title: Myoepithelial carcinoma treatment in children: a report from the TREP project.
abstract: BACKGROUND
  Myoepithelial carcinoma (MC) of soft tissues is an aggressive tumor that rarely affects children, for whom no established treatment protocols exist. As part of the TREP (Tumori Rari in Età Pediatrica) project - an Italian network dedicated to children and adolescents with very rare tumors - we present a series of patients with MC, who were treated homogeneously and achieved a satisfactory outcome.
  
  
  METHODS
  From 2005 to 2012, seven patients (age 0.5-9.2 years) with a diagnosis of MC were registered in the TREP study. After one patient treated with ifosfamide, cisplatin, and etoposide showed tumor shrinkage and experienced long-term disease remission, all subsequent patients were treated with the same chemotherapy regimen. All patients also received radiotherapy.
  
  
  RESULTS
  Initial surgical management involved a biopsy in three cases and tumor resection in 4. Response to initial chemotherapy was evaluable in four patients: two had a partial remission, one a minor response and one stable disease. Four patients received external-beam radiotherapy and three had brachytherapy. Overall, six patients are alive in first complete remission with a median follow-up of 2.5 years (0.9-5.1 years).
  
  
  CONCLUSIONS
  Though our experience is limited to a small number of patients, our treatment strategy for patients with MC is appears clinically useful and demonstrates how cooperation within the TREP project has enabled enough data to be collected to propose treatment recommendations for pediatric patients with this very rare tumor.
authors.affiliation: Hematology/Oncology Division, Woman's and Child's Health Department, Padova University Hospital, Padova, Italy.
authors.forename: Gianni
authors.initials: G
authors.lastname: Bisogno
authors.forename: Arianna
authors.initials: A
authors.lastname: Tagarelli
authors.forename: Amalia
authors.initials: A
authors.lastname: Schiavetti
authors.forename: Giovanni
authors.initials: G
authors.lastname: Scarzello
authors.forename: Andrea
authors.initials: A
authors.lastname: Ferrari
authors.forename: Giovanni
authors.initials: G
authors.lastname: Cecchetto
authors.forename: Rita
authors.initials: R
authors.lastname: Alaggio
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D002945:Cisplatin / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D005472:Fluorouracil / Q000008:administration & dosage; D005500:Follow-Up Studies; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D007223:Infant; D008297:Male; D009208:Myoepithelioma / Q000188:drug therapy* / Q000473:pathology; D009367:Neoplasm Staging; D011379:Prognosis; D012074:Remission Induction
chemical_list: D005047:Etoposide; D002945:Cisplatin; D005472:Fluorouracil; D007069:Ifosfamide
keywords: TREP project; myoepithelial carcinoma; rare tumors