Tumor syndromes predisposing to osteosarcoma Journal Article


Authors: Hameed, M.; Mandelker, D.
Article Title: Tumor syndromes predisposing to osteosarcoma
Abstract: Osteosarcoma (OS) is the most common primary bone tumor affecting predominantly adolescents and young adults. It accounts for about 5% of all childhood cancers. Although the majority of OSs are sporadic, a small percentage occur as a component of hereditary cancer syndromes. Early onset, bilateral, multifocal, and metachronous tumors suggest genetic predisposition. The inheritance patterns can be autosomal dominant or recessive. These syndromes predispose to a wide variety of mesenchymal and epithelial cancers with propensity for certain mutations being prevalent in specific cancer subtypes. Li-Fraumeni syndrome, retinoblastoma, Rothmund-Thompson syndrome (type 2), Werner syndrome, and Bloom syndrome, constitute the majority of the tumor syndromes predisposing to OS and will be the focus for this review. © 2018 Wolters Kluwer Health, Inc. All rights reserved.
Keywords: osteosarcoma; cancer predisposition; hereditary syndromes
Journal Title: Advances in Anatomic Pathology
Volume: 25
Issue: 4
ISSN: 1072-4109
Publisher: Lippincott Williams & Wilkins  
Date Published: 2018-07-01
Start Page: 217
End Page: 222
Language: English
DOI: 10.1097/pap.0000000000000190
PROVIDER: scopus
PUBMED: 29668499
PMCID: PMC6688172
DOI/URL:
Notes: Publisher-provided DOI does not resolve (as of 7/24/2018) -- Review -- Export Date: 2 July 2018 -- Source: Scopus
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  1. Meera Hameed
    281 Hameed
  2. Diana Lauren Mandelker
    178 Mandelker