Infant with Parotid Sialoblastoma and Nevus Sebaceous, Treated with Surgery and Adjuvant Chemotherapy

Sialoblastoma is an extremely rare embryonal tumor derived from salivary gland primordial cells. Treatment usually consists of surgery alone; however, in some cases, chemotherapy is required and is administered with good response. We present a case of a 5-week-old girl diagnosed with a parotid gland tumor and co-existing nevus sebaceous on the face. Initial tumorectomy was microscopically non-radical and histopathology revealed sialoblastoma. The patient received adjuvant chemotherapy consisting of vincristine, actinomycin, and cyclophosphamide. Due to imaging studies being inconclusive regarding response and possible residual disease, a second surgery (total parotidectomy) was performed. The histopathology results showed fields of necrosis in the parotid gland but no neoplastic cells in the material. The patient remains under watchful observation and there is no evidence of relapse 12 months after the second surgery. The adjuvant chemotherapy regimen with vincristine, actinomycin, and cyclophosphamide is a viable option of treatment in children with sialoblastoma.