Literature DB >> 26275867

Incidental diagnosis of HLRCC following investigation for Asperger Syndrome: actionable and actioned.

Bich-Thu Duong1, Ravi Savarirayan2, Ingrid Winship3,4.   

Abstract

Incidental findings are inevitable as clinical research and practice transitions from a single gene approach to a genomic approach. A novel deletion of the Fumarate Hydratase (FH) gene was identified in a 22 year old male who underwent a molecular karyotype as part of an autism spectrum disorder research project. This unexpected result implies a predisposition to Hereditary Leiomyomatosis and Renal Cell Cancer (HLRCC), a rare, autosomal dominant condition and has unforeseen implications for him and his family. We review the typical features and management of HLRCC and discuss the challenges that face health professionals, as genetic testing advances and becomes more accessible.

Entities:  

Keywords:  Actionable genes; Hereditary leiomyomatosis and renal cell cancer syndrome (HLRCC); Incidental finding

Mesh:

Substances:

Year:  2016        PMID: 26275867     DOI: 10.1007/s10689-015-9829-5

Source DB:  PubMed          Journal:  Fam Cancer        ISSN: 1389-9600            Impact factor:   2.375


  20 in total

Review 1.  Searching for the hereditary causes of renal-cell carcinoma.

Authors:  Christian P Pavlovich; Laura S Schmidt
Journal:  Nat Rev Cancer       Date:  2004-05       Impact factor: 60.716

2.  Detection of a novel FH whole gene deletion in the propositus leading to subsequent prenatal diagnosis in a sibship with fumarase deficiency.

Authors:  Amelia R Mroch; Mark Laudenschlager; Jason D Flanagan
Journal:  Am J Med Genet A       Date:  2011-11-08       Impact factor: 2.802

3.  HIF overexpression correlates with biallelic loss of fumarate hydratase in renal cancer: novel role of fumarate in regulation of HIF stability.

Authors:  Jennifer S Isaacs; Yun Jin Jung; David R Mole; Sunmin Lee; Carlos Torres-Cabala; Yuen-Li Chung; Maria Merino; Jane Trepel; Berton Zbar; Jorge Toro; Peter J Ratcliffe; W Marston Linehan; Len Neckers
Journal:  Cancer Cell       Date:  2005-08       Impact factor: 31.743

4.  Perspectives of clinical genetics professionals toward genome sequencing and incidental findings: a survey study.

Authors:  A A Lemke; D Bick; D Dimmock; P Simpson; R Veith
Journal:  Clin Genet       Date:  2012-12-07       Impact factor: 4.438

Review 5.  Genetic and clinical aspects of familial renal neoplasms.

Authors:  O Iliopoulos; C Eng
Journal:  Semin Oncol       Date:  2000-04       Impact factor: 4.929

6.  Biallelic truncating mutations in FMN2, encoding the actin-regulatory protein Formin 2, cause nonsyndromic autosomal-recessive intellectual disability.

Authors:  Rosalind Law; Tracy Dixon-Salazar; Julie Jerber; Na Cai; Ansar A Abbasi; Maha S Zaki; Kirti Mittal; Stacey B Gabriel; Muhammad Arshad Rafiq; Valeed Khan; Maria Nguyen; Ghazanfar Ali; Brett Copeland; Eric Scott; Nasim Vasli; Anna Mikhailov; Muhammad Nasim Khan; Danielle M Andrade; Muhammad Ayaz; Muhammad Ansar; Muhammad Ayub; John B Vincent; Joseph G Gleeson
Journal:  Am J Hum Genet       Date:  2014-12-04       Impact factor: 11.025

7.  Mutations in the fumarate hydratase gene cause hereditary leiomyomatosis and renal cell cancer in families in North America.

Authors:  Jorge R Toro; Michael L Nickerson; Ming-Hui Wei; Michelle B Warren; Gladys M Glenn; Maria L Turner; Laveta Stewart; Paul Duray; Ousman Tourre; Nirmala Sharma; Peter Choyke; Pamela Stratton; Maria Merino; McClellan M Walther; W Marston Linehan; Laura S Schmidt; Berton Zbar
Journal:  Am J Hum Genet       Date:  2003-05-22       Impact factor: 11.025

8.  Genetic and functional analyses of FH mutations in multiple cutaneous and uterine leiomyomatosis, hereditary leiomyomatosis and renal cancer, and fumarate hydratase deficiency.

Authors:  N A Alam; A J Rowan; N C Wortham; P J Pollard; M Mitchell; J P Tyrer; E Barclay; E Calonje; S Manek; S J Adams; P W Bowers; N P Burrows; R Charles-Holmes; L J Cook; B M Daly; G P Ford; L C Fuller; S E Hadfield-Jones; N Hardwick; A S Highet; M Keefe; S P MacDonald-Hull; E D A Potts; M Crone; S Wilkinson; F Camacho-Martinez; S Jablonska; R Ratnavel; A MacDonald; R J Mann; K Grice; G Guillet; M S Lewis-Jones; H McGrath; D C Seukeran; P J Morrison; S Fleming; S Rahman; D Kelsell; I Leigh; S Olpin; I P M Tomlinson
Journal:  Hum Mol Genet       Date:  2003-06-01       Impact factor: 6.150

9.  Revealing the incidentalome when targeting the tumor genome.

Authors:  Yvonne Bombard; Mark Robson; Kenneth Offit
Journal:  JAMA       Date:  2013-08-28       Impact factor: 56.272

10.  Hereditary leiomyomatosis and renal cell cancer: update on clinical and molecular characteristics.

Authors:  Heli J Lehtonen
Journal:  Fam Cancer       Date:  2011-06       Impact factor: 2.446

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  2 in total

1.  Strategies to Guide the Return of Genomic Research Findings: An Australian Perspective.

Authors:  Lisa Eckstein; Margaret Otlowski
Journal:  J Bioeth Inq       Date:  2018-05-16       Impact factor: 1.352

2.  Hemangioblastoma in Hereditary Leiomyomatosis and Renal Cell Cancer Syndrome: a phenotypic overlap between VHL and HLRCC Syndromes.

Authors:  Eryn Dow; Ingrid M Winship
Journal:  Fam Cancer       Date:  2019-01       Impact factor: 2.375

  2 in total

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