Evidence map›Paper›PMID 39682154›Full record

ArticleCancers2024

Molecular and Clinical Features of Adrenocortical Tumors in Beckwith-Wiedemann Spectrum.

Diana Carli, Federico Rondot, Maria Luca, Anna Campello, Stefano Gabriele Vallero, Elisa Tirtei, Andrea Gazzin, Simona Cardaropoli, Francesca Montanari, Claudio Graziano and 6 more

Abstract read
In one paragraph

Article in Cancers, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–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.

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

1 citing paper in PubMed.

  1. Review
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

16 authors.

Diana CarliDepartment of Medical Sciences, University of Torino, 10126 Torino, Italy.ORCID 0000-0001-5690-6504
Federico RondotDepartment of Medical Sciences, University of Torino, 10126 Torino, Italy.
Maria LucaDepartment of Medical Sciences, University of Torino, 10126 Torino, Italy.ORCID 0009-0006-1239-0829
Anna CampelloPediatric Onco-Hematology, Regina Margherita Children's Hospital, Città della Salute e della Scienza University Hospital, 10126 Torino, Italy.ORCID 0000-0002-1274-4395
Stefano Gabriele ValleroPediatric Onco-Hematology, Regina Margherita Children's Hospital, Città della Salute e della Scienza University Hospital, 10126 Torino, Italy.ORCID 0000-0001-5561-9263
Elisa TirteiPediatric Onco-Hematology, Regina Margherita Children's Hospital, Città della Salute e della Scienza University Hospital, 10126 Torino, Italy.ORCID 0000-0003-4144-5957
Andrea GazzinDepartment of Public Health and Pediatrics, University of Torino, 10126 Torino, Italy.ORCID 0000-0002-5230-2831
Simona CardaropoliDepartment of Public Health and Pediatrics, University of Torino, 10126 Torino, Italy.ORCID 0000-0002-8927-8900
Francesca MontanariMedical Genetics Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, Italy.
Claudio GrazianoMedical Genetics Unit, AUSL della Romagna, 48121 Ravenna, Italy.ORCID 0000-0003-3875-6869
Paola QuarelloPediatric Onco-Hematology, Regina Margherita Children's Hospital, Città della Salute e della Scienza University Hospital, 10126 Torino, Italy.ORCID 0000-0002-1940-7375
Abu SaadatDepartment of Environmental Biological and Pharmaceutical Sciences and Technologies (DiSTABiF), Università degli Studi della Campania "Luigi Vanvitelli", 81100 Caserta, Italy.ORCID 0000-0001-9636-2513
Angela SparagoDepartment of Environmental Biological and Pharmaceutical Sciences and Technologies (DiSTABiF), Università degli Studi della Campania "Luigi Vanvitelli", 81100 Caserta, Italy.ORCID 0000-0003-4352-945X
Giovanni Battista FerreroDepartment of Clinical and Biological Sciences, University of Torino, 10043 Orbassano, Italy.
Franca FagioliPediatric Onco-Hematology, Regina Margherita Children's Hospital, Città della Salute e della Scienza University Hospital, 10126 Torino, Italy.ORCID 0000-0002-9257-900X
Alessandro MussaDepartment of Public Health and Pediatrics, University of Torino, 10126 Torino, Italy.ORCID 0000-0003-2795-6013

Funding

AIRC-Associazione Italiana Ricerca sul Cancro IG 2020 24405
6 · The paper itself

Abstract

BACKGROUND/

objectivesAdrenocortical tumors (ACTs), including adrenocortical adenoma (ACA) and carcinoma (ACC), represent 0.3-0.4% of pediatric tumors. Beckwith-Wiedemann spectrum (BWSp) confer an increased risk of ACTs, but prognosis, management, and associated molecular characteristics are unclear.

methodsThis paper combines a literature review of 54 published cases of BWSp-ACT with a report of one newly identified patient, totaling 55 cases with a confirmed BWSp clinical and/or molecular diagnosis.

resultsNineteen patients with ACA, 33 with ACC, and 3 with ACT of uncertain malignant potential (umACT) were included. Twenty patients had uniparental disomy of chromosome 11p15.5 (patUPD11), 11imprinting Center 2 Loss-of-methylation (IC2-LoM), and had 2 11p15 locus duplication. Eleven patients were diagnosed during cancer screening procedures, including two metastatic at diagnosis ACC.

conclusionsAlmost half of ACC patients reached the minimum score for clinical BWSp diagnosis only after ACC onset, suggesting that the BWSp score has limited value for the early diagnosis in such a setting. Two patients with metastatic ACC had a histopathological Wieneke score ≤2, not correlating with clinical malignancy and confirming limitations of the current histopathological classification, as previously documented. Ultrasound screening failed identifying the ACC before metastasis in two cases, indicating an urgent need to develop new strategies for screening of ACTs in BWSp. Furthermore, some cases of metastatic ACC exhibited unexpectedly indolent behavior despite being malignant.

Indexed as

adrenocortical carcinomaadrenocortical tumorsBeckwith–Wiedemann spectrum

Identifiers

PMID39682154
PMCPMC11640037

What Socratic holds

Textmetadata
LicenceCC BY
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 Socratic graph.