Author/Editor		Pohar-Marinšek, Živa; Anžič, Jožica; Jereb, Berta
Title		Evolving strategies in the treatment of childhood rhabdomyosarcoma: Slovenian experience
Translated title		Spremenjeni načini zdravljenja rabdomiosarkoma pri otrocih, slovenske izkušnje
Type		članek
Source		Radiol Oncol
Vol. and No.		Letnik 35, št. 4
Publication year		2001
Volume		str. 259-66
Language		eng
Abstract		Background. Neoadjuvant chemotherapy (Cht) has changed the treatment of rhabdomyosarcoma (RMS) in children. The purpose of our study was to review the children treated for RMS between 1974 and 1996. Patients and methods. Fifty-one children, 1-15 years old, were included. Primary sites of tumour were: head and neck 15, orbit 6, genitourinary 12, extremity 9, torso 5 and paratesticular 4. Twelve patients were in stage 1, 10 in stage II, 26 in stage 111 and 3 in stage IV. Of 43 histologically confirmed RMS 25 were embryonal, 13 alveolar, 1 botryoid, 1 spindle cell and 3 sarcoma NOS. In 8 patients, only fine needle aspiration biopsy (FNAB) was available. All patients had Cht, 29 neoadjuvant, 20 had surgery first, 40 had irradiation (RT), 2 stage IV patients had bone marrow transplant (ABMT). Multidrug Cht varied: VCR, AMD, and cyclophosphamide (VAC) were used in the 1970s, with Adriablastine (T2), methotrexat (MTX) and/or other drugs (T6, T11) in the 1980s; and in the 1990s, cyclophosphamide was replaced by ifosfamide (VAIA). The treatment was started with Cht in orbital and head and neck tumours and in the majority of genitourinary tumours, but surgery was first in paratesticular and in the majority of extremity tumours. Results. The 3 patients with stage IV disease died. Of those with localised tumour, 34 (70%) were alive and well 5 years after treatment, 80% stage I, 75% stage II and 61% stage III. One patient died of heart failure, 3 of Cht toxicity and 1 of intereurrent disease. Conclusions. (Abstract truncated at 2000 characters)
Summary		Izhodišča. Neoadjuvantna kemoterapija (KT) je spremenila način zdravljenja rabdomiosarkoma (RMS) otrok. Namen naše študije je bil analizirati rezultate zdravljenja pri otrocih, ki so bili zdravljeni v letih med 1974 in 1996. Bolniki in metode. V študijo smo vključili 51 otrok med enim in 15 letom starosti. Primarni tumorji so bili v področju glave in vratu pri 15, v orbiti pri 6, v genitourinskem področju pri 12, na udih pri 9, na trupu pri 5 in paratestikularno pri 4 bolnikih. Dvanajst bolnikov je imelo bolezen s stadijem I, 10 s stadijem II, 26 s stadijem III in 3 s stadijem IV. Med 43 histološko potrjenimi primeri RMS je imelo 25 bolnikov embrionalni podtip, 13 alveolarni, 1 botrioidni, 1 vretenastocelični in 3 sarkom brez nadalnje opredelitve. Pri 8 bolnikih je bila morfološka diagnoza opredeljena le iz vzorca aspiracijske biopsije s tanko iglo (ABTI). Vsi bolniki so prejeli KT, 29 neoadjuvantno, 20 je bilo najprej operiranih, 40 je bilo obsevanih (RT), 2 bonika z boleznijo v stadiju IV sta imela presaditev kostnega mozga. Kombinirana KT se je v različnih obdobjih razlikovala: z VRC, AMD in ciklofosfamidom (VAC) smo zdravili v 70-tih letih, kombinirano z tždriablastinom (T2), MTX in/ali drugimi kemoterapevtiki (T6, T11) v 80-tih letih, ciklofosfamid smo zamenjali z ifosfamidom v 90-tih letih (VAIA): S kemoterapijo smo pričeli pri tumorjih orbite, glave in vratu in pri večini tumorjev genitourinskega področja. Operacija je bila na prvem mestu pri paratestikularnih tumorjih in pri večini tumorjev na udih. Rezultati. Vsi trije bolniki z boleznijo v stadiju IV so umrli. Med bolniki z lokaliziranimi tumorji je bilo 34 (70 %) živih in brez znakov bolezni 5 let po pričetku zdravljenja: 80% bolnikov s stadijem I, 75 % s stadujem II in 61 % s stadijem III. En bolnik je umrl zaradi odpovedi srca, 3 zaradi posledic KT in eden zaradi spremljajoče bolezni. (Izvleček skrajšan pri 2000 znakih)
Descriptors		RHABDOMYOSARCOMA NEOPLASM STAGING CHILD SURVIVAL ANALYSIS