ArticleThe Journal of clinical endocrinology and metabolism2017
Malignant Pheochromocytoma and Paraganglioma: 272 Patients Over 55 Years.
Article in The Journal of clinical endocrinology and metabolism, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 176 papers, 7 of them syntheses that pooled it.
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
176 citing papers in PubMed, 7 syntheses or guidelines pooled it.
- Japan Endocrine Society Clinical Practice Guideline for the Diagnosis and Management of Pheochromocytoma and Paraganglioma 2025.Endocrine journal · 2026Guideline
- Multidisciplinary practice guidelines for the diagnosis, genetic counseling and treatment of pheochromocytomas and paragangliomas.Clinical & translational oncology : official publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico · 2021Guideline
- Succinate Dehydrogenase Complex Iron Sulfur Subunit B (SDHB) Immunohistochemistry in Pheochromocytoma, Head and Neck Paraganglioma, Thoraco-Abdomino-Pelvic Paragangliomas: Is It a Good Idea to Use in Routine Work?Asian Pacific journal of cancer prevention : APJCP · 2021Pooled it
- The Role for Metyrosine in the Treatment of Patients With Pheochromocytoma and Paraganglioma.The Journal of clinical endocrinology and metabolism · 2021Pooled it
- Maternal and fetal outcomes in phaeochromocytoma and pregnancy: a multicentre retrospective cohort study and systematic review of literature.The lancet. Diabetes & endocrinology · 2021Pooled it
- Predictors of recurrence of pheochromocytoma and paraganglioma: a multicenter study in Piedmont, Italy.Hypertension research : official journal of the Japanese Society of Hypertension · 2020Pooled it
- Genotype-phenotype correlations in pheochromocytoma and paraganglioma: a systematic review and individual patient meta-analysis.Endocrine-related cancer · 2019Pooled it
- Phase II Study ofJournal of clinical oncology : official journal of the American Society of Clinical Oncology · 2025Trial
- Novel alternative tools for metastatic pheochromocytomas/paragangliomas prediction.Journal of endocrinological investigation · 2024Trial
- Genetic and Clinical Profiles of Pheochromocytoma and Paraganglioma: A Single Center Study.Frontiers in endocrinology · 2020Trial
- Efficacy and Safety of High-Specific-ActivityJournal of nuclear medicine : official publication, Society of Nuclear Medicine · 2019Trial
- Neurogenic tumors of the abdomen and pelvis: imaging features, differential diagnosis, and diagnostic pitfalls.Abdominal radiology (New York) · 2026Review
- PPGLomics: an interactive platform for pheochromocytoma and paraganglioma transcriptomics.Endocrine-related cancer · 2026Article
- Clinicopathological Features, Tumor Localization and Treatment Outcomes in Paraganglioma: A Single-Center Medical Oncology Cohort.Diagnostics (Basel, Switzerland) · 2026Article
- Metachronous contralateral pheochromocytoma detected 62 years after initial surgery.JCEM case reports · 2026Article
- Article
- [Diagnostics and treatment of pheochromocytomas and paragangliomas].Chirurgie (Heidelberg, Germany) · 2026Review
- Pilot study: transduction of primary paraganglioma chromaffin tumour cells with inducible c-MYC drives cell proliferation.Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association · 2026Article
- PPGLomics: An Interactive Platform for Pheochromocytoma and Paraganglioma Transcriptomics.bioRxiv : the preprint server for biology · 2026Article
- A silent pheochromocytoma with regional metastasis and negative germline testing.Endocrinology, diabetes & metabolism case reports · 2026Article
116 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
8 authors.
Funding
Abstract
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.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.