Literature DB >> 29315604

Trends of genetic screening in patients with pheochromocytoma and paraganglioma: 15-year experience in a high-volume tertiary referral center.

Ammar Asban1, Wouter P Kluijfhout2, Frederick T Drake3, Toni Beninato4, Elizabeth Wang5, Kate Chomsky-Higgins5, Wen T Shen5, Jessica E Gosnell5, Insoo Suh5, Quan-Yang Duh5.   

Abstract

BACKGROUND AND OBJECTIVES: Genetic testing for pheochromocytoma and paraganglioma allows for early detection of hereditary syndromes and enables close follow-up of high-risk patient. We investigated the trends in genetic testing among patients at a high-volume referral center and evaluated the prevalence of pheochromocytomas and paragangliomas.
METHODS: We reviewed the charts of 129 patients who underwent adrenalectomy for pheochromocytoma and paraganglioma between January 2000 and July 2015. To evaluate for trends in genetic testing, patients were divided by year of diagnosis: 2000-2005 (group 1, n = 35), 2006-2010 (group 2, n = 44), and 2011-2015 (group 3, n = 50).
RESULTS: Among 129 patients the mean age was 47 years and 56% were women. Groups 2 and 3 were more frequently referred for genetic consultation than group 1, 73%, and 94% versus 26% (P < 0.001). A total of 67% followed up on the referral. The prevalence of genetic mutation was 50% (21/42 tested). The percentage with a genetic syndrome was 23%, 28%, and 22% respectively for groups 1, 2, and 3.
CONCLUSIONS: Referral for genetic counseling significantly increased in the past 15 years. However, only two-thirds of patients followed up with genetic counselors and, therefore, clinicians can do more to improve the adherence rate for genetic counseling.
© 2018 Wiley Periodicals, Inc.

Entities:  

Keywords:  genetic and screening; paraganglioma; pheochromocytoma

Mesh:

Substances:

Year:  2018        PMID: 29315604     DOI: 10.1002/jso.24961

Source DB:  PubMed          Journal:  J Surg Oncol        ISSN: 0022-4790            Impact factor:   3.454


  4 in total

1.  Clinical Predictors of Pseudohypoxia-Type Pheochromocytomas.

Authors:  J J Baechle; P Marincola Smith; C A Ortega; T S Wang; C C Solórzano; C M Kiernan
Journal:  Ann Surg Oncol       Date:  2022-03-01       Impact factor: 4.339

2.  Clinical Presentation and Perioperative Management of Pheochromocytomas and Paragangliomas: A 4-Decade Experience.

Authors:  Thomas Uslar; Ignacio F San Francisco; Roberto Olmos; Stefano Macchiavelo; Alvaro Zuñiga; Pablo Rojas; Marcelo Garrido; Alvaro Huete; Gonzalo P Mendez; Ignacio Cortinez; José Tomás Zemelman; Joaquín Cifuentes; Fernando Castro; Daniela Olivari; José Miguel Domínguez; Eugenio Arteaga; Carlos E Fardella; Gloria Valdés; Rodrigo Tagle; Rene Baudrand
Journal:  J Endocr Soc       Date:  2021-04-22

3.  Mutational profile and genotype/phenotype correlation of non-familial pheochromocytoma and paraganglioma.

Authors:  Shatha Albattal; Meshael Alswailem; Yosra Moria; Hindi Al-Hindi; Majed Dasouki; Mohamed Abouelhoda; Hala Aba Alkhail; Entissar Alsuhaibani; Ali S Alzahrani
Journal:  Oncotarget       Date:  2019-10-15

Review 4.  Head and Neck Paragangliomas-A Genetic Overview.

Authors:  Anna Majewska; Bartłomiej Budny; Katarzyna Ziemnicka; Marek Ruchała; Małgorzata Wierzbicka
Journal:  Int J Mol Sci       Date:  2020-10-16       Impact factor: 5.923

  4 in total

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