title: Salivary gland carcinomas in children and adolescents: the Italian TREP project experience.
abstract: BACKGROUND
  Salivary gland carcinomas are extremely rare in pediatric age. We report the clinical features of a series of children/adolescents with salivary gland carcinomas prospectively registered in the Italian TREP (Rare Tumors in Pediatric Age) project.
  
  
  METHODS
  Diagnostic/therapeutic guidelines were developed and shared among Italian pediatric oncology/surgical centers.
  
  
  RESULTS
  Seventeen patients were registered between 2000 and 2012, representing 19% of the cases expected to be seen based on epidemiological data. Tumors arose mainly in the parotid gland (14 cases). In most cases they were low-grade tumors (14 cases), often with a favorable clinical presentation, and low-stage disease. All patients underwent surgical resection, achieving histologically free margins in 9/17 cases. Thirteen of the 14 patients with parotid gland tumors had parotidectomy (10 total, 3 superficial), while one had a tumorectomy. Postoperative facial nerve lesions were reported in two cases. Adjuvant radiotherapy was given to 6 patients. The overall prognosis was good: only one patient with a huge high-grade tumor experienced disease progression and died of the disease. The other 16 patients were alive in first continuous remission 1-8 years after diagnosis. In 4/17 cases, the salivary gland carcinoma was a second tumor occurring 6-9 years after another primary cancer.
  
  
  CONCLUSIONS
  This is the first reported prospective national cooperative series of pediatric salivary gland carcinoma patients. Compliance with the TREP recommendations was high. These tumors are rarely managed by pediatric oncologists/surgeons. A broader international cooperation and better networking with otolaryngologists and head-neck surgeons expert on adult salivary gland carcinomas would be advisable.
authors.affiliation: Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Chiaravalli
authors.forename: Marco
authors.initials: M
authors.lastname: Guzzo
authors.forename: Gianni
authors.initials: G
authors.lastname: Bisogno
authors.forename: Maria Debora
authors.initials: MD
authors.lastname: De Pasquale
authors.forename: Roberta
authors.initials: R
authors.lastname: Migliorati
authors.forename: Francesco
authors.initials: F
authors.lastname: De Leonardis
authors.forename: Paola
authors.initials: P
authors.lastname: Collini
authors.forename: Michela
authors.initials: M
authors.lastname: Casanova
authors.forename: Giovanni
authors.initials: G
authors.lastname: Cecchetto
authors.forename: Andrea
authors.initials: A
authors.lastname: Ferrari
mesh_terms: D000293:Adolescent; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D011446:Prospective Studies; D012468:Salivary Gland Neoplasms / Q000473:pathology* / Q000628:therapy
keywords: TREP project; adolescents; carcinoma; children; rare pediatric tumors; salivary glands