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