Yonsei Med J.  2011 Jan;52(1):45-50. 10.3349/ymj.2011.52.1.45.

Clinical Experiences of Pheochromocytoma in Korea

Affiliations
  • 1Department of Urology, Urological Science Institute, Yonsei University College of Medicine, Seoul, Korea. youngd74@yuhs.ac
  • 2Department of Urology, Inje University College of Medicine, Busan, Korea.
  • 3Department of Urology, Chungbuk National University College of Medicine, Cheongju, Korea.

Abstract

PURPOSE
We report herein 119 patients with pheochromocytoma at our institute over the last 23 years.
MATERIALS AND METHODS
Between 1986 and 2009, 119 patients were diagnosed with pheochromocytoma at our institute. We reviewed the medical records of these patients.
RESULTS
Of 119 patients, 45 were male and 74 were female, and mean age was 43.83 +/- 13.49 years. Forty-three patients (36.1%) were diagnosed incidentally, and 8 patients (6.7%) were found to have familial pheochromocytoma. The mean dimension of the tumors was 5.89 +/- 3.18 cm. 4 patients had bilateral tumors; three of these patients were found to have familial pheochromocytoma and 1 patient was diagnosed with malignant pheochromocytoma. A total of eight patients (6.7%) were found to have malignant pheochromocytoma. In 1 patient, metastasis to a lymph node was found at the time of diagnosis. Metastases were found at a mean of 49 +/- 25.83 (6-75) months after surgery in the other seven patients. 6 patients died of malignant pheochromocytoma at a mean of 31 +/- 28.71 months (1-81) after diagnosis, and the other 2 patients survived for 15 and 24 months, respectively.
CONCLUSION
Approximately 35% of patients with pheochromocytoma are diagnosed incidentally, and the number of detected cases is increasing. Although familial pheochromocytoma was found only in 6.7% of the patients, genetic testing should be considered in all patients, especially in patients with a family history, young age, or multifocal, bilateral, extra-adrenal, or malignant tumors. Given that malignant pheochromocytomas are frequently diagnosed during the follow-up period, long-term follow-up is necessary to confirm the absence of recurrence or metastasis.

Keyword

Pheochromocytoma; incidentaloma; adrenal tumor

MeSH Terms

Adolescent
Adrenal Gland Neoplasms/diagnosis/pathology
Adult
Aged
Child
Female
Humans
Male
Middle Aged
Pheochromocytoma/*diagnosis/pathology
Young Adult

Cited by  1 articles

Genetic Analysis and Clinical Characteristics of Hereditary Pheochromocytoma and Paraganglioma Syndrome in Korean Population
Heewon Choi, Kyoung Jin Kim, Namki Hong, Saeam Shin, Jong-Rak Choi, Sang Wook Kang, Seung Tae Lee, Yumie Rhee
Endocrinol Metab. 2020;35(4):858-872.    doi: 10.3803/EnM.2020.683.


Reference

1. Adler JT, Meyer-Rochow GY, Chen H, Benn DE, Robinson BG, Sippel RS, et al. Pheochromocytoma: current approaches and future directions. Oncologist. 2008. 13:779–793.
Article
2. Kasperlik-Zaluska AA, Roslonowska E, Slowinska-Srzednicka J, Otto M, Cichocki A, Cwikla J, et al. 1,111 patients with adrenal incidentalomas observed at a single endocrinological center: incidence of chromaffin tumors. Ann N Y Acad Sci. 2006. 1073:38–46.
Article
3. Sidhu S, Bambach C, Pillinger S, Reeve T, Stokes G, Robinson B, et al. Changing pattern of adrenalectomy at a tertiary referral centre 1970-2000. ANZ J Surg. 2002. 72:463–466.
Article
4. Goldstein RE, O'Neill JA Jr, Holcomb GW 3rd, Morgan WM 3rd, Neblett WW 3rd, Oates JA, et al. Clinical experience over 48 years with pheochromocytoma. Ann Surg. 1999. 229:755–764.
Article
5. Mannelli M, Ianni L, Cilotti A, Conti A. Pheochromocytoma in Italy: a multicentric retrospective study. Eur J Endocrinol. 1999. 141:619–624.
Article
6. Lee YS, Oh KH. Clinical evaluation of 12 cases of pheochromocytoma. Korean J Urol. 1993. 34:619–625.
7. Pacak K, Linehan WM, Eisenhofer G, Walther MM, Goldstein DS. Recent advances in genetics, diagnosis, localization, and treatment of pheochromocytoma. Ann Intern Med. 2001. 134:315–329.
Article
8. Kopetschke R, Slisko M, Kilisli A, Tuschy U, Wallaschofski H, Fassnacht M, et al. Frequent incidental discovery of phaeochromocytoma: data from a German cohort of 201 phaeochromocytoma. Eur J Endocrinol. 2009. 161:355–361.
Article
9. La Batide-Alanore A, Chatellier G, Plouin PF. Diabetes as a marker of pheochromocytoma in hypertensive patients. J Hypertens. 2003. 21:1703–1707.
Article
10. Pruszczyk P, Januszewicz W, Feltynowski T, Chodakowska J, Wocial B, Pachocki R, et al. Long term follow-up after surgical removal of pheochromocytoma--observations in 61 patients. Clin Exp Hypertens A. 1991. 13:1179–1194.
Article
11. Favia G, Lumachi F, Polistina F, D'Amico DF. Pheochromocytoma, a rare cause of hypertension: long-term follow-up of 55 surgically treated patients. World J Surg. 1998. 22:689–693.
Article
12. Lucon A, Pereira MA, Mendonsa BB, Halpern A, Wajchenbeg BL, Arap S. Pheochromocytoma: study of 50 cases. J Urol. 1997. 157:1208–1212.
Article
13. Modlin IM, Farndon JR, Shepherd A, Johnston ID, Kennedy TL, Montgomery DA, et al. Phaeochromocytomas in 72 patients: clinical and diagnostic features, treatment and long term results. Br J Surg. 1979. 66:456–465.
Article
14. Lenders JW, Eisenhofer G, Mannelli M, Pacak K. Phaeochromocytoma. Lancet. 2005. 366:665–675.
Article
15. McNeil AR, Blok BH, Koelmeyer TD, Burke MP, Hilton JM. Phaeochromocytomas discovered during coronial autopsies in Sydney, Melbourne and Auckland. Aust N Z J Med. 2000. 30:648–652.
Article
16. Lo CY, Lam KY, Wat MS, Lam KS. Adrenal pheochromocytoma remains a frequently overlooked diagnosis. Am J Surg. 2000. 179:212–215.
Article
17. Chrisoulidou A, Kaltsas G, Ilias I, Grossman AB. The diagnosis and management of malignant phaeochromocytoma and paraganglioma. Endocr Relat Cancer. 2007. 14:569–585.
Article
18. Gimenez-Roqueplo AP, Favier J, Rustin P, Rieubland C, Crespin M, Nau V, et al. Mutations in the SDHB gene are associated with extra-adrenal and/or malignant phaeochromocytomas. Cancer Res. 2003. 63:5615–5621.
19. Neumann HP, Pawlu C, Peczkowska M, Bausch B, McWhinney SR, Muresan M, et al. Distinct clinical features of paraganglioma syndromes associated with SDHB and SDHD gene mutations. JAMA. 2004. 292:943–951.
Article
20. Brouwers FM, Eisenhofer G, Tao JJ, Kant JA, Adams KT, Linehan WM, et al. High frequency of SDHB germline mutations in patients with malignant catecholamine-producing paragangliomas: implications for genetic testing. J Clin Endocrinol Metab. 2006. 91:4505–4509.
Article
21. Neumann HP, Bausch B, McWhinney SR, Bender BU, Gimm O, Franke G, et al. Germ-line mutations in nonsyndromic pheochromocytoma. N Engl J Med. 2002. 346:1459–1466.
Article
22. Pigny P, Cardot-Bauters C, Do Cao C, Vantyghem MC, Carnaille B, Pattou F, et al. Should genetic testing be performed in each patient with sporadic pheochromocytoma at presentation? Eur J Endocrinol. 2009. 160:227–231.
Article
23. Lenders JW, Pacak K, Walther MM, Linehan WM, Mannelli M, Friberg P, et al. Biochemical diagnosis of pheochromocytoma: which test is best? JAMA. 2002. 287:1427–1434.
24. Sawka AM, Jaeschke R, Singh RJ, Young WF Jr. A comparison of biochemical tests for pheochromocytoma: measurement of fractionated plasma metanephrines compared with the combination of 24-hour urinary metanephrines and catecholamines. J Clin Endocrinol Metab. 2003. 88:553–558.
Article
25. Sawka AM, Prebtani AP, Thabane L, Gafni A, Levine M, Young WF Jr. A systematic review of the literature examining the diagnostic efficacy of measurement of fractionated plasma free metanephrines in the biochemical diagnosis of pheochromocytoma. BMC Endocr Disord. 2004. 4:2.
Article
26. Park K, Lee JL, Ahn H, Koh JM, Park I, Choi JS, et al. Sunitinib, a novel therapy for anthracycline- and cisplatin-refractory malignant pheochromocytoma. Jpn J Clin Oncol. 2009. 39:327–331.
Article
27. Amar L, Servais A, Gimenez-Roqueplo AP, Zinzindohoue F, Chatellier G, Plouin P. Year of diagnosis, features at presentation, and risk of recurrence in patients with pheochromocytoma or secreting paraganglioma. J Clin Endocrinol Metab. 2005. 90:2110–2116.
Article
Full Text Links
  • YMJ
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr