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The size of the primary tumor and age at initial diagnosis are independent predictors of the metastatic behavior and survival of patients with SDHB-related pheochromocytoma and paraganglioma: a retrospective cohort study.

Authors
  • Schovanek, Jan
  • Martucci, Victoria
  • Wesley, Robert
  • Fojo, Tito
  • Del Rivero, Jaydira
  • Huynh, Thanh
  • Adams, Karen
  • Kebebew, Electron
  • Frysak, Zdenek
  • Stratakis, Constantine A
  • Pacak, Karel
Type
Published Article
Journal
BMC Cancer
Publisher
Springer (Biomed Central Ltd.)
Publication Date
Jan 01, 2014
Volume
14
Pages
523–523
Identifiers
DOI: 10.1186/1471-2407-14-523
PMID: 25048685
Source
Medline
License
Unknown

Abstract

Receiver operating characteristic curves established 4.5 cm as the best value to dichotomize the primary SDHB-related PHEO/PGL in order to evaluate the development of metastatic disease and 5.5 cm as the best value for survival prediction. Subsequently, the size of the primary tumor was found as an age-independent predictor of patient survival and metastases development in PGL. In both PHEO and PGL, age at diagnosis was found to be a size-independent predictor of patient survival. No significant difference was found in metastases development or patient survival between males and females or among specific SDHB mutations. This data further extends and supports previous recommendations that carriers with SDHB mutations must undergo early and regular evaluations to detect PHEO/PGL in order to achieve the best clinical outcome.

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