Evidence map›Paper›PMID 33433885›Full record

ReviewEndocrine pathology2021

What Have We Learned from Molecular Biology of Paragangliomas and Pheochromocytomas?

Thomas G Papathomas, Diederik P D Suurd, Karel Pacak, Arthur S Tischler, Menno R Vriens, Alfred K Lam, Ronald R de Krijger

Registry-linked trialAbstract readReview
PubMed Publisher
In one paragraph

Review in Endocrine pathology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT06523582 (Genetic Bases of Neuroendocrine Neoplasms in Mexican Patients), which is not on this map. Cited by 21 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
21citing papers in PubMed, 2 pooled it
–field-weighted citation impact
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.

NCT06523582 recruitingnot on this mapstarted 2022, after this paper: background citation

Genetic Bases of Neuroendocrine Neoplasms in Mexican Patients

TypeobservationalSponsorUniversidad Nacional Autonoma de MexicoRan2022 to 2037Enrolled750ConditionsNeuroendocrine Neoplasm, Neuroendocrine Neoplasm of Gastrointestinal Tract, Neuroendocrine Neoplasm of Lung, Thymic Neuroendocrine Neoplasm
3 · Its place in the literature

Who cites it

21 citing papers in PubMed, 2 syntheses or guidelines pooled it.

  1. Pooled it
  2. 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 · 2021
    Guideline
  3. Phase II Study of ONC201 in Neuroendocrine Tumors including Pheochromocytoma-Paraganglioma and Desmoplastic Small Round Cell Tumor.Clinical cancer research : an official journal of the American Association for Cancer Research · 2022
    Trial
  4. Article
  5. The evolving roles of pathologists in paragangliomas and pheochromocytomas.Virchows Archiv : an international journal of pathology · 2026
    Review
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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

7 authors.

Thomas G Papathomas *Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK.
Diederik P D Suurd *Department of Surgical Oncology and Endocrine Surgery, University Medical Center Utrecht, Utrecht, The Netherlands.
Karel PacakSection on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA.
Arthur S TischlerDepartment of Pathology and Laboratory Medicine, Tufts Medical Center, Boston Massachusetts, USA.
Menno R VriensDepartment of Surgical Oncology and Endocrine Surgery, University Medical Center Utrecht, Utrecht, The Netherlands.
Alfred K Lam *School of Medicine, Griffith University, Gold Coast, QLD, Australia. a.lam@griffith.edu.au.ORCID http://orcid.org/0000-0003-2771-564X
Ronald R de Krijger *Princess Maxima Center for Pediatric Oncology, Utrecht, The Netherlands. r.r.dekrijger-2@prinsesmaximacentrum.nl.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Recent advances in molecular genetics and genomics have led to increased understanding of the aetiopathogenesis of pheochromocytomas and paragangliomas (PPGLs). Thus, pan-genomic studies now provide a comprehensive integrated genomic analysis of PPGLs into distinct molecularly defined subtypes concordant with tumour genotypes. In addition, new embryological discoveries have refined the concept of how normal paraganglia develop, potentially establishing a developmental basis for genotype-phenotype correlations for PPGLs. The challenge for modern pathology is to translate these scientific discoveries into routine practice, which will be based largely on histopathology for the foreseeable future. Here, we review recent progress concerning the cell of origin and molecular pathogenesis of PPGLs, including pathogenetic mechanisms, genetic susceptibility and molecular classification. The current roles and tools of pathologists are considered from a histopathological perspective, including differential diagnoses, genotype-phenotype correlations and the use of immunohistochemistry in identifying hereditary predisposition and validating genetic variants of unknown significance. Current and potential molecular prognosticators are also presented with the hope that predictive molecular biomarkers will be integrated into risk stratification scoring systems to assess the metastatic potential of these intriguing neoplasms and identify potential drug targets.

Indexed as

Adrenal Gland NeoplasmsGenetic Predisposition to DiseaseGenomicsGenotypeHumansParagangliomaPheochromocytomaImmunohistochemistryMolecular biologyParagangliomaPheochromocytoma

Identifiers

What OpenQuestion holds

Textmetadata
Read underepoch 390

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.