Bilateral Pheochromocytomas: Clinical Presentation and Surgical Treatment of 94 Patients over 64 Years

Presentation Number: OR04-2
Date of Presentation: April 1st, 2017

Nana Esi Nkuma Kittah*, Irina Bancos, Oksana Hamidi, Shrikant Tamhane, Nicole Iniguez Ariza, Dusica Babovic-Vuksanovic, Cristian Bancos, William F Young Jr. and Dana Erickson
Mayo Clinic, Rochester, MN

Abstract

Background:

Pheochromocytomas (PHEOs) are neuroendocrine tumors arising from the adrenal medulla. They are frequently unilateral and sporadic. Bilateral PHEOs are typically associated with hereditary syndromes.

Objectives:

Our aim was to describe the clinical presentation, genetics, treatment and outcomes of patients with bilateral PHEOs.

Methods:

Medical records of patients with bilateral PHEOs at Mayo Clinic, Rochester, MN from 1951to 2015 were retrospectively reviewed. Diagnosis of PHEO was based on biochemical, imaging, or histological findings.

Results:

Out of 1161 patients with PHEO, 94 (7.9%) patients (48 women, 51%) were diagnosed with bilateral PHEOs. Seventy-six (81%) patients presented with synchronous tumors, while 18 (19%) patients developed metachronous tumors. The median age at the time of surgery for the first PHEO was 31 yrs (range, 4-70). In metachronous tumors, the second PHEO was detected at a median time of 4.5 yrs after the initial surgery (range, 1-38). Median tumor size of the first PHEO was 31 mm (range, 6-120). The second PHEO measured 23 mm (range, 4-87). In 32 (34%) patients the tumors were multicentric. A genetic syndrome was documented in 75 (80%) patients and in 16 (17%) patients very limited or no genetic testing was performed. Of the patients who had a detected genetic syndrome, 41 (43.6%) had MEN 2A, 18 (19%) VHL, 8 (8.5%) MEN 2B, and 8 (8.5%) NF1.

PHEO was discovered because of symptoms of catecholamine excess in 47 (50%) patients, genetic case detection testing in 26 (28%), an incidentaloma imaging finding in 17 (18%), mass effect symptoms in 1 (1%), and in 3 (3%) patients the method of discovery was not known. Pre-operative biochemical information was available for 69 patients and showed catecholamine excess in 67 (97%) patients. Fifty (53%) patients underwent adrenalectomy by the open approach; laparoscopic in 22 (23%), and unknown type surgical approach in 21 (22%) patients who had surgery performed elsewhere. Sixteen of 72 (22%) patients had cortical sparing surgery and of these 7 (44%) required permanent glucocorticoid replacement. One patient was not operated.

Patients were followed for a median of 10 yrs (range, 0-64). Recurrent PHEO within the adrenal gland occurred in 8 (8.5%) patients. Malignant PHEO occurred in 8 (8.5%) patients. Of 20 (23%) patients who died during follow-up, 2 (10%) died from PHEO.

Conclusion:

Bilateral PHEO was diagnosed in 94 (7.9%) of 1161 patients with PHEO over 64 years. MEN 2A and VHL were the two most common syndromes associated with bilateral PHEO. At the time of diagnosis, most bilateral PHEOs are likely to be synchronous. However, in metachronous PHEOs the second tumor may be detected anywhere from 1 to 38 yrs (median, 4.5) after the initial tumor resection.

 

Nothing to Disclose: NENK, IB, OH, ST, NI, DB, CB, WFY Jr., DE