ReviewEndocrine-related cancer2020
HEREDITARY ENDOCRINE TUMOURS: CURRENT STATE-OF-THE-ART AND RESEARCH OPPORTUNITIES: Metastatic pheochromocytomas and paragangliomas: proceedings of the MEN2019 workshop.
Review in Endocrine-related cancer, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 30 papers.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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
30 citing papers in PubMed, 50 citations in OpenAlex.
- Article
- The Biochemical-Imaging Connection: Urinary Noradrenaline and Fluorodeoxyglucose-Positron Emission Tomography in Unresectable or Metastatic Pheochromocytomas and Paragangliomas.Diagnostics (Basel, Switzerland) · 2025Article
- Urinary Dopamine Levels Can Predict the Avidity of Post-Therapy [Pharmaceuticals (Basel, Switzerland) · 2025Article
- Multidisciplinary management of a rare case of a large functional paraganglioma in the posterior mediastinum in an adolescent: a case report and literature review.Frontiers in oncology · 2025Article
- Management of Pheochromocytomas and Paragangliomas.The Surgical clinics of North America · 2024Review
- Successful delivery in the setting of SDHB metastatic paraganglioma.Endocrinology, diabetes & metabolism case reports · 2024Article
- Overview of endocrine tumor syndromes manifesting as adrenal tumors.Ewha medical journal · 2024Review
- [Metastatic risk factors in pheochromocytoma/paraganglioma].Problemy endokrinologii · 2023Review
- Update on the genetics of paragangliomas.Endocrine-related cancer · 2023Review
- Hypoxia-Inducible Factor 2 Alpha (HIF2α) Inhibitors: Targeting Genetically Driven Tumor Hypoxia.Endocrine reviews · 2023Article
- Genomic and immune landscape Of metastatic pheochromocytoma and paraganglioma.Nature communications · 2023Article
- Metastatic paraganglioma.BMJ case reports · 2023Article
- Hereditary Endocrine Tumor Registries.Journal of the Endocrine Society · 2023Article
- Pheochromocytoma: a changing perspective and current concepts.Therapeutic advances in endocrinology and metabolism · 2023Review
- New Biology of Pheochromocytoma and Paraganglioma.Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists · 2022Review
- Single-nuclei and bulk-tissue gene-expression analysis of pheochromocytoma and paraganglioma links disease subtypes with tumor microenvironment.Nature communications · 2022Article
- Succinate Dehydrogenase, Succinate, and Superoxides: A Genetic, Epigenetic, Metabolic, Environmental Explosive Crossroad.Biomedicines · 2022Review
- Personalized Management of Pheochromocytoma and Paraganglioma.Endocrine reviews · 2022Review
- Perioperative Management of Pheochromocytomas and Sympathetic Paragangliomas.Journal of the Endocrine Society · 2022Review
- Retroperitoneal Paraganglioma With Asymptomatic Follicular Lymphoma: A Case Report.Journal of the Endocrine Society · 2021Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
5 authors at 4 institutions in 4 countries.
Funding
Abstract
Pheochromocytomas and paragangliomas (PPGLs) are adrenal or extra-adrenal autonomous nervous system-derived tumors. Most PPGLs are benign, but approximately 15% progress with metastases (mPPGLs). mPPGLs are more likely to occur in patients with large pheochromocytomas, sympathetic paragangliomas, and norepinephrine-secreting tumors. Older subjects, those with larger tumors and synchronous metastases, advance more rapidly. Germline mutations of SDHB, FH, and possibly SLC25A11, or somatic MAML3 disruptions relate to a higher risk for metastatic disease. However, it is unclear whether these mutations predict outcome. Once diagnosed, there are no well-established predictors of outcome in mPPGLs, and aggressive tumors have few therapeutic options and limited response. High-specific activity (HSA) metaiodine-benzyl-guanidine (MIBG) is the first FDA approved treatment and shows clinical effectiveness for MIBG-avid mPPGLs. Ongoing and future investigations should involve validation of emerging candidate outcome biomarkers, including somatic ATRX, TERT, and microRNA disruptions and identification of novel prognostic indicators. Long-term effect of HSA-MIBG and the role of other radiopharmaceuticals should be investigated. Novel trials targeting molecular events prevalent in SDHB/FH mutant tumors, such as activated hypoxia inducible factor 2 (HIF2), angiogenesis, or other mitochondrial defects that might confer unique vulnerability to these tumors should be developed and initiated. As therapeutic options are anticipated to expand, multi-institutional collaborations and well-defined clinical and molecular endpoints will be critical to achieve higher success rates in improving care for patients with mPPGLs.
Indexed as
Identifiers
What Socratic holds
Registered trials
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 Socratic graph.