Malignant pheochromocytoma and paraganglioma: a population level analysis of long-term survival over two decades.


Journal Article

BACKGROUND AND OBJECTIVES: 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. METHODS: Patients were identified in SEER, 1988-2009. Analyses included chi-square, ANOVA, Kaplan-Meier, and Cox proportional hazard regression. RESULTS: 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 (P < 0.001). Compared to PGL, PHEO were larger (mean size 7.7 vs. 4.5 cm PGL, P = 0.001) and more were SEER-staged as localized (17.3% vs. 49.6%, respectively, P < 0.001). PGLs were more often located in the trunk than in the head/neck (53.8% vs. 38.0%, P < 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, P < 0.001 and P = 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. CONCLUSIONS: 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.

Full Text

Cited Authors

  • Goffredo, P; Sosa, JA; Roman, SA

Published Date

  • May 2013

Published In

Volume / Issue

  • 107 / 6

Start / End Page

  • 659 - 664

PubMed ID

  • 23233320

Pubmed Central ID

  • 23233320

Electronic International Standard Serial Number (EISSN)

  • 1096-9098

International Standard Serial Number (ISSN)

  • 0022-4790

Digital Object Identifier (DOI)

  • 10.1002/jso.23297


  • eng