Abstract
Background. Hereditary pheochromocytoma (HP) is characterized by early onset, bilateral adrenal
involvement, low malignancy rate, and genetic linkage with certain familial syndromes.
This retrospective review is intended to show the high yield of surveillance, predictable
bilaterality, and the challenge of cortex-sparing adrenalectomy. Methods. From 1964 to 1999, 32 patients with HP were treated at a single institution and followed
for a mean of 7 years. There were 15 cases of multiple endocrine neoplasia type 2A
(MEN 2A), 12 cases of von Hippel-Lindau (VHL) disease, 3 cases of von Recklinghausen's
disease (VRD), and 2 cases of familial pheochromocytoma. Twenty-four of 32 patients
underwent bilateral adrenalectomy (9 metachronous). Subtotal resection with orthotopic
cortex preservation was performed in 5 patients, and heterotopic autografting was
performed in 14 patients. Results. Pheochromocytoma was the first manifestation in 50% of patients with VHL disease
and in 27% of patients with MEN 2A. Surveillance uncovered medullary thyroid cancer
in 5 of 15 patients with MEN 2A and hemangioblastomas, renal cell carcinoma, and islet
cell tumors in 7 of 15 patients with VHL disease and VRD. HP was bilateral in 24 of
32 patients (14/15 in patients with MEN 2A, 7/12 in patients with VHL disease, 2/3
in patients with VRD, and 1/2 in patients with familial pheochromocytoma). In 9 cases
of metachronous adrenalectomy, the mean interval was 67 months (range, 9-156 months).
Three of 5 patients who underwent orthotopic preservation of the adrenal cortex experienced
recurrence compared with 0 of 14 patients with heterotopic autotransplantation of
cortical tissue. Conclusions. Pheochromocytoma frequently heralds coexisting silent VHL disease or MEN-2, mandating
surveillance for inherited associations. The long interval of metachronous pheochromocytoma
argues against prophylactic removal of the contralateral “normal” adrenal gland. Total
adrenalectomy and heterotopic autotransplantation of medulla-free cortex may diminish
the need for lifelong steroid substitution and eliminates recurrence. (Surgery 2000;128:1007-12.)
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Article info
Footnotes
*Reprint requests: William B. Inabnet, MD, 5 East 98th St, Box 1259, New York, NY 10029.
**Surgery 2000;128:1007-12.
Identification
Copyright
© 2000 Mosby, Inc. Published by Elsevier Inc. All rights reserved.
