Secretory carcinoma of the parotid gland in childhood: A case report and literature review

Abstract

We report the first case of pediatric parotid gland secretory carcinoma harboring the ETV6::RET fusion gene in Japan. A 10-year-old boy presented with gradual enlargement of a parotid mass that had been present since infancy. A painless, mobile mass was palpable in the right parotid gland, without facial paralysis. A contrast-enhanced CT showed a cystic mass with an enhanced margin in the right parotid gland. Fine-needle aspiration cytology showed no malignant findings, and partial superficial parotidectomy was performed for the purpose of diagnosis and treatment. Pathological diagnosis detected the ETV6::RET fusion gene, leading to the diagnosis of parotid secretory carcinoma. Surgical margins were negative, and no apparent recurrence was observed 1 year after the operation. It has been reported that salivary gland secretory carcinomas are mainly associated with the ETV6::NTRK3 fusion gene. Because pediatric cases with the ETV6::RETE gene are very rare and the characteristics of the disease are uncertain, careful follow-up is important.