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Radiation therapy for squamous cell cancer of the skin in a patient with xeroderma pigmentosum

1 Department of Radiation Oncology, Umraniye Training and Research Hospital, İstanbul, Turkey
2 Department of Radiation Oncology and Plastic, Reconstructive, and Aesthetic Surgery, Faculty of Medicine, Bezmialem Vakif University, İstanbul, Turkey

Date of Submission26-Apr-2020
Date of Decision08-Jun-2020
Date of Acceptance01-Oct-2020
Date of Web Publication15-Jun-2022

Correspondence Address:
Pelin Altinok,
Department of Radiation Oncology, Umraniye Training and Research Hospital, Elmalikent Mahallesi, Adem Yavuz Cd. No: 1, 34766 Umraniye, Istanbul
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcrt.JCRT_534_20

 > Abstract 

Xeroderma Pigmentosum is an autosomal recessive disease characterized by increased sensitivity to ultraviolet radiation. Adjuvant radiotherapy (RT) is an important locoregional treatment modality for high-risk skin squamous cell cancers (SCCs). We present a case of an adult with high-risk skin SCC treated with standard adjuvant RT and followed-up for >4 years with acceptable side effects.

Keywords: Radiation therapy, squamous cell skin cancer, xeroderma Pigmentosum

How to cite this URL:
Altinok P, Tekçe E, Ergün SS, Mayadagli A. Radiation therapy for squamous cell cancer of the skin in a patient with xeroderma pigmentosum. J Can Res Ther [Epub ahead of print] [cited 2022 Aug 16]. Available from: https://www.cancerjournal.net/preprintarticle.asp?id=347678

 > Introduction Top

Xeroderma Pigmentosum (XP) is an autosomal recessive disease characterized by increased sensitivity to ultraviolet (UV) radiation.[1] Because of defective mechanisms of DNA repair in patients with XP, the frequency of cutaneous malignancies are 1000 times higher than the normal population.[2]

Adjuvant radiotherapy (RT) is an important locoregional treatment modality for high-risk skin squamous cell cancers (SCCs) with invasion to the muscle, cartilage, or bone, perineural involvement (PNI) and lymph node metastasis. Nevertheless, there is no standard approach for patients with XP.

We present a case of an adult with high-risk skin SCC treated with standard adjuvant RT.

 > Case Report Top

A 48-year-old male patient applied to our clinic in April 2015. He had a diagnosis of XP since he was 14 years old and he had multiple surgical procedures for intradermal SCC and basal cell carcinoma. During his last visit in November 2014, an unhealed lesion on his right malar region was excised. The diagnosis was intradermal SCC with negative margins, so the patient was followed up. On his next examination in February 2015, a new lesion on the right ala was identified. The lesion was spreading to the right infraorbital region and zygoma with the invasion of the maxilla, orbital floor, palatine bone, and vomer [Figure 1] and [Figure 2].
Figure 1: Preoperative aspect of the patient

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Figure 2: Magnetic resonance imaging showing the extension of disease to the inferior wall of the right orbital, the anterior wall of the maxillary sinus, and hard palate. No pathologic lymph node was identified

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Macroscopic total excision of the tumor by maxillectomy and flap repair was performed on March 17, 2015. A poorly differentiated SCC with extensive PNI was the diagnosis. The tumor was 5.8 cm in diameter infiltrating the spina nasalis anterior superior and adjacent soft tissues and bones. Margins were positive on medial, lateral, superior sites of the specimen, and on the side of the maxillary sinus. Re-excision would cause major comorbidity and the patient refused the right orbital exenteration also. Thus, additional surgery was canceled and the patient was admitted to have postoperative RT. Possible acute and late side effects were described to the patient in detail and informed written consent was obtained. The tumor bed and the regional lymphatics were irradiated between May 12 and July 1, 2015. RT was applied by a linear accelerator with 6 MV photons and wielding the dynamic intensity-modulated radiation therapy technique. In the first phase of the RT, the tumor bed and the regional lymphatics got 50 Gy in 25 fractions. A boost dose of 16 Gy in 8 fractions was given to the tumor bed. A 0.5 cm bolus material was used for the entire RT application [Figure 3]. After the 20th fraction, the treatment was interrupted for consecutive 10 days (including weekend days) due to Grade 3 mucositis and esophagitis.[3] A combination of sucralfate suspension, metamizole (5%) drops and chlorhexidine digluconate (1.12%) plus benzydamine hydrochloride (0.15%) oral rinse solution were used. Grade 1 acute radiation dermatitis was also seen and dealed with dexpanthenol lotion.
Figure 3: Isodose distributions of the first (a) and second (b-boost) phases of the radiation therapy

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The patient was followed up with physical examination and magnetic resonance imaging scans every 3 months for the first 2 years and biannually thereafter. He lost his right vision approximately 1 year after the completion of therapy. Mild cutaneous fibrosis was observed. The last scanning and physical examination were performed on October 2019 [Figure 4]. He still has no evidence of recurrent or metastatic disease.
Figure 4: The patient's appearance at his last visit

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 > Discussion Top

There are a few patients in the literature who were treated with RT for miscellaneous disease sites.[4] In terms of cutaneous malignancies of the patients with XP, RT could be given without serious acute side effects.[5] However, no skin bolus material usage was mentioned for these cases. The technique, doses, and fields varied. The applications of RT >50 Gy were generally used electrons to protect the subcutaneous tissue. Atrophy and telangiectasis in the skin are the most common late side effects.[6],[7],[8] Very few patients had in-field recurrences after RT for skin cancer.

As far as we know, our case is the only irradiation application in the literature which used a radical dose with a bolus in the entire treatment process. Furthermore, he was followed up for >4 years which is a period long enough to observe the long-term side effects.

The main form of a genetic defect in XP is nucleotide excision repair.[9] There are a number of different complementation groups of XP and radiation sensitivity may differ both in vivo and in vitro.[4] Ionizing radiation mainly causes double-strand breaks, single-strand breaks, and base lesions within short DNA.[10] The altered mechanism of the radiation damage may be the cause of different endpoints of radiation exposure to UV and therapeutic radiation in patients with XP. Determining the DNA repair defect and complementation group before the initiation of RT or testing the cellular sensitivity with a small initial dose may be a way of avoiding side effects of radiation treatment.[4] However, it should be noted that RT is one of the primary treatment modalities for high-risk cutaneous SCC and the risk of a fatal recurrence should always be taken into account when trying to avoid side effects.

Standard RT approach seems to be safe in the treatment of skin cancer in patients with XP.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.


The authors declare that no external fundings were received for this study, therefore there is no conflict of interest from third parties. The authors do not have any financial relationship with any organization. The study conforms to the principles outlined in the Helsinki Declaration.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

 > References Top

Lehmann J, Seebode C, Martens MC, Emmert S. Xeroderma Pigmentosum-Facts and Perspectives. Anticancer Res 2018;38:1159-64.  Back to cited text no. 1
Budden T, Bowden NA. The role of altered nucleotide excision repair and UVB-induced DNA damage in melanomagenesis. Int J Mol Sci 2013;14:1132-51.  Back to cited text no. 2
Common Toxicity Criteria for Adverse Events 4.0 XLS document. Available from: https://www.rtog.org/researchassociates/ adverseeventreporting.aspx. [Last accessed on 2020 Apr 24].  Back to cited text no. 3
Schaffer JV, Orlow SJ. Radiation therapy for high-risk squamous cell carcinomas in patients with xeroderma pigmentosum: Report of two cases and review of the literature. Dermatology 2011;223:97-103.  Back to cited text no. 4
Kim R, Brascho DJ, Lawson AJ. Xeroderma pigmentosum in radiation oncology practice. Int J Radiat Oncol Biol Phys 1982;8:313.  Back to cited text no. 5
Lasso JM, Yordanov YP, Pinilla C, Shef A. Invasive basal cell carcinoma in a xeroderma pigmentosum patient: Facing secondary and tertiary aggressive recurrences. J Craniofac Surg 2014;25:e336-8.  Back to cited text no. 6
Adu EJ. Xeroderma pigmentosum in Ghanaians: A report of three cases and review of literature. West Afr J Med 2014;33:82-5.  Back to cited text no. 7
Ben Salah H, Bahri M, Turki H, Abdelmoula M, Frikha M, Daoud J. Radiotherapy for cutaneous cancers with xeroderma pigmentosum. Cancer Radiother 2011;15:400-3.  Back to cited text no. 8
Cleaver JE. Common pathways for ultraviolet skin carcinogenesis in the repair and replication defective groups of xeroderma pigmentosum. J Dermatol Sci 2000;23:1-1.  Back to cited text no. 9
Mavragani IV, Nikitaki Z, Souli MP, Aziz A, Nowsheen S, Aziz K, et al. Complex DNA damage: A route to radiation induced genomic instability and carcinogenesis. Cancers (Basel) 2017;9:91.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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