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Begoña GRAÑA-SUÁREZ1Xavier HERNANDEZ-YAGÜE1 Xeroderma pigmentosum is a genodermatosis characterized by defects in the DNA nucleotide excision repair pathway, which increases the Hospital Josep Trueta,AV Francia s/n 17007 Girona. Spain risk of suffering skin cancers. There is lack of information about the efficacy and toxicity of chemotherapy and radiotherapy in the treat- ment of these patients. We present the case of a xeroderma pigmento- sum patient with a neck node recurrence of a squamous cell skin carcinoma that achieved a good response and survival with cetuximab.
Key words: cetuximab, skin cancer, squamous cell carcinoma, Reprints: J.R. Casadevall<jrubio@ico.scs.es>Article accepted on 7/10/2008 X erodermapigmentosum(XP)isarareautosomal tion chemotherapy followed by radiation and/or surgery, defects in the DNA nucleotide excision repair Although the effect of loss of the NER pathway on the (NER) pathway. Patients are at an increased risk of suffer- response to therapeutic agents is not well known, we ing skin cancers in sun-exposed sites, due to faulty repair decided to use the schema: cisplatin 50 mg per m2 day 1, of ultraviolet light damage to DNA [1, 2]. We present a and 5-fluorouracil 1000 mg per m2 days 1-3, every two patient with XP diagnosed as having a primary squamous weeks, in order to avoid toxicity. After four cycles we cell skin carcinoma, spread to a neck node. The tumour stopped chemotherapy due to delayed grade 3 thrombo- was treated with chemotherapy, radiotherapy and, ulti- cytopenia and lack of objective response.
Three months after the initial consultation the patientbegan radiotherapy, receiving a total dose of 50 Gy. Themetastatic neck node decreased in diameter by more than 50%, achieving a partial response without any relevantacute radiation side effect. Chemotherapy was continuedwith weekly vinorelbine, which was stopped because of A 37-year-old man presented for evaluation of a level II, refractory grade 2 leukopenia after 8 weeks of treatment.
fixed, growing, cervical node. He had been clinically The patient progressed only locally six months after the diagnosed with xeroderma pigmentosum early in his end of radiotherapy (figure 1A). A new TC excluded dis- infancy. His face showed multiple teleangiectases as tant metastases. Cetuximab was then started at a load dose well as atrophy and spotty hypopigmentations. He showed of 400 mg per m2 continuing with 250 mg per m2 every loss of vision due to clouding of the corneas. No relevant week. Immunohistochemical staining for the EGF recep- neurological abnormality had ever been reported. Several tor was not performed. After 12 weeks of treatment, the squamous and basal cell carcinomas had been surgically cutaneous fistula closed definitively, the patient decreased removed since he was an adolescent. Three months before the use of analgesia and he improved his quality of life. At consultation, a squamous cell carcinoma of his outer left this time, the node experienced a good clinical response ear was completely excised. A computed tomographic (figure 1B) and almost a 50% diameter reduction mea- scanning (CT) and a magnetic resonance imaging of the sured by computed tomography and magnetic resonance.
neck revealed a left 60 by 30 mm node with a cutaneous After eleven months of treatment with weekly cetuximab, fistula. The node infiltrated the sternocleidomastoid mus- patient suffered neck node enlargement and jaw bone cle and involved neck vascular structures. A fine needle infiltration. He was referred to palliative care, dying two aspiration of the node was performed, which confirmed years after his initial consultation.
the diagnosis of squamous cell carcinoma. Neither otolar- The patient was referred for genetic counselling before yngology endoscopies nor radiology image techniques dying. He had an older sister also affected by xeroderma revealed a new primary site. Therefore, he was diagnosed pigmentosum, who suffered from melanoma, a carcinoma as suffering an N3 node recurrence of a primary skin car- of the lower lip, several squamous cell skin carcinomas cinoma. Pre-treatment evaluation by a multidisciplinary and blindness. The pedigree shown consanguinity, the team dismissed surgery. It was decided to start with induc- xeroderma pigmentosum cases in the family resulted Xeroderma pigmentosum is characterized by a more than1000-fold increase in the frequency of all types of majorskin cancers (basal cell cancers, squamous cell cancers,malignant melanoma) in areas exposed to sunlight, com-pared to the normal population. The age of onset of thefirst skin cancers is 8 years-old [3]. The worldwide inci-dence of XP is 1: 250,000 live births, usually presentingat age 1-2 years with photosensitivity and burning afterminimal sun exposure. Cutaneous manifestations alsoinclude increasing dryness of skin, freckling, and telangi-ectasia. Ocular abnormalities include photophobia, ectro-pion, conjunctival injection, keratitis, and tumours. About20-30% of patients with XP also have neurological abnor-malities. XP patients may also be at increased risk for thedevelopment of internal neoplasias (brain tumors, sarco- mas, leukaemia and lung cancers). Although heterozy-gotes (carriers) are asymptomatic, it is unknown whetherthey have a higher risk of developing cancer comparing tonormal population [4].
Patients affected by XP should be referred for geneticcounselling and regular follow up by a multidisplinarymedical team early in life. Support and information forXP patients and their families is invaluable in order tohelp them to understand the condition and improve theprognosis and quality of life of these individuals [5].
The goal of treatment of xeroderma pigmentosum is tomake an early diagnosis and to protect the patient fromsunlight as there is no cure for this disease. Currently,there is a lack of information about the efficacy and safetyof conventional treatment in patients affected by XP andwith wide-spread cancer. The use of cisplatin in these patients could both increase the expected toxicity and effi-cacy, as the XP cells are unable to repair DNA adducts Figure 1. A) Before and B) after cetuximab induced by this agent [6, 7]. T4 endonuclease liposomelotion [8] and oral retinoids like isotretinoin [9] have a from marriage between cousins. The father had survived a role in the chemo-prevention of skin cancer and have Hodgkin disease diagnosed in his adolescence while the also been used concomitantly with radiotherapy with paternal grandfather died of lung cancer. Both our patient and his sister refused any molecular genetic testing. After Considering radiotherapy for XP patients, very few our patient’s death, his sister blamed her parents for their reports have been published and show conflicting out- XP diagnosis and refused any follow up by medical and comes: from no toxic events [10] to life-threatening side effects before ending radiotherapy [11].
Cetuximab is a monoclonal antibody against the epider-mal growth factor receptor (EGFR). EGFR is commonly overexpressed in squamous cell carcinomas of the headand neck and its genetic alterations are correlated withthe patient’s clinical outcome [12]. According to the liter- Xeroderma pigmentosum is an autosomal recessive ature published at the time we treated our patient [13] and photosensitive genodermatosis belonging to an expand- recently, [14] when it is used in monotherapy it could ing family of diseases with defects in the DNA nucleotide achieve a response rate of 13% and a disease control excision repair (NER) pathway. Patients have a genetic rate of 46%. It has also shown activity with cisplatin in inability to repair DNA damage induced by ultraviolet refractory patients [15]. Nowadays, cetuximab is accepted light. There are two NER systems: 1) transcription- as an option for first line treatment with concurrent radio- coupled repair, and 2) global genome repair. Many fac- theraphy for locally advanced head and neck cancer [16].
tors involved in these pathways are related to the patho- In this case, a recurrent squamous cell skin carcinoma genesis of XP [1]. Genetically, xeroderma pigmentosum diagnosed in an XP patient was treated with cetuximab is a very heterogeneous disease that can be subdivided as a monotherapy agent in a palliative setting. The use into seven complementation groups (XPA through XPG) of this targeted therapy facilitated a long stabilization of and a XP Variant (XPV). Recently, the isolation and char- the disease with very low toxicity. Cetuximab could be an acterization of the genes responsible for XP have made it acceptable treatment for XP patients affected with possible to use molecular biological techniques to diag- advanced squamous skin cell carcinomas. There are no nose XP patients, for carrier detection and for prenatal reports of the use of cetuximab concurrent with radiother- Acknowledgements. Authors disclose no conflict of sum: a randomised study. Xeroderma Pigmentosum Study Group. Lan- 9. Kraemer KH, DiGiovanna JJ, Peck GL. Chemoprevention of skin cancer in xeroderma pigmentosum. J Dermatol 1992; 19: 715-8.
10. DiGiovanna JJ, Patronas N, Katz D, et al. Xeroderma Pigmento- sum: spinal cord astrocytoma with 9 year survival alter radiotherapy and isotretinoin therapy. J Cutaneous Med Surg 1998; 2: 153-8.
11. Rogers PB, Plowman PN, Harris SJ, et al. Four radiation hyper- sensitivity cases and their implications for clinical radiotherapy.
12. Temam S, Kawaguchi H, El-Naggar AK, et al. Epidermal growth factor receptor copy number alterations correlate with poor clinical outcome in patients with head and neck squamous cancer. J Clin 1. Cleaver JE. Cancer in xeroderma pigmentosum and related disor- 13. Trigo JM, Hitt P, Koralewski E, et al. Cetuximab monotherapy is ders of DNA repair. Nat Rev Cancer 2005; 5: 564-73.
active in patients with platinum-refractory recurrent/metastatic squa- 2. Stary A, Sarasin A. The genetics of the hereditary xeroderma pig- mous cell carcinoma of the head and neck (SSCH): Results of a mentosum syndrome. Biochimie 2002; 84: 49-60.
phase II study. J Clin Oncol 2004; 22: 488S (suppl; abstr 5502).
3. Kraemer KH, Lee MM, Scotto J. Xeroderma Pigmentosum. Cutane- 14. Vermorken JB, Trigo J, Hitt R, et al. Open-label, uncontrolled, mul- ous, ocular and neurologic abnormalities in 830 published cases.
ticenter phase II study to evaluate the efficacy and toxicity of cetuxi- mab as a single agent in patients with recurrent and/or metastatic 4. Norgauer J, Idzko M, Panther E, et al. Xeroderma pigmentosum.
squamous cell carcinoma of the head and neck who failed to respond to platinum-based therapy. J Clin Oncol 2007; 25: 2171-7.
. Webb S. Xeroderma pigmentosum. BMJ 2008; 336: 444-6.
6. Zhen W, Evans MK, Haggerty CM, Bohr VA. Deficient gene spe- 15. Baselga J, Trigo JM, Bourhis J, et al. Phase II multicenter study of cific repair of cisplatin-induced lesions in Xeroderma Pigmentosum the antiepidermal growth factor receptor monoclonal antibody cetux- and Fanconi’s anemia cell lines. Carcinogenesis 1993; 14: 919-24.
imab in combination with platinum-based chemotherapy in patients 7. Kennedy RD, D’Andrea AD. DNA repair pathways in clinical prac- with platinum-refractory metastatic and/or recurrent squamous cell tice: lessons from pediatric cancer susceptibility syndromes. J Clin carcinoma of the head and neck. J Clin Oncol 2005; 23: 5568-77.
16. Bonner JA, Harari PM, Giralt J, et al. Radiotherapy plus cetuxi- 8. Yarosh D, Klein J, O’Connor A, et al. Effect of topically applied T4 mab for squamous-cell carcinoma of the head and neck. N Engl endonuclease V in liposomes on skin cancer in xeroderma pigmento-

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