title: Oral Cancer-related Inherited Cancer Syndromes: A Comprehensive Review.
abstract: Oral squamous cell carcinoma is the most common malignancy of the oral cavity, which is usually preceded by a myriad of oral potentially malignant disorders (OPMDs). In the classification of OPMDs, inherited cancer syndromes (ICSs) were proposed as one of the categories. Inherited cancer syndromes are genetic disorders in which inherited genetic mutation in one or more genes predispose the affected individuals to the development of cancer and may also cause its early onset. Many of these syndromes are caused by mutations in tumor suppressor genes, oncogenes, and genes involved in angiogenesis. General dental practitioners frequently come across OPMDs in their day-to-day practice. It becomes of paramount importance to have knowledge about these rare but prognostically important OPMDs. With this view in mind, in this article, efforts have been made to comprehensively discuss about various ICSs that have higher potential of transformation into oral cancer. The ICSs discussed in this article are xeroderma pigmentosum (XP), ataxia telangiectasia (AT), Bloom syndrome (BS), Fanconi's anemia (FA), and Li-Fraumeni syndrome (LFS), with special emphasis on signs, symptoms, and genetic considerations.
authors.affiliation: Associate Professor, Department of Oral Pathology and Microbiology, Dr. D.Y. Patil Dental College & Hospital, Dr. D.Y. Patil Vidyapeeth, Pune Maharashtra, India, Phone: +919823871462, e-mail: gargi14@gmail.com.
authors.forename: Gargi S
authors.initials: GS
authors.lastname: Sarode
authors.affiliation: Department of Oral Pathology and Microbiology, Dr. D.Y. Patil Dental College & Hospital, Dr. D.Y. Patil Vidyapeeth, Pune Maharashtra, India.
authors.forename: Akshit
authors.initials: A
authors.lastname: Batra
authors.affiliation: Department of Oral Pathology and Microbiology, Dr. D.Y. Patil Dental College & Hospital, Dr. D.Y. Patil Vidyapeeth, Pune Maharashtra, India.
authors.forename: Sachin C
authors.initials: SC
authors.lastname: Sarode
authors.affiliation: Department of Orthodontics and Dentofacial Orthopedics Dr. D.Y. Patil Dental College & Hospital, Dr. D.Y. Patil Vidyapeeth Pune, Maharashtra, India.
authors.forename: Sujata
authors.initials: S
authors.lastname: Yerawadekar
authors.affiliation: Department of Diagnostic Sciences, Division of Oral Pathology College of Dentistry, Jazan University, Jazan, Kingdom of Saudi Arabia.
authors.forename: Shankargouda
authors.initials: S
authors.lastname: Patil
mesh_terms: D020022:Genetic Predisposition to Disease; D006801:Humans; D009062:Mouth Neoplasms / Q000235:genetics*; D009386:Neoplastic Syndromes, Hereditary / Q000235:genetics*
keywords: Ataxia telangiectasia; Bloom syndrome; Fanconi's anemia; Inherited cancer syndromes; Li-Fraumeni syndrome; Xeroderma pigmentosum.