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SHORT COMMUNICATION
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Advances in dermatology and venereology Acta Dermato-Venereologica
Electrochemotherapy for Non-melanoma Skin Cancer in a Child with Xeroderma Pigmentosum
Eszter BALTÁS 1 #, Erika KIS 1 #, Nikoletta NAGY 1 – 3, Nicolette SOHÁR 4, Erika VARGA 1, Márta SZÉLL 2, 3, Lajos KEMÉNY 1, 3 and Judit OLÁH 1 * Departments of 1 Dermatology and Allergology, 2 Medical Genetics and 4 Ophtalmology, University of Szeged, Korányi fasor 6, HU-6721 Szeged, and 3 MTA-SZTE Dermatological Research Group, Szeged, Hungary. * E-mail: lazarne. olah. judit @ med. u-szeged. hu
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These authors contributed equally to this manuscript and should be considered as first authors. Accepted Apr 27, 2017; Epub ahead of print Apr 27, 2017
Xeroderma pigmentosum( XP) is a potentially lifethreatening disease, in which avoidance of ultraviolet light( UV) is necessary( 1). In cases in which preventive measures fail, treatment of skin neoplasms is challenging and requires a multidisciplinary approach. Consecutive surgical interventions may result in devastating cutaneous defects, which determine the quality of life( QoL) of the patients. Electrochemotherapy( ECT) is a novel therapeutic option that ablates tumours with electrical current and simultaneously administered anticancer drugs( 2). In various cutaneous and subcutaneous neoplasia, this method has been proven to provide longlasting local control with minimal side-effects and good cosmetic results( 3, 4).
CASE REPORT
An 11-year-old girl with XP was referred to our dermato-oncology centre with advanced non-melanoma skin cancers( NMSCs) on her face. Xerosis, skin atrophy, freckling and numerous scars from previous surgical interventions were noted on the skin surface. Unfortunately, her photoprotection in the past had been inadequate. Numerous plaques, nodules and ulcers 5 – 10 mm in diameter had presented, with the clinical appearance of basal cell carcinomas( BCCs). A locally advanced squamous cell carcinoma( SCC) had destroyed the surrounding tissues around the left nostril. On the left thigh, a tumour resembling keratoacanthoma( KA) 15 mm in diameter with a central keratotic plug was growing rapidly. Ophthalmological examination revealed photophobia and ectropion. Neurological examination, intelligence quotient( IQ) testing, radiological imaging and laboratory results were without significant pathological findings. Genetic investigations were performed on the genomic deoxyribonucleic acid( DNA) isolated from a peripheral blood sample from the patient. Direct sequencing of the XPC gene revealed a recurrent disease-causing homozygous nonsense mutation( c. 463C / T; p. Arg155X) in the third exon( 5).
Since the SCC in her central facial region could not be resected without causing a devastating defect, our multidisciplinary tumour board considered other treatment options. Although there are no published data regarding ECT in XP-C or in childhood, we decided to explore this treatment modality in our case( 6). Our patient did not have risk factors for pulmonary fibrosis, which we had to consider before the administration of bleomycin. Prior to the procedure we obtained approval from the local ethics committee, the dermato-oncology and the paediatric-oncology tumour board. The patient’ s parents also gave their written informed consent. ECT sessions were carried out in our department in accordance with the European Standard Operating Procedure guidelines with the Cliniporator TM device( IGEA Ltd., Modena, Italy)( 7). Depending on the size and clinical feature of the tumours, different types of electrodes were chosen. In case of nodules smaller than 1.0 cm in diameter electrical pulses were delivered either using parallel arrays or, for exophytic lesions, plate electrodes. For larger nodules, hexagonal array needle electrodes were connected to the electroporation device. If required, a second run was delivered or the electrode was repositioned until complete tumour electroporation was obtained. A safety margin of 3 – 5 mm was treated around every nodule. Electrical parameters were as follows: for type II electrodes, 8 electric pulses of 1,000 V / cm amplitude over distance ratio and 100 ms duration, delivered at 1 or 5,000 Hz repetition frequency and, for type III electrodes, 96 electric pulses( 8 pulses per pair of needles) of 1,000 V / cm amplitude over distance ratio and 100 ms duration, delivered at 5,000 Hz repetition frequency. At each session, the patient received intravenous bleomycin( 15 mg / m 2). Electrical pulses were delivered to the tumours during the pharmacokinetic peak( 8 – 28 min following the administration of bleomycin). The ECT sessions were carried out under general anaesthesia, and the patient was monitored for the subsequent 24 h. During the follow-up period, dermoscopy, confocal microscopy and histology were used to detect residual tumours and new lesions. Response to treatment was assessed according to the World Health Organization( WHO) criteria at least 4 months after intervention. Sessions were repeated at 2-monthly intervals until a complete response( CR) was achieved.
The patient underwent 5 ECT sessions in one year. The mean number of treated tumours was 17 per session( 11 – 25), the mean diameter was 0.85 cm( 0.3 – 3.6 cm), and most of them( 82 %) were localized in the head and neck region. After 5 sessions of ECT, CR was achieved in 33( 97 %) of the treated tumours( Fig. 1 and Fig. S1 1). Progressive disease( PD) was noticed in one lesion( 3 %), a
Fig. 1. Clinical course before and after therapy.( a) Squamous cell carcinoma and basal cell carcinomas before treatment,( b) 8 weeks after the first session of electrochemotherapy( ECT),( c) 2 weeks and( d) 15 months after the second session of ECT.
doi: 10.2340 / 00015555-2687 Acta Derm Venereol 2017; 97: 962 – 964
This is an open access article under the CC BY-NC license. www. medicaljournals. se / acta Journal Compilation © 2017 Acta Dermato-Venereologica.