Clinical course and prognostic factors in patients with malignant pheochromocytoma and paraganglioma: A single institution experience. Issue 8 (14th October 2015)

• Record Type: Journal Article
• Authors: Choi, Yun Mi; Sung, Tae‐Yon; Kim, Won Gu; Lee, Jong Jin; Ryu, Jin‐Sook; Kim, Tae Yong; Kim, Won Bae; Hong, Suck Joon; Song, Dong Eun; Shong, Young Kee
• Is Part Of: Journal of surgical oncology. Volume 112:Issue 8(2015:Dec. 15)
• Page Start: 815
• Page End: 821
• Publication Date: 2015-10-14
• Subjects: neoplasms -- pheochromocytoma -- paraganglioma -- distant metastasis; Cancer -- Surgery -- Periodicals; Neoplasms -- Periodicals; 616
• DOI: 10.1002/jso.24063
• Languages: English
• ISSNs: 0022-4790

Abstract

Abstract : Objectives: Malignant pheochromocytoma (PCC)/paraganglioma (PG) are rare neuroendocrine malignancies, and their clinical courses and prognoses are not well understood. This study aimed to evaluate prognostic factors associated with the survival of malignant PCC/PG. Methods: This retrospective study reviewed 299 patients with PCC and 46 with PG treated between 1997 and 2013 at our single tertiary hospital. Malignant PCC/PG was defined as the presence of distant metastasis or recurrence at sites where neuroendocrine tissue is normally not present. Results: Twenty‐seven patients (9%) were confirmed with malignant PCC and six patients (13%) with malignant PG. Twenty‐seven patients (82%) had distant metastases, nine patients (27%) presented with a metastasis at the initial diagnosis, whilst 24 patients (73%) were diagnosed with malignant PCC/PG during follow‐up (median, 4.3 year). The median survival was 7.2 years, and the 5 year survival rate was 75.4%. Older age (>45 years), larger tumor size (>6 cm), synchronous metastasis, and absence of surgical excision were associated with poor survival by univariate analysis. By multivariate analysis, older age (HR = 4.3, P = 0.02) and synchronous metastasis (HR = 4.3, P = 0.01) were significantly associated with a poor prognosis. Conclusions: Patients with malignant PCC/PG have diverse clinical courses. Poor survival was independently associated with older age and synchronous metastasis. J. Surg. Oncol. 2015;112:815–821 . © 2015 Wiley Periodicals, Inc.