Arch Plast Surg.  2016 Jul;43(4):344-351. 10.5999/aps.2016.43.4.344.

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

• Sibar S
• Findikcioglu K
• Erdal AI
• Barut I
• Ozmen S

• KMID: 2344333
• DOI: http://doi.org/10.5999/aps.2016.43.4.344

Abstract

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.

Keyword

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

MeSH Terms

Carcinogenesis
Carcinoma, Squamous Cell
DNA Repair
Follow-Up Studies
Free Tissue Flaps
Head and Neck Neoplasms
Head*
Humans
Ichthyosis*
Lost to Follow-Up
Male
Neck*
Orbit
Recurrence
Siblings
Skin Neoplasms
Ultraviolet Rays
Xeroderma Pigmentosum*