Melanotic neuroectodermal tumor of infancy associated with pulmonary neoplasia. A case report

Abstract

Introduction: Melanotic Neuroectodermal Tumor of Infancy (MNTI) is a rare neoplasm of neural crest origin, with just over 500 reported cases. It is benign and rapidly growing, typically occurring in children under one year of age. It primarily affects the head and neck, with additional reported sites including the epididymis, testicles, ovaries, soft tissue, and bones of the extremities. Metastases have been reported mainly in lymph nodes and the central nervous system (CNS), with no documented cases of respiratory metastasis or association with primary lung tumours. We present the case of a pediatric patient who underwent resection for recurrent MNTI at two years of age and subsequently developed a primary myoepithelial lung carcinoma (PMLC) at six years old. Objective: To analyze a clinical case of PMLC in a pediatric patient with a history of MNTI, emphasizing its diagnosis, progression, and therapeutic response. Materials and Methods: Case information was collected from the Dr. Jorge Lizarraga Children's Hospital medical system, and the scientific literature was reviewed. Results: Imaging studies and biopsy confirmed the diagnosis of PMLC. The patient underwent chemotherapy, with a favourable clinical outcome, representing the first documented pediatric case with a positive response to treatment in this hospital. Conclusion: This case highlights the importance of long-term follow-up in patients with MNTI, given the potential development of primary pulmonary neoplasms. It also highlights the need for further studies to better understand the natural history and optimal management of PMLC in children.