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Record W4362466994 · doi:10.1016/s2213-8587(23)00038-4

Clinical consensus guideline on the management of phaeochromocytoma and paraganglioma in patients harbouring germline SDHD pathogenic variants

2023· review· en· W4362466994 on OpenAlexaff
David Taïeb, George B. Wanna, Maleeha Ahmad, Charlotte Lussey‐Lepoutre, Nancy D. Perrier, Svenja Nölting, Laurence Amar, Henri Timmers, Zachary G. Schwam, Anthony L. Estrera, Michael Lim, Erqi L. Pollom, Lucas K. Vitzthum, Isabelle Bourdeau, Ruth Casey, Frédéric Castinetti, Roderick Clifton‐Bligh, Eleonora P.M. Corssmit, Ronald R. de Krijger, Jaydira Del Rivero, Graeme Eisenhofer, Hans K. Ghayee, Anne‐Paule Gimenez‐Roqueplo, Ashley Grossman, Alessio Impériale, Jeroen C. Jansen, Abhishek Jha, Michiel N. Kerstens, Henricus P. M. Kunst, James K. Liu, Eamonn R. Maher, Daniele Marchioni, Leilani B Mercado-Asis, Özgür Mete, Mitsuhide Naruse, Naris Nilubol, Neeta Pandit‐Taskar, F. Sébag, Akiyo Tanabe, J Widimský, Leah Meuter, Jacques W.M. Lenders, Karel Pacák

Bibliographic record

VenueThe Lancet Diabetes & Endocrinology · 2023
Typereview
Languageen
FieldMedicine
TopicAdrenal and Paraganglionic Tumors
Canadian institutionsUniversity of TorontoMultiple Sclerosis Society of CanadaCentre Hospitalier de l’Université de Montréal
FundersNational Institute of Child Health and Human DevelopmentEunice Kennedy Shriver National Institute of Child Health and Human DevelopmentNational Cancer InstituteNational Institutes of Health
KeywordsSDHDMedicineParagangliomaGuidelinePheochromocytomaWatchful waitingMultiple endocrine neoplasia type 2DiseaseGermline mutationInternal medicineSurgeryPathologyGeneticsCancer

Abstract

fetched live from OpenAlex

Patients with germline SDHD pathogenic variants (encoding succinate dehydrogenase subunit D; ie, paraganglioma 1 syndrome) are predominantly affected by head and neck paragangliomas, which, in almost 20% of patients, might coexist with paragangliomas arising from other locations (eg, adrenal medulla, para-aortic, cardiac or thoracic, and pelvic). Given the higher risk of tumour multifocality and bilaterality for phaeochromocytomas and paragangliomas (PPGLs) because of SDHD pathogenic variants than for their sporadic and other genotypic counterparts, the management of patients with SDHD PPGLs is clinically complex in terms of imaging, treatment, and management options. Furthermore, locally aggressive disease can be discovered at a young age or late in the disease course, which presents challenges in balancing surgical intervention with various medical and radiotherapeutic approaches. The axiom-first, do no harm-should always be considered and an initial period of observation (ie, watchful waiting) is often appropriate to characterise tumour behaviour in patients with these pathogenic variants. These patients should be referred to specialised high-volume medical centres. This consensus guideline aims to help physicians with the clinical decision-making process when caring for patients with SDHD PPGLs.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame machine prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.

metaresearch head score (Codex)0.002
metaresearch head score (Gemma)0.004
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Not applicable · Consensus signal: none
GenreCandidate signal: Review · Consensus signal: Review
Teacher disagreement score0.010
Threshold uncertainty score0.021

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0020.004
Meta-epidemiology (narrow)0.0010.001
Meta-epidemiology (broad)0.0030.003
Bibliometrics0.0030.002
Science and technology studies0.0000.000
Scholarly communication0.0010.001
Open science0.0030.001
Research integrity0.0030.003
Insufficient payload (model declined to judge)0.0060.004

Machine scores (provisional)

The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.

Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.

Opus teacher head0.107
GPT teacher head0.385
Teacher spread0.278 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designNot applicable
Domainnot available
GenreReview

How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".

Quick stats

Citations87
Published2023
Admission routes1
Has abstractno

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