Dyskeratosis Congenita and Squamous Cell Cancer of the Head and Neck: A Case Report and Systematic Review

xlomafota13 shared this article with you from Inoreader Dyskeratosis Congenita and Squamous Cell Cancer of the Head and Neck: A Case Report and Systematic Review Via otol rhinol Ann Otol Rhinol Laryngol. 2021 Oct 15:34894211047470. doi: 10.1177/00034894211047470. Online ahead of print. ABSTRACT OBJECTIVES: Dyskeratosis congenita (DC) is a progressive congenital disorder that predisposes patients to squamous cell cancers (SCC) of the head and neck. We report a case of a patient who underwent primary osteocutaneous free flap for mandibular SCC followed by additional treatments for positive margins and discuss a systematic review on therapeutic managem ent for this patient population. METHODS: Case report of a 39-year-old male with DC who underwent resection and reconstruction with a fibular free flap for mandible SCC, followed by revision surgery and adjuvant radiotherapy for positive margins. A systematic review was completed afterward with the following terms: "dyskeratosis congenita" AND "oral cancer" OR "head and neck" OR "otolaryngology" on Medline and Web of Science for articles between 1980 and 2021. In total, 12 articles were included that reported on DC and SCC in the head and neck. RESULTS: Of the case reports that were included in this review, half the patients had recurrence within 1 year of primary treatments. Only 2 patients did not require revision surgery, adjuvant, or salvage therapy. Half of patients that received radiation therapy had severe side effects. CONCLUSIONS: This is the largest review of DC and SCC in the head and neck. Based off our case report and review, these patients have aggres sive disease that often requires multi-modality treatment. Consideration should be taken in regards to reports of side effects with radiation therapy. PMID:34651516 | DOI:10.1177/00034894211047470 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.