Technical Aspects and Difficulties in the Management of Head and Neck Cutaneous Malignancies in Xeroderma Pigmentosum
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 pati...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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Thieme Medical Publishers, Inc.
2016-07-01
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| Series: | Archives of Plastic Surgery |
| Subjects: | |
| Online Access: | http://www.thieme-connect.de/DOI/DOI?10.5999/aps.2016.43.4.344 |
| _version_ | 1798041708837994496 |
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| author | Serhat Sibar Kemal Findikcioglu Ayhan Isik Erdal Ismail Barut Selahattin Ozmen |
| author_facet | Serhat Sibar Kemal Findikcioglu Ayhan Isik Erdal Ismail Barut Selahattin Ozmen |
| author_sort | Serhat Sibar |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-11T22:25:19Z |
| format | Article |
| id | doaj.art-264037c0929b497990fa5bb3a6cf8e69 |
| institution | Directory Open Access Journal |
| issn | 2234-6163 2234-6171 |
| language | English |
| last_indexed | 2024-04-11T22:25:19Z |
| publishDate | 2016-07-01 |
| publisher | Thieme Medical Publishers, Inc. |
| record_format | Article |
| series | Archives of Plastic Surgery |
| spelling | doaj.art-264037c0929b497990fa5bb3a6cf8e692022-12-22T03:59:45ZengThieme Medical Publishers, Inc.Archives of Plastic Surgery2234-61632234-61712016-07-01430434435110.5999/aps.2016.43.4.344690Technical Aspects and Difficulties in the Management of Head and Neck Cutaneous Malignancies in Xeroderma PigmentosumSerhat Sibar0Kemal Findikcioglu1Ayhan Isik Erdal2Ismail Barut3Selahattin Ozmen4Department of Plastic, Reconstructive and Aesthetic Surgery, Gazi University Hospital, Ankara, TurkeyDepartment of Plastic, Reconstructive and Aesthetic Surgery, Gazi University Hospital, Ankara, TurkeyDepartment of Plastic, Reconstructive and Aesthetic Surgery, Gazi University Hospital, Ankara, TurkeyDepartment of Plastic, Reconstructive and Aesthetic Surgery, Gazi University Hospital, Ankara, TurkeyDepartment of Plastic, Reconstructive and Aesthetic Surgery, Koc University Hospital, Istanbul, TurkeyBackground 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.http://www.thieme-connect.de/DOI/DOI?10.5999/aps.2016.43.4.344skin neoplasmshead and neck neoplasmsxeroderma pigmentosumfree tissue flaps |
| spellingShingle | Serhat Sibar Kemal Findikcioglu Ayhan Isik Erdal Ismail Barut Selahattin Ozmen Technical Aspects and Difficulties in the Management of Head and Neck Cutaneous Malignancies in Xeroderma Pigmentosum Archives of Plastic Surgery skin neoplasms head and neck neoplasms xeroderma pigmentosum free tissue flaps |
| title | Technical Aspects and Difficulties in the Management of Head and Neck Cutaneous Malignancies in Xeroderma Pigmentosum |
| title_full | Technical Aspects and Difficulties in the Management of Head and Neck Cutaneous Malignancies in Xeroderma Pigmentosum |
| title_fullStr | Technical Aspects and Difficulties in the Management of Head and Neck Cutaneous Malignancies in Xeroderma Pigmentosum |
| title_full_unstemmed | Technical Aspects and Difficulties in the Management of Head and Neck Cutaneous Malignancies in Xeroderma Pigmentosum |
| title_short | Technical Aspects and Difficulties in the Management of Head and Neck Cutaneous Malignancies in Xeroderma Pigmentosum |
| title_sort | technical aspects and difficulties in the management of head and neck cutaneous malignancies in xeroderma pigmentosum |
| topic | skin neoplasms head and neck neoplasms xeroderma pigmentosum free tissue flaps |
| url | http://www.thieme-connect.de/DOI/DOI?10.5999/aps.2016.43.4.344 |
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