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CC BY-NC 4.0 · Arch Plast Surg 2016; 43(04): 344-351
DOI: 10.5999/aps.2016.43.4.344
Original Article

Technical Aspects and Difficulties in the Management of Head and Neck Cutaneous Malignancies in Xeroderma Pigmentosum

Serhat Sibar
Department of Plastic, Reconstructive and Aesthetic Surgery, Gazi University Hospital, Ankara, Turkey
,
Kemal Findikcioglu
Department of Plastic, Reconstructive and Aesthetic Surgery, Gazi University Hospital, Ankara, Turkey
,
Ayhan Isik Erdal
Department of Plastic, Reconstructive and Aesthetic Surgery, Gazi University Hospital, Ankara, Turkey
,
Ismail Barut
Department of Plastic, Reconstructive and Aesthetic Surgery, Gazi University Hospital, Ankara, Turkey
,
Selahattin Ozmen
Department of Plastic, Reconstructive and Aesthetic Surgery, Koc University Hospital, Istanbul, Turkey
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Background Xeroderma pigmentosum (XP) is an autosomal recessive disorder characterized by xerosis, ultraviolet light sensitivity, and cutaneous dyspigmentation. Due to defects in their DNA repair mechanism, genetic mutations and carcinogenesis inevitably occurs in almost all patients. In these patients, reconstruction of cutaneous malignancies in the head and neck area is associated with some challenges such as likelihood of recurrence and an aggressive clinical course. The aim of this study is to discuss the therapeutic options and challenges commonly seen during the course of treatment.

Methods Between 2005 and 2015, 11 XP patients with head and neck cutaneous malignancies were included in this study. Demographic data and treatment options of the patients were evaluated.

Results The mean age of the patients was 32 years (range, 10–43) (4 males, 7 females). The most common tumor type and location were squamous cell carcinoma (6 patients) and the orbital region (4 patients), respectively. Free tissue transfer was the most commonly performed surgical intervention (4 patients). The average number of surgical procedures was 5.5 (range, 1–25). Six patients were siblings with each other, 5 patients had local recurrences, and one patient was lost to follow-up.

Conclusions Although genetic components of the disease have been elucidated, there is no definitive treatment algorithm. Early surgical intervention and close follow-up are the gold standard modalities due to the tendency toward rapid tumor growth and possible recurrence. Treatment must be individualized for each patient. In addition, the psychological aspect of the disease is an important issue for both patients and families.

Keywords

Skin neoplasms - Head and neck neoplasms - Xeroderma pigmentosum - Free tissue flaps


Publication History

Received: 07 February 2016

Accepted: 07 June 2016

Article published online:
20 April 2022

© 2016. The Korean Society of Plastic and Reconstructive Surgeons. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonCommercial License, permitting unrestricted noncommercial use, distribution, and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes. (https://creativecommons.org/licenses/by-nc/4.0/)

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