Evidence map›Paper›PMID 35973976›Full record

ReviewThe Journal of clinical endocrinology and metabolism2022

Targeted Therapies in Pheochromocytoma and Paraganglioma.

Katharina Wang, Joakim Crona, Felix Beuschlein, Ashley B Grossman, Karel Pacak, Svenja Nölting

Open access · bronzeAbstract readReview
In one paragraph

Review in The Journal of clinical endocrinology and metabolism, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 37 papers.

0numbers the graph read from it
0cells of the map it votes in
37citing papers in PubMed
11.1field-weighted citation impact, top 1% 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

37 citing papers in PubMed, 55 citations in OpenAlex.

  1. Article
  2. Article
  3. 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 · 2026
    Article
  4. Article
  5. Article
  6. Article
  7. Article
  8. Article
  9. Review
  10. Review
  11. Current Strategies for Managing Pheochromocytoma and Paraganglioma in Children and Adolescents.Medical science monitor : international medical journal of experimental and clinical research · 2025
    Review
  12. Clinical Activity of Selpercatinib in RET-mutant Pheochromocytoma.The Journal of clinical endocrinology and metabolism · 2025
    Article
  13. Neuroendocrine Tumors: Germline Genetics and Hereditary Syndromes.Current treatment options in oncology · 2025
    Review
  14. Progress in surgical approaches and outcomes of patients with pheochromocytoma and paraganglioma.Best practice & research. Clinical endocrinology & metabolism · 2025
    Review
  15. Review
  16. Article
  17. Non-Susceptibility Gene Variants in Head and Neck Paragangliomas.International journal of molecular sciences · 2024
    Article
  18. Article
  19. Article
  20. 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

6 authors at 5 institutions in 5 countries.

Katharina WangDepartment of Internal Medicine IV, University Hospital, LMU Klinikum, Ludwig Maximilian University of Munich, 80336 Munich, Germany.ORCID 0000-0002-7795-1395
Joakim CronaDepartment of Medical Sciences, Uppsala University, 75185 Uppsala, Sweden.
Felix BeuschleinDepartment of Internal Medicine IV, University Hospital, LMU Klinikum, Ludwig Maximilian University of Munich, 80336 Munich, Germany.ORCID 0000-0001-7826-3984
Ashley B GrossmanGreen Templeton College, University of Oxford, Oxford OX2 6HG, United Kingdom.ORCID 0000-0003-1176-6186
Karel PacakEunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892-1109, USA.ORCID 0000-0002-3541-3767
Svenja NöltingDepartment of Internal Medicine IV, University Hospital, LMU Klinikum, Ludwig Maximilian University of Munich, 80336 Munich, Germany.ORCID 0000-0002-7064-590X
University of Zurich · CHLMU Klinikum · DENational Institutes of Health · USThe Royal Free Hospital · GBUppsala University · SE

Funding

DFG 314061271 -TRR 205University Medicine Zurich
6 · The paper itself

Abstract

Molecular targeted therapy plays an increasingly important role in the treatment of metastatic pheochromocytomas and paragangliomas (PPGLs), which are rare tumors but remain difficult to treat. This mini-review provides an overview of established molecular targeted therapies in present use, and perspectives on those currently under development and evaluation in clinical trials. Recently published research articles, guidelines, and expert views on molecular targeted therapies in PPGLs are systematically reviewed and summarized. Some tyrosine kinase inhibitors (sunitinib, cabozantinib) are already in clinical use with some promising results, but without formal approval for the treatment of PPGLs. Sunitinib is the only therapeutic option which has been investigated in a randomized placebo-controlled clinical trial. It is clinically used as a first-, second-, or third-line therapeutic option for the treatment of progressive metastatic PPGLs. Some other promising molecular targeted therapies (hypoxia-inducible factor 2 alpha [HIF2α] inhibitors, tumor vaccination together with checkpoint inhibitors, antiangiogenic therapies, kinase signaling inhibitors) are under evaluation in clinical trials. The HIF2α inhibitor belzutifan may prove to be particularly interesting for cluster 1B-/VHL/EPAS1-related PPGLs, whereas antiangiogenic therapies seem to be primarily effective in cluster 1A-/SDHx-related PPGLs. Some combination therapies currently being evaluated in clinical trials, such as temozolomide/olaparib, temozolomide/talazoparib, or cabozantinib/atezolizumab, will provide data for novel therapy for metastatic PPGLs. It is likely that advances in such molecular targeted therapies will play an essential role in the future treatment of these tumors, with more personalized therapy options paving the way towards improved therapeutic outcomes.

Indexed as

Adrenal Gland NeoplasmsParagangliomaPheochromocytomaAnilidesHumansPyridinesRandomized Controlled Trials as TopicSunitinibTemozolomideAnilidescabozantinibPyridinesSunitinibTemozolomidemetastaticmolecular targeted therapyparagangliomapheochromocytoma

Identifiers

PMID35973976
PMCPMC9923802
OpenAlexW4292170703

What OpenQuestion holds

Textmetadata
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.