Congenital sialoblastoma in a newborn: diagnostic challenge of a rare entity

Background The occurrence of a head and neck tumoral mass at birth or shortly afterwards may cause concern and the differential diagnosis may be complex. Sialoblastoma is a rare epithelial tumor of the salivary glands of uncertain malignant potential, which typically affects children and arises more frequently in the parotid gland. The diagnosis may be challenging, as the differential diagnosis is wide. Methods The present paper reports a case of sialoblastoma, present at birth, diagnosed in a 20-day-old girl. The patient was submitted to surgery and the neoplasm was excised "en bloc". We also carried out a thorough review of all cases of sialoblastoma, diagnosed in the first year of life and reported in the literature, using the PubMed database. Results A final diagnosis of sialoblastoma was rendered through histological examination and immunohistochemistry. The neoplasm resulted entirely excised with disease-free surgical margins, and no other therapy was necessary. The 2 years clinical and instrumental follow-up was uneventful. Conclusions Through the present case and the review of the literature, the state of the art is established with regard the clinical aspects, the morphological and immunophenotypic features for diagnostic purpose. Moreover, parameters that can influence the prognosis are evaluated, paying attention especially to the cases of sialoblastoma diagnosed in the first year of life.