Literature DB >> 10199897

Malignancies in pediatric patients with ataxia telangiectasia.

R C Murphy1, W E Berdon, C Ruzal-Shapiro, E J Hall, A Kornecki, A Daneman, F Brunelle, J B Campbell.   

Abstract

BACKGROUND: Patients with ataxia telangiectasia (AT), known to have an inherent increased susceptibility to the development of cancer, may present with malignancies that are unusual for the patient's age, are often difficult to diagnose clinically and radiographically and respond poorly to conventional therapy.
MATERIALS AND METHODS: We reviewed the clinical presentation and imaging studies of 12 AT patients who developed malignancies.
RESULTS: Eight of the twelve patients developed non-Hodgkin's lymphoma (CNS, thorax, bone), two developed Hodgkin's disease, and two were diagnosed with gastrointestinal mucinous adenocarcinoma.
CONCLUSION: The lymphomas were commonly extra nodal, and infiltrative rather than mass-like. The recognition of the tumors was often delayed due to confusion with the known infectious complications in AT patients.

Entities:  

Mesh:

Year:  1999        PMID: 10199897     DOI: 10.1007/s002470050578

Source DB:  PubMed          Journal:  Pediatr Radiol        ISSN: 0301-0449


  6 in total

1.  Childhood colon cancer in a patient with ataxia telangiectasia.

Authors:  Kyeong Min Jo; Sung Yeun Yang; Jong Ha Park; Tae Oh Kim; Heui Jeong Jeong; Chang Min Heo; Ji Hoon Jang; So Chong Hur; Na Ri Jeong; Su Jin Jeong; Sang Hoon Seol; Kyung Han Nam
Journal:  Ann Transl Med       Date:  2016-01

Review 2.  A Comprehensive Review of Pediatric Tumors and Associated Cancer Predisposition Syndromes.

Authors:  Sarah Scollon; Amanda Knoth Anglin; Martha Thomas; Joyce T Turner; Kami Wolfe Schneider
Journal:  J Genet Couns       Date:  2017-03-29       Impact factor: 2.537

Review 3.  Immunogenetics of gastrointestinal cancers: A systematic review and retrospective survey of inborn errors of immunity in humans.

Authors:  Beishi Zheng; Michael G Artin; Howard Chung; Bing Chen; Siming Sun; Benjamin L May; Chin Hur; Peter H R Green; Timothy C Wang; Jiheum Park; Xiao-Fei Kong
Journal:  J Gastroenterol Hepatol       Date:  2022-04-13       Impact factor: 4.369

Review 4.  Challenges in Diagnosis and Treatment of Lung Cancer in People with Intellectual Disabilities: Current State of Knowledge.

Authors:  Daniel Satgé; Emmanuelle Kempf; Jean-Bernard Dubois; Motoi Nishi; Jean Trédaniel
Journal:  Lung Cancer Int       Date:  2016-09-26

5.  The natural history of ataxia-telangiectasia (A-T): A systematic review.

Authors:  Emily Petley; Alexander Yule; Shaun Alexander; Shalini Ojha; William P Whitehouse
Journal:  PLoS One       Date:  2022-03-15       Impact factor: 3.752

Review 6.  Gastric outlet obstruction due to adenocarcinoma in a patient with Ataxia-Telangiectasia syndrome: a case report and review of the literature.

Authors:  Iyore A Otabor; Shahab F Abdessalam; Steven H Erdman; Sue Hammond; Gail E Besner
Journal:  World J Surg Oncol       Date:  2009-03-12       Impact factor: 2.754

  6 in total

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