References of "HENRY, Jean-François"
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See detailEctopic Hormones-Secreting Pheochromocytoma: A Francophone Observational Study
KIRKBY-BOTT, James; BRUNAUD, Laurent; MATHONET, Muriel et al

in World Journal of Surgery (2012), 36

Background Ectopic hormone-secreting pheochromocytomas <br />are rare; only case reports exist in the literature. This <br />condition has been linked with increased malignancy, <br />familial syndromes ... [more ▼]

Background Ectopic hormone-secreting pheochromocytomas <br />are rare; only case reports exist in the literature. This <br />condition has been linked with increased malignancy, <br />familial syndromes, and ACTH secretion. We wanted to <br />test these hypotheses and shed light on the nature of ectopic <br />hormone-secreting pheochromocytomas. <br />Methods This is a multicenter (francophone) observational <br />study. Inclusion was based upon abnormal preoperative <br />hormone tests in patients with pheochromocytoma <br />that normalized after removal of the tumor. Where <br />possible, immunohistochemistry was performed to confirm <br />that ectopic secretion came from the tumor. <br />Results Sixteen cases were found: nine female and seven <br />male patients. Median age was 50.5 (range 31–89) years. <br />Most presented with hypertension, diabetes, or cushingoid <br />features. Ten patients had specific symptoms from the <br />ectopic hormone secretion. Two had a familial syndrome. <br />Of eight patients with excess cortisol secretion, three died <br />as a result of the tumor resection: two had pheochromocytomas[ <br />15 cm and their associated cortisol hypersecretion <br />complicated their postoperative course. The other died <br />from a torn subhepatic vein. The 13 survivors did not <br />develop any evidence of malignancy during follow-up <br />(median 50 months). Symptoms from the ectopic secretion <br />resolved after removal of the tumor. Immunohistochemistry <br />was performed and was positive in eight tumors: five <br />ACTH, three calcitonins, and one VIP. <br />Conclusions Most pheochromocytomas with ectopic <br />secretion are neither malignant nor familial. Most ectopic <br />hormone-secreting pheochromocytoma cause hypercortisolemia. <br />Patients with a pheochromocytoma should be <br />worked up for ectopic hormones, because removal of the <br />pheochromocytoma resolves those symptoms. Associated <br />cortisol secretion needs careful attention. [less ▲]

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