An inborn mass in the premaxilla of an infant

Background: Melanotic neuroectodermal tumour of infancy (MNTI) is a rare congenital neoplasm of early infancy characterised by rapid growth and a high rate of local recurrences. It rarely metastasises but is locally aggressive. Since its description in 1918 the tumour was reported under a variety of names, reflecting uncertainty about its histogenesis. Recent evidence shows that the tumour is probably of the neural crest origin. The clinical differential diagnosis of an inborn mass in the pre maxilla of an infant includes MNTI, congenital granular cell tumour, congenital, epulis, teratoma, neuroblastoma, Ewing's sarcoma, melanoma, and metastatic retinoblastoma. We present a one-day-old male with a huge maxillary soft tissue mass. Case Report: We report a one-day-old male with a huge maxillary soft tissue mass obliterating the entire oral cavity making breathing and feeding extremely difficult. Result: Fine needle aspiration from the mass showed a lesion composed of small neuroblast-like cells and large melanin-containing epithelial cells. The appearances were consistent with MNTI. Histologically, the excised mass is composed of dense fibro vascular tissue containing neuroblast-like cells in a fibrous stroma and alveolar spaces lined by melanin-containing cells. The mass was conservatively excised. The patient remained well thirteen years postoperatively. Conclusion: In our case the lesion had been overlooked during prenatal sonographic examination and that led to an emergency intervention within departmental lack of critical care facilities. So, In utero diagnosis of a sizeable congenital tumour minimises possible postnatal surgical management risk and complications.