglioma, the patient was free of symptoms. The 12-year-old son of the proband was discovered to have relatively asymptomatic sustained hypertension on routine examination. Biochemical and radiological tests confirmed the diagnosis of a left adrenal pheochromocytoma, which was removed successfully.

Pheochromocytoma of the Urinary Bladder - a Case Report and Review of the Literature

“…In addition, a condition was found in our patient which resembled Raynaud's syndrome with a feeling of coldness and pale red marbling of the fingers shortly after micturition, which has rarely been reported in the literature, together with pheochromocytoma [4,29]. The pathophysiological effects of the excess hormone in pheochromocytoma of extra-adrenal location arc pre dominantly caused by epinephrine and to a lesser degree by norepinephrine.…”

“…vesical pheochromocytoma, in most cases an angiogra phy of the pelvis was performed. But even under premed ication of a-and (3-receptor blockers, instrumentation may lead to dangerous crises of blood pressure [26,29]. Being noninvasive methods, CT and sonography are of special importance [2,7,[32][33][34], Till now, a CT has been performed in 3 patients with pheochromocytoma of the urinary bladder [12,27,28], In 1 case [28] the tumor escaped the computed tomographical demonstration, while cystoscopically, a tumor measuring about 1.5 cm in diameter could be localized in the vault of the bladder.…”

“…Angiography, CT, sonography and histology do not give evidence as to the malignity of a pheochromocytoma. Only the recurring course and the localization in organs like lymph nodes, liver, lungs or bones, in which a pheochromocytoma normally does not develop [5,13,29,[35][36][37], points to a malign tumor which is to be expected in about 10% of cases. As a ther apeutic consequence, the pheochromocytoma of the uri nary bladder has to be resected in the sound tissue by partial cystectomy, and the organs of the pelvis have to be inspected for possible multiple tumors and involvement of the lymph nodes respectively.…”

Klinik, Diagnose und Therapie des Phäochromozytoms

“…Das Phäochromozytom tritt sporadisch auf; in 5 bis 10% der Fälle, besonders bei solchen mit biadrenalem oder ektopischem Sitz, handelt es sichjedoch um ein familiäres, autosomal dominant vererbtes Leiden (22,35,39,40…”