Evidence map›Paper›PMID 37450881›Full record

ReviewEndocrine-related cancer2023

Pacak-Zhuang syndrome: a model providing new insights into tumor syndromes.

Jared S Rosenblum, Herui Wang, Matthew A Nazari, Zhengping Zhuang, Karel Pacak

Open access · bronzeAbstract readReview
In one paragraph

Review in Endocrine-related cancer, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers.

0numbers the graph read from it
0cells of the map it votes in
9citing papers in PubMed
3.5field-weighted citation impact, top 7% of its field
1 · What the graph read from it

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.

2 · The registry

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.

3 · Its place in the literature

Who cites it

9 citing papers in PubMed, 11 citations in OpenAlex.

  1. Article
  2. The clinical landscape of HIF2α inhibitors in oncology.Nature reviews. Clinical oncology · 2026
    Review
  3. Article
  4. Expanding the clinical tumor phenotype of the EPAS1-asssociated tumor syndrome.The Journal of clinical endocrinology and metabolism · 2026
    Article
  5. Review
  6. Article
  7. Review
  8. Article
  9. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

5 authors at 2 institutions in 1 country.

Jared S RosenblumNeuro-Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, United States.ORCID 0000-0001-5941-9082
Herui WangNeuro-Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, United States.
Matthew A NazariEunice Kennedy Shriver National Institute of Child Health and Development, Bethesda, Maryland, United States.ORCID 0000-0002-1956-6077
Zhengping ZhuangNeuro-Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, United States.ORCID 0000-0002-0009-7370
Karel PacakEunice Kennedy Shriver National Institute of Child Health and Development, Bethesda, Maryland, United States.ORCID 0000-0002-3541-3767
National Institutes of Health · USEunice Kennedy Shriver National Institute of Child Health and Human Development · US

Funding

Hypoxia signaling in cancer developmentZIABC011773 · NCI · DIVISION OF BASIC SCIENCES - NCI · PI ZHUANG, ZHENGPING · 2017 to 2025
$13.0M
Intramural NIH HHS Z99 HD999999
6 · The paper itself

Abstract

This article is a summary of the plenary lecture presented by Jared Rosenblum that was awarded the Manger Prize at the Sixth International Symposium on Pheochromocytoma/Paraganglioma held on 19-22 October 2022 in Prague, Czech Republic. Herein, we review our initial identification of a new syndrome of multiple paragangliomas, somatostatinomas, and polycythemia caused by early postzygotic mosaic mutations in EPAS1, encoding hypoxia-inducible factor 2 alpha (HIF-2α), and our continued exploration of new disease phenotypes in this syndrome, including vascular malformations and neural tube defects. Continued recruitment and close monitoring of patients with this syndrome as well as the generation and study of a corresponding disease mouse model as afforded by the pheochromocytoma/paraganglioma translational program at the National Institutes of Health has provided new insights into the natural history of these developmental anomalies and the pathophysiologic role of HIF-2α. Further, these studies have highlighted the importance of the timing of genetic defects in the development of related disease phenotypes. The recent discovery and continued study of this syndrome has not only rapidly evolved our understanding of pheochromocytoma and paraganglioma but also deepened our understanding of other developmental tumor syndromes, heritable syndromes, and sporadic diseases.

Indexed as

Adrenal Gland NeoplasmsParagangliomaPheochromocytomaAnimalsBasic Helix-Loop-Helix ProteinsMiceSyndromeBasic Helix-Loop-Helix ProteinsEPAS1HIF-2αmosaicismPacak–Zhuang syndromeparagangliomapheochromocytomasyndrometumor

Identifiers

PMID37450881
PMCPMC10512798
OpenAlexW4384282056

What OpenQuestion holds

Textmetadata
LicenceTDM
Read underepoch 390

Registered trials

None linked

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.