Article (Scientific journals)
Ectopic Hormones-Secreting Pheochromocytoma: A Francophone Observational Study
KIRKBY-BOTT, James; BRUNAUD, Laurent; MATHONET, Muriel et al.
2012In World Journal of Surgery, 36, p. 1382-1388
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Abstract :
[en] 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.
Disciplines :
Surgery
Author, co-author :
KIRKBY-BOTT, James
BRUNAUD, Laurent
MATHONET, Muriel
HAMOIR, Etienne ;  Centre Hospitalier Universitaire de Liège - CHU > Chirurgie abdominale- endocrinienne et de transplantation
KRAIMPS, Jean-Louis
TRESALLET, Christophe
AMAR, Laurence
RAULT, Alexandre
HENRY, Jean-François
CARNAILLE, Bruno
Language :
English
Title :
Ectopic Hormones-Secreting Pheochromocytoma: A Francophone Observational Study
Publication date :
2012
Journal title :
World Journal of Surgery
ISSN :
0364-2313
eISSN :
1432-2323
Publisher :
Springer Verlag, New York, United States - New York
Volume :
36
Pages :
1382-1388
Peer reviewed :
Peer Reviewed verified by ORBi
Available on ORBi :
since 10 January 2013

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