Malignant Pheochromocytoma and Paraganglioma: 272 Patients Over 55 Years

Autor: Oksana Hamidi, Nicole M. Iñiguez-Ariza, Shrikant Tamhane, Nana Esi Kittah, Irina Bancos, William F. Young, Lucinda Gruber, Cristian Bancos
Rok vydání: 2017
Předmět:
Male
Databases
Factual
Endocrinology
Diabetes and Metabolism
Clinical Biochemistry
Adrenal Gland Neoplasms
Biochemistry
0302 clinical medicine
Endocrinology
Paraganglioma
Cause of Death
Young adult
Child
Cause of death
Incidence
Age Factors
Adrenalectomy
Middle Aged
Prognosis
Primary tumor
030220 oncology & carcinogenesis
Predictive value of tests
Female
Adult
medicine.medical_specialty
Adolescent
030209 endocrinology & metabolism
Context (language use)
Pheochromocytoma
Risk Assessment
Disease-Free Survival
Statistics
Nonparametric
Young Adult
03 medical and health sciences
Sex Factors
Predictive Value of Tests
Internal medicine
medicine
Humans
Clinical Research Articles
Survival analysis
Aged
Retrospective Studies
business.industry
Biochemistry (medical)
Retrospective cohort study
medicine.disease
Survival Analysis
Logistic Models
Multivariate Analysis
business
Zdroj: The Journal of Clinical Endocrinology & Metabolism. 102:3296-3305
ISSN: 1945-7197
0021-972X
Popis: Context Malignant pheochromocytoma (PHEO) and paraganglioma (PGL) are rare and knowledge of the natural history is limited. Objective We aimed to describe baseline characteristics and outcomes of patients with malignant PHEO and PGL (PPGL) and to identify predictors of shorter survival. Design Retrospective review of patients with malignant PPGL evaluated from 1960 to 2016. Setting Referral center. Patients The group comprised 272 patients. Main Outcome Measures Baseline description, survival outcomes, and predictors of shorter survival were evaluated in patients with rapidly progressive (n = 29) and indolent disease (n = 188). Results Malignant 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. Conclusions The 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.
Databáze: OpenAIRE
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