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ActaDV ActaDV
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 .