Clinical Experiences of Pheochromocytoma in Korea

다국어 초록 (Multilingual 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.

참고문헌 (Reference)

1 Amar L, "Year of diagnosis, features at presentation, and risk of recurrence in patients with pheochromocytoma or secreting paraganglioma" 90 : 2110-2116, 2005

2 Chrisoulidou A, "The diagnosis and management of malignant phaeochromocytoma and paraganglioma" 14 : 569-585, 2007

3 Park K, "Sunitinib, a Novel Therapy for Anthracycline- and Cisplatin-refractory Malignant Pheochromocytoma" OXFORD UNIV PRESS 39 (39): 327-331, 2009

4 Pigny P, "Should genetic testing be performed in each patient with sporadic pheochromocytoma at presentation" 160 : 227-231, 2009

5 Pacak K, "Recent advances in genetics, diagnosis, localization, and treatment of pheochromocytoma" 134 : 315-329, 2001

6 Lucon A, "Pheochromocytoma: study of 50 cases" 157 : 1208-1212, 1997

7 Adler JT, "Pheochromocytoma: current approaches and future directions" 13 : 779-793, 2008

8 Favia G, "Pheochromocytoma, a rare cause of hypertension: long-term follow-up of 55 surgically treated patients" 22 : 689-693, 1998

9 Mannelli M, "Pheochromocytoma in Italy: a multicentric retrospective study" 141 : 619-624, 1999

10 Modlin IM, "Phaeochromocytomas in 72 patients: clinical and diagnostic features, treatment and long term results" 66 : 456-465, 1979

11 McNeil AR, "Phaeochromocytomas discovered during coronial autopsies in Sydney, Melbourne and Auckland" 30 : 648-652, 2000

12 Lenders JW, "Phaeochromocytoma" 366 : 665-675, 2005

13 Gimenez-Roqueplo AP, "Mutations in the SDHB gene are associated with extra-adrenal and/or malignant phaeochromocytomas" 63 : 5615-5621, 2003

14 Pruszczyk P, "Long term follow-up after surgical removal of pheochromocytoma--observations in 61 patients" 13 : 1179-1194, 1991

15 Brouwers FM, "High frequency of SDHB germline mutations in patients with malignant catecholamine-producing paragangliomas: implications for genetic testing" 91 : 4505-4509, 2006

16 Neumann HP, "Germ-line mutations in nonsyndromic pheochromocytoma" 346 : 1459-1466, 2002

17 Kopetschke R, "Frequent incidental discovery of phaeochromocytoma: data from a German cohort of 201 phaeochromocytoma" 161 : 355-361, 2009

18 Neumann HP, "Distinct clinical features of paraganglioma syndromes associated with SDHB and SDHD gene mutations" 292 : 943-951, 2004

19 La Batide-Alanore A, "Diabetes as a marker of pheochromocytoma in hypertensive patients" 21 : 1703-1707, 2003

20 Goldstein RE, "Clinical experience over 48 years with pheochromocytoma" 229 : 755-764, 1999

21 Lee YS, "Clinical evaluation of 12 cases of pheochromocytoma" 34 : 619-625, 1993

22 Sidhu S, "Changing pattern of adrenalectomy at a tertiary referral centre 1970-2000" 72 : 463-466, 2002

23 Lenders JW, "Biochemical diagnosis of pheochromocytoma: which test is best" 287 : 1427-1434, 2002

24 Lo CY, "Adrenal pheochromocytoma remains a frequently overlooked diagnosis" 179 : 212-215, 2000

25 Sawka AM, "A systematic review of the literature examining the diagnostic efficacy of measurement of fractionated plasma free metanephrines in the biochemical diagnosis of pheochromocytoma" 4 : 2-, 2004

26 Sawka AM, "A comparison of biochemical tests for pheochromocytoma: measurement of fractionated plasma metanephrines compared with the combination of 24-hour urinary metanephrines and catecholamines" 88 : 553-558, 2003

27 Kasperlik-Zaluska AA, "1,111 patients with adrenal incidentalomas observed at a single endocrinological center: incidence of chromaffin tumors" 1073 : 38-46, 2006