433 - Chronicling a Case of Melanotic Nueroectodermal Tumor of Infancy in a Pediatric Patient

Introduction:  Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, benign neoplasm that most commonly affects the anterior maxilla of young infants.  Its clinical presentation is typically a soft, pigmented swelling capable of rapid expansion that destroys underlying bone and may displace developing teeth. Radiographically, it presents as a regional radiolucency that may or may not have well-defined margins.  Its histopathologic features are key in definitively diagnosing the lesion: fibrovascular stroma with nests and clusters of tumor cells arranged in an alveolar pattern. The central part of the alveoli contain small and round cells with large nuclei in comparison to the amount of cytoplasm while the peripheral portions contain flattened cuboidal epithelial cells. This lesion is most common in infants below the age of one.  
Case Abstract: A 3-year, 8-month-old female presented to Riley Children’s Hospital dental clinic as a new patient for a comprehensive examination.  Her medical history includes well-controlled asthma and a surgical removal of an MNTI in 2018 when she was 4 months old.  She takes over-the-counter analgesics such as acetaminophen and ibuprofen as needed for pain and has an albuterol rescue inhaler which she requires infrequently. She has an allergy to the antibiotic Omnicef.  The patient presents with primary dentition, but is missing #E, F, G, H, I, and J, which were consequently removed along with her tumor. The patient’s mother states she has been having severe, chronic pain for many weeks that almost always causes the patient to develop a rash along the V2 region of their left trigeminal nerve.  A plan was proposed to fabricate a removable partial dental prosthesis to restore the patient’s daily oral functions and prescribe Neurontin to manage their neuropathic pain.  This report will include clinical photographs of the patient prior to- and following surgical removal of the tumor, as well as photos of their current state with and without a fabricated prosthesis in place. Additionally, radiographic imaging and histologic findings used in the diagnosis of the patient’s MNTI will be presented along with the pharmacological management of their neuropathic pain.