World Journal of Endocrine Surgery

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VOLUME 3 , ISSUE 3 ( September-December, 2011 ) > List of Articles

RESEARCH ARTICLE

Postoperative Outcomes of Familial Adrenal Pheochromocytoma1

Chikara Kagawa, Yatsuka Hibi, Hiroki Uchida, Sumiyo Noda, Ai Idota, Shigenori Sato, Hironori Hayashi, Toyone Kikumori, Tsuneo Imai, Tetsuya Kiuchi

Citation Information : Kagawa C, Hibi Y, Uchida H, Noda S, Idota A, Sato S, Hayashi H, Kikumori T, Imai T, Kiuchi T. Postoperative Outcomes of Familial Adrenal Pheochromocytoma1. World J Endoc Surg 2011; 3 (3):112-115.

DOI: 10.5005/jp-journals-10002-1071

Published Online: 01-12-2011

Copyright Statement:  Copyright © 2011; Jaypee Brothers Medical Publishers (P) Ltd.


Abstract

There are no current guidelines for the management of familial pheochromocytoma (FP). We tried to determine the optimal management of patients with FP. Among 191 patients with pheochromocytoma who underwent surgical resection between 1979 and 2010, there were 18 FP (13 different kindreds; 11 females/7 males; mean age at initial operation: 38.7 years). The 18 FP cases comprised 10 with MEN2A, 2 with MEN2B, 4 with von Hippel-Lindau disease, and 2 with FP only, and all pheochromocytomas were of adrenal origin. The number of probands and family members was 9 and 9 respectively. Mean tumor size was 6.4 cm in diameter. Simultaneous bilateral adrenalectomy was performed in 6 patients, and unilateral adrenalectomy was performed as the initial surgery in 12 patients. A metachronous contralateral adrenalectomy was performed in 3 patients, 90, 236 and 312 months after the primary operation, respectively. None of the patients received partial adrenalectomy. Among another 9 patients with unilateral adrenalectomy, contralateral pheochromocytomas were suspected in 4 cases at the initial operation. However, none of these contralateral lesions developed severe symptoms or tumor enlargement during a median follow-up of 116 months. In the remaining 5 patients, pheochromocytoma did not develop in the contralateral adrenals over a median follow-up of 80.5 months. Bilateral lesions of adrenal pheochromocytoma in familial cases occurred in 78% of cases (14/18); 9 patients (including 4 with contralateral pheochromocytoma) did not require a contralateral adrenalectomy during a median follow-up of 119 months. No patients have suffered from Addisonian crisis. The ipsilateral adrenalectomy and follow-up of contralateral small pheochromocytoma is one of the management options to preserve adrenocortical function in FP patients.


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  1. Recent advances in the genetics of SDHrelated paraganglioma and pheochromocytoma. Fam Cancer 2011;10:355-63.
  2. Genetics of pheochromocytomas and paragangliomas. Best Pract Res Clin Endocrinol Metab 2010;24: 943-56.
  3. Multiple endocrine neoplasia type 2. Best Pract Res Clin Endocrinol Metab 2010;24:371-87.
  4. Surgical management of Cushing's syndrome. Biomed Pharmacother 2000;54 Suppl 1:140s-5s.
  5. Referral from secondary care and to aftercare in a tertiary care university hospital in Japan. BMC Health Serv Res 2006;6:11.
  6. Pheochromocytoma in MEN2A syndrome. Study of 54 patients. World J Surg 2008;32:2520-26.
  7. Corticalsparing adrenalectomy for patients with bilateral pheochromocytoma. Surgery 1996;120:1064-71.
  8. Estimated risk of pheochromocytoma recurrence after adrenal-sparing surgery in patients with multiple endocrine neoplasia type 2A. Arch Surg 2006;141:1199-1205.
  9. Adrenocortical tumors: Recent advances in basic concepts and clinical management. Annals of Internal Medicine 1999;130:759-71.
  10. Iatrogenic pheochromocytomatosis: A previously unreported result of laparoscopic adrenalectomy. Surgery 2001;130:1072-77.
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