Secretory Carcinoma in a Labial Minor Salivary Gland of a Pediatric Patient

Secretory carcinoma (SC) is a low-grade malignant salivary gland tumor that most commonly harbors an ETV6::NTRK3 fusion. Few pediatric cases have been reported, particularly in minor salivary glands.

We describe a 9-year-old girl who presented with a painless, 2 cm, sessile nodule on the upper lip one year after local trauma. Excisional biopsy revealed a well-circumscribed neoplasm composed of microcystic nests filled with eosinophilic secretions that were periodic-acid-Schiff-diastase-positive. Tumor cells showed diffuse positivity for CK7, mammaglobin, and S100, supporting the diagnosis of SC. Magnetic resonance imaging demonstrated heterogeneous enhancement confined to the surgical bed with no nodal involvement. A margin-extension procedure found no residual tumor. The patient remains disease-free 36 months post-treatment.

This case highlights that SC, although rare in children and in the upper lip, should enter the differential diagnosis of circumscribed labial nodules. Early recognition permits complete excision with minimal morbidity and excellent prognosis. Awareness of this entity among clinicians and pathologists is essential to prevent delays in management.