ArticleEndocrine-related cancer2016
Novel insights into the polycythemia-paraganglioma-somatostatinoma syndrome.
Article in Endocrine-related cancer, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 53 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
53 citing papers in PubMed.
- Surgical Management of Solid Tumors With Hematologic Manifestations: A Mechanism-Based Clinical Review.European journal of haematology · 2026Review
- Expanding the clinical tumor phenotype of the EPAS1-asssociated tumor syndrome.The Journal of clinical endocrinology and metabolism · 2026Article
- Incidental Adrenal Hemangioma: A Diagnostic and Management Challenge.JCEM case reports · 2025Article
- A schema for sporadic and heritable disease pathogenesis integrating spatiotemporal distribution with the character of genetic variants.Communications medicine · 2025Article
- Clinicopathological and genomic analysis of pediatric pheochromocytoma and sympathetic paraganglioma.Endocrine journal · 2025Article
- Urinary Dopamine Levels Can Predict the Avidity of Post-Therapy [Pharmaceuticals (Basel, Switzerland) · 2025Article
- Head and neck paraganglioma in Pacak-Zhuang syndrome.JNCI cancer spectrum · 2025Article
- International consensus statement on the diagnosis and management of phaeochromocytoma and paraganglioma in children and adolescents.Nature reviews. Endocrinology · 2024Review
- Imaging of Pheochromocytomas and Paragangliomas.Endocrine reviews · 2024Review
- Deficiency in PHD2-mediated hydroxylation of HIF2α underlies Pacak-Zhuang syndrome.Communications biology · 2024Article
- Tumor metabolism in pheochromocytomas: clinical and therapeutic implications.Exploration of targeted anti-tumor therapy · 2024Review
- Improving susceptibility of neuroendocrine tumors to radionuclide therapies: personalized approaches towards complementary treatments.Theranostics · 2024Review
- Pacak-Zhuang syndrome: a model providing new insights into tumor syndromes.Endocrine-related cancer · 2023Review
- Metastatic Pheochromocytoma and Paraganglioma: Somatostatin Receptor 2 Expression, Genetics, and Therapeutic Responses.The Journal of clinical endocrinology and metabolism · 2023Article
- Pheochromocytoma in an Ectopic Adrenal Gland.Cureus · 2023Article
- ComprehensiveHaematologica · 2023Article
- An Insight on Functioning Pancreatic Neuroendocrine Neoplasms.Biomedicines · 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
- Article
- Personalized Management of Pheochromocytoma and Paraganglioma.Endocrine reviews · 2022Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
28 authors.
Funding
Abstract
Worldwide, the syndromes of paraganglioma (PGL), somatostatinoma (SOM) and early childhood polycythemia are described in only a few patients with somatic mutations in the hypoxia-inducible factor 2 alpha (HIF2A). This study provides detailed information about the clinical aspects and course of 7 patients with this syndrome and brings into perspective these experiences with the pertinent literature. Six females and one male presented at a median age of 28 years (range 11-46). Two were found to have HIF2A somatic mosaicism. No relatives were affected. All patients were diagnosed with polycythemia before age 8 and before PGL/SOM developed. PGLs were found at a median age of 17 years (range 8-38) and SOMs at 29 years (range 22-38). PGLs were multiple, recurrent and metastatic in 100, 100 and 29% of all cases, and SOMs in 40, 40 and 60%, respectively. All PGLs were primarily norepinephrine-producing. All patients had abnormal ophthalmologic findings and those with SOMs had gallbladder disease. Computed tomography (CT) and magnetic resonance imaging revealed cystic lesions at multiple sites and hemangiomas in 4 patients (57%), previously thought to be pathognomonic for von Hippel-Lindau disease. The most accurate radiopharmaceutical to detect PGL appeared to be [
Indexed as
Identifiers
What OpenQuestion 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 OpenQuestion graph.