Parotid secretory carcinoma with high-risk features in a paediatric patient: case report and review of the literature

Background: Paediatric secretory carcinoma (SC) of the salivary glands (SCSG) is very rare. The natural history and optimal management of paediatric SCSG, particularly in the subset of cases with high-risk pathological features, remains unclear. Case Description: We report on a 16-year-old female diagnosed with a left parotid SC—proven on histopathology and fluorescence in situ hybridisation (FISH) testing—complicated by lymphovascular invasion (LVI), perineural invasion (PNI), and nodal metastasis. The patient was managed with left total parotidectomy, left II–V neck dissection and adjuvant radiotherapy to the left parotid bed and neck. She remains disease-free 12 months postoperatively. A literature review of published cases of paediatric SCSG on PubMed and Embase was also performed to evaluate aspects of natural history and management for this tumour subtype, with a focus on cases with high-risk features. Conclusions: Of the 52 published cases of paediatric SCSG, including the case presented herein, nine cases had evidence of local invasion, nodal spread or locoregional recurrence, or required multimodality treatment. There is a limited body of evidence to guide treatment decisions in paediatric SCSG, especially for patients with high-risk features. Further research is required to clarify the natural history and determine the role of multimodality treatment for children with this very rare tumour subtype.