Melanotic Neuroectodermal Tumour of Infancy: A Benign but Locally Destructive Growth

Abstract

Melanotic Neuroectodermal Tumour of Infancy (MNTI) is a rare neoplasm typically reported in infancy, involving the head and neck regions. Although described as a benign tumour, the growth is rapid and has a high local recurrence rate. Due to the rapid and locally destructive growth, it can easily be misdiagnosed as a malignant tumour, leading to unwarranted and potentially harmful interventions. Therefore, histopathological assessment is pertinent in confirming the diagnosis. Early surgical resection is critical to limiting cosmetic disfigurement but is challenging as it requires prompt and careful planning to ensure optimal clearance. Therefore, it is crucial to approach anterior maxillary swellings in an infant with an index of suspicion and to consider MNTI as a rare but important differential diagnosis to ensure a timely diagnosis and plan for appropriate early intervention. We report a case of an infant presenting with a rapidly growing and locally destructive intra-oral swelling who underwent tumour excision and was later confirmed to be MNTI. We discuss the clinical, radiological, and histological features and the challenges in diagnosing and managing this rare childhood tumour.