Clinical risk factors for malignancy and overall survival in patients with pheochromocytomas and sympathetic paragangliomas: Primary tumor size and primary tumor location as prognostic indicators

Montserrat Ayala-Ramirez, Lei Feng, Marcella M. Johnson, Shamim Ejaz, Mouhammed Amir Habra, Thereasa Rich, Naifa Busaidy, Gilbert J. Cote, Nancy Perrier, Alexandria Phan, Shreyaskumar Patel, Steven Waguespack, Camilo Jimenez

Research output: Contribution to journalArticle

208 Scopus citations

Abstract

Context: Pheochromocytomas and sympathetic paragangliomas are rare neuroendocrine tumors for which no precise histological or molecular markers have been identified to differentiate benign from malignant tumors. Objective: The aim was to determine whether primary tumor location and size are associated with malignancy and decreased survival. Design and Setting: We performed a retrospective chart review of patients with either pheochromocytoma or sympathetic paraganglioma. Patients: The study group comprised 371 patients. Main Outcome Measures: Overall survival and disease-specific survival were analyzed according to tumor size and location. Results: Sixty percent of patients with sympathetic paragangliomas and 25% of patients with pheochromocytomas had metastatic disease. Metastasis was more commonly associated with primary tumors located in the mediastinum (69%) and the infradiaphragmatic paraaortic area, including the organ of Zuckerkandl (66%). The primary tumor was larger in patients with metastases than in patients without metastatic disease (P < 0.0001). Patients with sympathetic paragangliomas had a shorter overall survival than patients with pheochromocytomas (P < 0.0001); increased tumor size was associated with shorter overall survival (P < 0.001). Patients with sympathetic paragangliomas were twice as likely to die of disease than patients with pheochromocytomas (hazard ratio = 1.93; 95% confidence interval = 1.20-3.12; P = 0.007). As per multivariate analysis, the location of the primary tumor was a stronger predictor of metastases than was the size of the primary tumor. Conclusions: The size and location of the primary tumor were significant clinical risk factors for metastasis and decreased overall survival duration. These findings delineate the follow-up and treatment for these tumors.

Original languageEnglish (US)
Pages (from-to)717-725
Number of pages9
JournalJournal of Clinical Endocrinology and Metabolism
Volume96
Issue number3
DOIs
StatePublished - Mar 2011

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Biochemistry
  • Endocrinology
  • Clinical Biochemistry
  • Biochemistry, medical

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