Dyskeratosis congenita and squamous cell carcinoma of the mandibular alveolar ridge.


Journal

BMJ case reports
ISSN: 1757-790X
Titre abrégé: BMJ Case Rep
Pays: England
ID NLM: 101526291

Informations de publication

Date de publication:
11 May 2021
Historique:
entrez: 12 5 2021
pubmed: 13 5 2021
medline: 14 5 2021
Statut: epublish

Résumé

Dyskeratosis congenita is a rare disease caused by telomerase dysfunction classically characterised by the triad: skin pigmentation, nail dystrophy and mucosal leukoplakia. Few cases are described in literature regarding patients with head and neck squamous cell carcinoma affected by dyskeratosis congenita, and the therapeutic decisions are not yet well defined. A review of the literature of the last 20 years (2001-2021) was performed, and it was analysed the case of a 38-year-old male patient affected by dyskeratosis congenita diagnosed with a squamous cell carcinoma of the inferior alveolar ridge, treated with surgery. The absence of complications and the good postoperative recovery of the patient comfort in saying that resection and reconstructive surgery can be safely performed. The occurrence of disseminated disease 6 months after the treatment warns about the extreme aggressiveness of the pathology, its often systemic nature and the necessity of a multidisciplinary approach as well as further studies.

Identifiants

pubmed: 33975847
pii: 14/5/e242459
doi: 10.1136/bcr-2021-242459
pmc: PMC8117988
pii:
doi:

Types de publication

Case Reports Journal Article Review

Langues

eng

Sous-ensembles de citation

IM

Informations de copyright

© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.

Déclaration de conflit d'intérêts

Competing interests: None declared.

Références

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Auteurs

Alfonso Manfuso (A)

Head and Neck Department, Operative Unit of Maxillo-Facial Surgery and Otolaryngology, IRCCS Ospedale Casa Sollievo della Sofferenza, San Giovanni Rotondo, Puglia, Italy.

Antonio Maria Risitano (AM)

Department of Clinical Medicine and Surgery, University of Naples Federico II School of Medicine and Surgery, Naples, Campania, Italy.

Chiara Copelli (C)

Head and Neck Department, Operative Unit of Maxillo-Facial Surgery and Otolaryngology, IRCCS Ospedale Casa Sollievo della Sofferenza, San Giovanni Rotondo, Puglia, Italy copkids@tin.it.
Department of Surgical Sciences, Operative Unit of Maxillo-Facial Surgery, University of Turin, Torino, Piemonte, Italy.

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