{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,20]],"date-time":"2026-03-20T15:46:20Z","timestamp":1774021580512,"version":"3.50.1"},"reference-count":24,"publisher":"Georg Thieme Verlag KG","issue":"04","license":[{"start":{"date-parts":[[2022,4,20]],"date-time":"2022-04-20T00:00:00Z","timestamp":1650412800000},"content-version":"vor","delay-in-days":2119,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2016,7,20]],"date-time":"2016-07-20T00:00:00Z","timestamp":1468972800000},"content-version":"tdm","delay-in-days":19,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["www.e-aps.org"],"crossmark-restriction":true},"short-container-title":["Arch Plast Surg"],"published-print":{"date-parts":[[2016,7]]},"abstract":"<jats:p>\nBackground  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.<\/jats:p><jats:p>\nMethods  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.<\/jats:p><jats:p>\nResults  The mean age of the patients was 32 years (range, 10\u201343) (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\u201325). Six patients were siblings with each other, 5 patients had local recurrences, and one patient was lost to follow-up.<\/jats:p><jats:p>\nConclusions  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.<\/jats:p>","DOI":"10.5999\/aps.2016.43.4.344","type":"journal-article","created":{"date-parts":[[2016,7,25]],"date-time":"2016-07-25T05:45:47Z","timestamp":1469425547000},"page":"344-351","update-policy":"https:\/\/doi.org\/10.5999\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Technical Aspects and Difficulties in the Management of Head and Neck Cutaneous Malignancies in Xeroderma Pigmentosum"],"prefix":"10.1055","volume":"43","author":[{"given":"Serhat","family":"Sibar","sequence":"additional","affiliation":[{"name":"Department of Plastic, Reconstructive and Aesthetic Surgery, Gazi University Hospital, Ankara, Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kemal","family":"Findikcioglu","sequence":"additional","affiliation":[{"name":"Department of Plastic, Reconstructive and Aesthetic Surgery, Gazi University Hospital, Ankara, Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ayhan Isik","family":"Erdal","sequence":"additional","affiliation":[{"name":"Department of Plastic, Reconstructive and Aesthetic Surgery, Gazi University Hospital, Ankara, Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ismail","family":"Barut","sequence":"additional","affiliation":[{"name":"Department of Plastic, Reconstructive and Aesthetic Surgery, Gazi University Hospital, Ankara, Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Selahattin","family":"Ozmen","sequence":"additional","affiliation":[{"name":"Department of Plastic, Reconstructive and Aesthetic Surgery, Koc University Hospital, Istanbul, Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2022,4,20]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","DOI":"10.1007\/978-3-642-97931-6","volume-title":"Dermatology","author":"O Braun-Falco","year":"2000"},{"key":"ref2","first-page":"1848","volume-title":"Heritable diseases with increased sensitivity to cellular injury","author":"KH Kraemer","year":"1999","edition":"5th ed"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1016\/S0094-1298(20)30611-8","article-title":"Nonpigmented premalignant lesions of the skin","volume":"14","author":"D Gard","year":"1987","journal-title":"Clin Plast Surg"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1562\/2004-07-29-IR-250.1","article-title":"Reconstruction of DNA repair-deficient xeroderma pigmentosum skin in vitro: a model to study hypersensitivity to UV light","volume":"81","author":"F Bernerd","year":"2005","journal-title":"Photochem Photobiol"},{"key":"ref5","first-page":"252","article-title":"On diseases of the skin including exanthemata","volume":"61","author":"F Hebra","year":"1874","journal-title":"New Sydenham Soc"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"768","DOI":"10.1101\/gad.13.7.768","article-title":"Molecular mechanism of nucleotide excision repair","volume":"13","author":"WL de Laat","year":"1999","journal-title":"Genes Dev"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1016\/S0065-2660(01)43004-5","article-title":"Xeroderma pigmentosum and related disorders: defects in DNA repair and transcription","volume":"43","author":"M Berneburg","year":"2001","journal-title":"Adv Genet"},{"key":"ref8","first-page":"138","article-title":"Kseroderma pigmentozum","volume":"2","author":"S Kilic","year":"2004","journal-title":"Guncel Pediatri"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1001\/archderm.1987.01660260111026","article-title":"Xeroderma pigmentosum: cutaneous, ocular, and neurologic abnormalities in 830 published cases","volume":"123","author":"KH Kraemer","year":"1987","journal-title":"Arch Dermatol"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"221","DOI":"10.7326\/0003-4819-80-2-221","article-title":"Xeroderma pigmentosum: an inherited diseases with sun sensitivity, multiple cutaneous neoplasms, and abnormal DNA repair","volume":"80","author":"JH Robbins","year":"1974","journal-title":"Ann Intern Med"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"1388","DOI":"10.1016\/j.neuroscience.2006.12.020","article-title":"Xeroderma pigmentosum, trichothiodystrophy and Cockayne syndrome: a complex genotype-phenotype relationship","volume":"145","author":"KH Kraemer","year":"2007","journal-title":"Neuroscience"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"1442","DOI":"10.1212\/WNL.55.10.1442","article-title":"Cockayne syndrome and xeroderma pigmentosum","volume":"55","author":"I Rapin","year":"2000","journal-title":"Neurology"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"e336","DOI":"10.1097\/SCS.0000000000000596","article-title":"Invasive basal cell carcinoma in a xeroderma pigmentosum patient: facing secondary and tertiary aggressive recurrences","volume":"25","author":"JM Lasso","year":"2014","journal-title":"J Craniofac Surg"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"1542","DOI":"10.1097\/SCS.0b013e31824e660e","article-title":"Facial resurfacing with a monoblock full-thickness skin graft after multiple malignant melanomas excision in xeroderma pigmentosum","volume":"23","author":"S Ozmen","year":"2012","journal-title":"J Craniofac Surg"},{"key":"ref15","doi-asserted-by":"crossref","first-page":"1121","DOI":"10.1097\/00006534-199106000-00018","article-title":"Facial resurfacing in xeroderma pigmentosum with monoblock full-thickness skin graft","volume":"87","author":"K Atabay","year":"1991","journal-title":"Plast Reconstr Surg"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"1290","DOI":"10.1097\/01.PRS.0000025230.84677.C7","article-title":"Is facial resurfacing with monobloc full-thickness skin graft a remedy in xeroderma pigmentosum?","volume":"110","author":"SS Ergun","year":"2002","journal-title":"Plast Reconstr Surg"},{"key":"ref17","doi-asserted-by":"crossref","first-page":"657","DOI":"10.1097\/00000637-199912000-00015","article-title":"New tumor formation on split-thickness skin grafted areas in xeroderma pigmentosum","volume":"43","author":"G Aslan","year":"1999","journal-title":"Ann Plast Surg"},{"key":"ref18","first-page":"238","article-title":"How to perform Mohs micrographic surgery?","volume":"49","author":"G Elcin","year":"2015","journal-title":"Arch Turkish Dermatol Venerol"},{"key":"ref19","first-page":"236","article-title":"Mohs micrographic surgery: what if there is no Mohs micrographic surgery in Turkey?","volume":"49","author":"G Elcin","year":"2015","journal-title":"Arch Turkish Dermatol Venerol"},{"key":"ref20","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1159\/000324509","article-title":"Radiation therapy for high-risk squamous cell carcinomas in patients with xeroderma pigmentosum: report of two cases and review of the literature","volume":"223","author":"JV Schaffer","year":"2011","journal-title":"Dermatology"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1177\/120347549800200308","article-title":"Xeroderma pigmentosum: spinal cord astrocytoma with 9-year survival after radiation and isotretinoin therapy","volume":"2","author":"JJ DiGiovanna","year":"1998","journal-title":"J Cutan Med Surg"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1146\/annurev.biochem.73.011303.073723","article-title":"Molecular mechanisms of mammalian DNA repair and the DNA damage checkpoints","volume":"73","author":"A Sancar","year":"2004","journal-title":"Annu Rev Biochem"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1111\/php.12385","article-title":"Development of effective skin cancer treatment and prevention in xeroderma pigmentosum","volume":"91","author":"WC Lambert","year":"2015","journal-title":"Photochem Photobiol"},{"key":"ref24","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1016\/S0748-7983(97)80141-2","article-title":"Malignant melanoma in xeroderma pigmentosum patients: report of five cases","volume":"23","author":"O Kocabalkan","year":"1997","journal-title":"Eur J Surg Oncol"}],"container-title":["Archives of Plastic Surgery"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.e-aps.org\/upload\/pdf\/aps-43-344.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/www.e-aps.org\/journal\/view.php?doi=10.5999\/aps.2016.43.4.344","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.5999\/aps.2016.43.4.344.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,4,20]],"date-time":"2022-04-20T23:49:32Z","timestamp":1650498572000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.5999\/aps.2016.43.4.344"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,7]]},"references-count":24,"journal-issue":{"issue":"04","published-online":{"date-parts":[[2022,4,20]]},"published-print":{"date-parts":[[2016,7]]}},"URL":"https:\/\/doi.org\/10.5999\/aps.2016.43.4.344","archive":["Portico","CLOCKSS"],"relation":{},"ISSN":["2234-6163","2234-6171"],"issn-type":[{"value":"2234-6163","type":"print"},{"value":"2234-6171","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,7]]},"assertion":[{"value":"2016-02-07","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2016-06-07","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2016-07-20","order":2,"name":"published","label":"Published online","group":{"name":"publication_history","label":"Publication History"}},{"value":"2016-07-15","order":3,"name":"published","label":"Print publication","group":{"name":"publication_history","label":"Publication History"}}]}}