Salivary gland carcinomas in children and adolescents: the Italian TREP project experience.
Pediatr Blood Cancer
; 61(11): 1961-8, 2014 Nov.
Article
en En
| MEDLINE
| ID: mdl-25132368
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. PROCEDURES 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.Palabras clave
Texto completo:
1
Bases de datos:
MEDLINE
Asunto principal:
Neoplasias de las Glándulas Salivales
Tipo de estudio:
Guideline
/
Observational_studies
Límite:
Adolescent
/
Child
/
Female
/
Humans
/
Male
Idioma:
En
Revista:
Pediatr Blood Cancer
Asunto de la revista:
HEMATOLOGIA
/
NEOPLASIAS
/
PEDIATRIA
Año:
2014
Tipo del documento:
Article
País de afiliación:
Italia