Paediatric salivary gland cancer in Finland
ABSTRACT Worldwide, only 5% of all salivary gland tumours are reported in children and 35-50% of these are malignant. According to the Finnish Cancer Registry, 15 children were diagnosed with salivary gland cancer (SGC) during 1990-2009 in Finland. There are no previous studies available on the histopathology, treatment, and survival of patients with these rare malignancies in Finland.
Retrospective patient and tumour data covering the past 20 years were retrieved from the five University Hospitals in Finland. Ten paediatric SGC patients with a median age of 14 years (range, 9-19 years) and with available adequate patient data were included. The series comprised four boys and six girls. Follow-up time varied from five months to 14 years.
Two patients had a history of a previous malignancy. The majority of cases had a parotid gland cancer (7/10), and mucoepidermoid carcinoma was the most common histological subtype (5/10). All patients presented with Stage I-II disease and were treated surgically with curative intent. One patient with a Stage I mucoepidermoid carcinoma of the parotid gland received postoperative radiotherapy. No recurrences were encountered and all patients were free of disease at the last follow up.
The individual treatment design for paediatric SGC should follow consistent treatment strategies. All patients in the present study had low-grade tumours with a favourable prognosis. Since paediatric SGC is infrequent worldwide, the diagnostics, treatment, and follow up should be centralized in multidisciplinary Head and Neck Centres.
[Show abstract] [Hide abstract]
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.ProceduresDiagnostic/therapeutic guidelines were developed and shared among Italian pediatric oncology/surgical centers.ResultsSeventeen 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. Pediatr Blood Cancer © 2014 Wiley Periodicals, Inc.Pediatric Blood & Cancer 08/2014; 61(11). DOI:10.1002/pbc.25139 · 2.35 Impact Factor
Article: Salivary Gland Diseases in Childhood[Show abstract] [Hide abstract]
ABSTRACT: Salivary gland diseases in children are rare, apart from viral--induced diseases. Nevertheless, it is essential for the otolaryngologist to recognize these uncommon findings in children and adolescents and to diagnose and initiate the proper treatment. The present work provides an overview of the entire spectrum of congenital and acquired diseases of the salivary glands in childhood and adolescence. The current literature was reviewed and the results discussed and summarized.Besides congenital diseases of the salivary glands in children, the main etiologies of viral and bacterial infections, autoimmune diseases and tumors of the salivary glands were considered. In addition to the known facts, new developments in diagnostics, imaging and therapy, including sialendoscopy in obstructive diseases and chronic recurrent juvenile sialadenitis were taken into account. In addition, systemic causes of salivary gland swelling and the treatment of sialorrhoea were discussed. Although salivary gland diseases in children are usually included in the pathology of the adult, they differ in their incidence and some-times in their symptoms. Clinical diagnostics and especially the surgical treatment are influenced by a stringent indications and a less invasive strategy. Due to the rarity of tumors of the salivary glands in children, it is recommended to treat them in a specialized center with greater surgical experience.Altogether the knowledge of the differential diagnoses in salivary gland diseases in children is important for otolaryngologists, to indicate the proper therapeutic approach.Laryngo-Rhino-Otologie 03/2014; 93 Suppl 1:S103-25. DOI:10.1055/s-0033-1363969 · 0.82 Impact Factor
[Show abstract] [Hide abstract]
ABSTRACT: The management of pediatric salivary gland cancer (SGC) remains a challenge, and long-term outcome data are lacking for these rare tumors. The heterogeneity of histopathological features in 24 different SGCs further complicates unequivocal treatment recommendations across different age groups. This review aims to outline the management guidelines for pediatric SGC. Compared with adults, pediatric SGC is more often localized to the primary site and of low-grade histology. Surgery remains the treatment of choice accompanied by oncological treatment in selected patients. Elective neck dissection seems unnecessary as the occurrence of occult metastases is rare. This review provides information for clinicians to manage pediatric SGC highlighting the need for individually based treatment decisions at multidisciplinary head and neck centers.Current opinion in otolaryngology & head and neck surgery 01/2014; DOI:10.1097/MOO.0000000000000026 · 1.39 Impact Factor