Malignant pheochromocytoma and paraganglioma: A population level analysis of long‐term survival over two decades12. Issue 6 (11th December 2012)

• Record Type: Journal Article
• Authors: Goffredo, Paolo; Sosa, Julie A.; Roman, Sanziana A.
• Is Part Of: Journal of surgical oncology. Volume 107:Issue 6(2013:May 01)
• Page Start: 659
• Page End: 664
• Publication Date: 2012-12-11
• Subjects: Cancer -- Surgery -- Periodicals; Neoplasms -- Periodicals; 616
• DOI: 10.1002/jso.23297
• Languages: English
• ISSNs: 0022-4790

Abstract

<abstract abstract-type="main" xml:lang="en"> <title>Abstract</title> <sec id="abs1-1" sec-type="section"> <title>Background and Objectives</title> <p>Pheochromocytoma (PHEO) and paraganglioma (PGL) are rare tumors. Aims of this study were to describe and to compare demographic, clinical, pathologic, and survival characteristics of malignant PHEO and PGL.</p> </sec> <sec id="abs1-2" sec-type="section"> <title>Methods</title> <p>Patients were identified in SEER, 1988–2009. Analyses included chi‐square, ANOVA, Kaplan–Meier, and Cox proportional hazard regression.</p> </sec> <sec id="abs1-3" sec-type="section"> <title>Results</title> <p>Gender distribution and mean age were similar for PHEO and PGL. Surgery was performed in 74.3% of PHEO and 78.9% of PGL; external beam radiation was administered in 8.0% of PHEO and 28.1% of PGL (<italic>P</italic> < 0.001). Compared to PGL, PHEO were larger (mean size 7.7 vs. 4.5 cm PGL, <italic>P</italic> = 0.001) and more were SEER‐staged as localized (17.3% vs. 49.6%, respectively, <italic>P</italic> < 0.001). PGLs were more often located in the trunk than in the head/neck (53.8% vs. 38.0%, <italic>P</italic> < 0.001). PHEO had lower overall and disease‐specific survival than PGL (54.0% and 73.5% vs. 73.3% and 80.5% for PGL, respectively, <italic>P</italic> < 0.001 and <italic>P</italic> = 0.118). Independent factors associated with mortality for PHEO included not undergoing surgery and metastases at diagnosis; for PGL, these were age 61–75 years, size ≥5 cm, and presenting with metastases.</p> </sec> <sec id="abs1-4" sec-type="section"> <title>Conclusions</title> <p>Malignant PHEO has a more aggressive course than malignant PGL; long‐term survival has not improved over the last two decades. Multi‐institutional efforts should be pursued to seek novel treatments. J. Surg. Oncol. 2013;107:659–664. © 2012 Wiley Periodicals, Inc.</p> </sec> </abstract>