Evidence map›Paper›PMID 39382746›Full record

ArticleAnnals of surgical oncology2024

SDHB-Associated Pheochromocytomas: What is Their Clinical Behavior?

Thomas Szabo Yamashita, Andres Tame-Elorduy, Catherine M Skefos, Jeena M Varghese, Mouhammed Amir Habra, Sarah B Fisher, Paul H Graham, Elizabeth G Grubbs, Steven G Waguespack, Camilo Jimenez and 1 more

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

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1citing papers in PubMed
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1 · What the graph read from it

What it found

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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

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3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

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4 · The record

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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

11 authors.

Thomas Szabo YamashitaDepartment of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Andres Tame-ElorduyDepartment of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Catherine M SkefosDepartment of Clinical Cancer Genetics, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Jeena M VargheseDepartment of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Mouhammed Amir HabraDepartment of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Sarah B FisherDepartment of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Paul H GrahamDepartment of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Elizabeth G GrubbsDepartment of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Steven G WaguespackDepartment of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Camilo JimenezDepartment of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Nancy D PerrierDepartment of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. NDPerrier@mdanderson.org.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

introductionGermline pathogenic variants in succinate dehydrogenase subunit B (SDHB) cause paraganglioma/pheochromocytoma syndrome type 4 (PGL-4). SDHB-associated pheochromocytomas (PCC) are thought to be rare and little data exist about their clinical behavior. PATIENTS AND

methodsRetrospective review of patients treated (1993-2023) at a tertiary cancer center for SDHB-associated PCC. Clinical and demographic variables were retrieved to characterize disease-free survival, disease progression, and overall survival.

resultsIn total, 90 SDHB-carriers were identified, 18% had PCC (n = 16). Median age at diagnosis of was 40 (19-76) years, 50% (n = 8) of patients were male, 25% (n = 4) had distant metastasis (DM) at diagnosis, and 13% (n = 2) had synchronous PGL. No patients had bilateral disease, and 94% of patients underwent surgery as initial treatment with a curative intent in 75%. Overall, 64% of patients underwent open resection. Recurrence occurred in 77% of patients (n = 10), 75% in minimally invasive surgery (MIS) versus 77% open, p = 0.63. Bone was the most common site of DM (100%, n = 13). Metaidobenzyleguanidine (MIBG) imaging was performed in 69% of patients, 91% of which were positive. Median time from surgery until recurrence was 36 months (1-295 months). Radiation therapy was the most common adjuvant treatment (44%) followed by Iobenguane I-131 (31%) and systemic therapy (31%). Median follow-up time was 56 months (1-408 months). Overall, 33% of patients were alive, 19% of patients were disease-free, and 50% of the patients with DM had stable disease at last follow-up.

conclusionsOverall, 18% of germline SDHB mutation-carriers were diagnosed with PCC, all of which were unilateral. SDHB-associated PCC was associated with advanced and recalcitrant disease and was often MIBG positive. More studies are needed to better understand the clinical behavior of PCC in PGL-4.

Indexed as

Adrenal Gland NeoplasmsPheochromocytomaSuccinate DehydrogenaseAdultAgedFemaleFollow-Up StudiesGerm-Line MutationHumansMaleMiddle AgedNeoplasm Recurrence, LocalParagangliomaPrognosisRetrospective StudiesSurvival RateSDHB protein, humanSuccinate Dehydrogenase

Identifiers

PMID39382746

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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.