Journal Article

Malignant Pheochromocytoma and Paraganglioma: 272 Patients Over 55 Years

Iñiguez-Ariza, Nicole M, Bancos, Cristian, Young, William F, Hamidi, Oksana, Kittah, Nana Esi, Tamhane, Shrikant, Bancos, Irina, Gruber, Lucinda

2017

Overview

ContextMalignant pheochromocytoma (PHEO) and paraganglioma (PGL) are rare and knowledge of the natural history is limited.ObjectiveWe aimed to describe baseline characteristics and outcomes of patients with malignant PHEO and PGL (PPGL) and to identify predictors of shorter survival.DesignRetrospective review of patients with malignant PPGL evaluated from 1960 to 2016.SettingReferral center.PatientsThe group comprised 272 patients.Main Outcome MeasuresBaseline description, survival outcomes, and predictors of shorter survival were evaluated in patients with rapidly progressive (n = 29) and indolent disease (n = 188).ResultsMalignant PPGL was diagnosed at a median age of 39 years (range, 7 to 83 years), with synchronous metastases in 96 (35%) patients. In 176 (65%) patients, metastases developed at a median of 5.5 years (range, 0.3 to 53.4 years) from the initial diagnosis. Median follow-up was 8.2 years (range, 0.01 to 54.1 years). Median overall and disease-specific survivals were 24.6 and 33.7 years, respectively. Shorter survival correlated with male sex (P = 0.014), older age at the time of primary tumor (P = 0.0011), synchronous metastases (P < 0.0001), larger primary tumor size (P = 0.0039), elevated dopamine (P = 0.0195), and not undergoing primary tumor resection (P < 0.0001). There was no difference in the type of primary tumor or presence of SDHB mutation.ConclusionsThe clinical course of patients with malignant PPGL is remarkably variable. Rapid disease progression is associated with male sex, older age at diagnosis, synchronous metastases, larger tumor size, elevated dopamine, and not undergoing resection of primary tumor. An individualized approach to patients with metastatic PPGL is warranted.We studied patients with malignant pheochromocytoma and paraganglioma. Overall and disease-specific survivals were 24.6 and 33.7 years, respectively. Patients had a markedly variable clinical course.

• Publisher: Endocrine Society,Copyright Oxford University Press,Oxford University Press
• Subject: Adolescent / Adrenal Gland Neoplasms - mortality / Adrenal Gland Neoplasms - pathology / Adrenal Gland Neoplasms - surgery / Adrenalectomy - methods / Adrenalectomy - mortality / Adult / Age Factors / Aged / Cause of Death / Child / Clinical s / Databases, Factual / Diagnosis / Disease-Free Survival / Dopamine / Female / Geriatrics / Humans / Incidence / Logistic Models / Male / Metastases / Metastasis / Middle Aged / Multivariate Analysis / Neuroendocrine tumors / Paraganglioma / Paraganglioma - mortality / Paraganglioma - pathology / Paraganglioma - surgery / Pheochromocytoma / Pheochromocytoma - mortality / Pheochromocytoma - pathology / Pheochromocytoma - surgery / Predictive Value of Tests / Prognosis / Retrospective Studies / Risk Assessment / Sex Factors / Statistics, Nonparametric / Survival / Survival Analysis / Tumors / Young Adult