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 |
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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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