Salivary Gland Neoplasms in Children and Adolescents

Abstract

Salivary gland neoplasms (SGNs) in children are uncommon. Epithelial SGNs (ESGNs) comprise the majority (95%), with the remaining being mesenchymal SGNs (MeSGNs). Pleomorphic adenoma is the most frequently encountered benign neoplasm, mucoepidermoid carcinoma is the most frequent malignant ESGN, and rhabdomyosarcoma is the most frequent malignant MeSGN. ESGN presents in the second decade, whereas MeSGN presents in the first and second decades. Swelling without pain or neurological signs is the main presentation of both benign and malignant neoplasms. Making an accurate preoperative histological diagnosis is important, so a needle biopsy or a perioperative frozen section is useful when there is doubt about the disease status of the patient; the excised tumour margin is also important. Surgical excision should aim to achieve clear margin excision in benign and malignant ESGNs, minimising the need for adjuvant radiotherapy and maximising the long-term likelihood of patient cure. Benign ESGNs are uncommon, and excision is curative, whereas malignant ESGN and MeSGN should be managed by a multidisciplinary paediatric oncology team.

Survival and characteristics of pediatric salivary gland cancer: A systematic review and meta‐analysis

“…One‐fifth of the children experienced pain in conjunction with palpable masses, however facial nerve involvement was rare, which is in agreement with previous reviews . Previous reviews note that children experience symptoms of salivary gland masses for at least 8‐12 months prior to diagnosis . Furthermore, with fine needle cytology, there is a risk of misdiagnosing masses as benign tumors, when they are in fact malignant .…”

“…Radomski et al found a lower 5‐year overall survival (∼84%) with adjuvant radiotherapy in children versus surgery as the only treatment modality (∼98%) . However, the authors did not adjust for tumor grade or stage, which is important considering adjuvant radiotherapy is often reserved for highly aggressive or recurrent tumors . Of the seven studies that estimated 5‐year overall survival, four studies had a survival less than 100%.…”

“…Children with SGC most commonly present with a slowly enlarging mass in the major salivary glands or oral cavity over the past 8‐12 months without metastases . Diagnosis is often delayed due to the asymptomatic nature and rarity of the disease, and knowledge on salivary gland tumors in children is sparse …”

“…Considering histopathology, pediatric SGC is usually of epithelial origin, the most common being mucoepidermoid carcinoma (MEC), acinic cell carcinoma (AciCC), and adenoid cystic carcinoma (AdCC) …”

“…Pediatric SGC is often of lower grade in children older than 10 years and higher grade in children younger than 10 years, which leads to a worse prognosis in younger children …”