title: Melanotic neuroectodermal tumor of infancy: a wolf in sheep's clothing.
abstract: BACKGROUND
  The melanotic neuroectodermal tumor of infancy (MNTI) is an exceptionally rare neoplasm. Despite their potential for local invasion, MNTI are considered benign neoplasms. Sporadic cases have been reported of MNTI acting in a malignant fashion; however, the majority of these tumors were of extragnathic origin.
  
  
  METHODS
  A 2-month-old male presented with a rapidly expanding maxillary mass. The patient underwent excisional biopsy of the mass and a diagnosis of MNTI was returned. Tumor recurrence was appreciated postoperatively. Neoadjuvant chemotherapy was initiated to render it more amenable to surgical resection. Chemotherapy was suspended prematurely and the patient returned to the operating room for hemimaxillectomy. The postchemotherapy surgical pathology report demonstrated a unique mixed chemotherapy response.
  
  
  CONCLUSIONS
  This report highlights the aggressive nature of gnathic MNTI, the importance of early diagnosis and definitive intervention, and the mixed clinical and histologic response of the lesion to neoadjuvant chemotherapy.
authors.affiliation: Division of Oral and Maxillofacial Surgery, Hofstra North-Shore Long Island Jewish Health System, New Hyde Park, New York, USA jdavis2009@dents.uwo.ca.
authors.forename: Joel M
authors.initials: JM
authors.lastname: Davis
authors.affiliation: Division of Hematology and Oncology, Hofstra North-Shore Long Island Jewish Health System, New Hyde Park, New York, USA.
authors.forename: Marianna
authors.initials: M
authors.lastname: DeBenedictis
authors.affiliation: Division of Head and Neck Surgery, Hofstra North-Shore Long Island Jewish Health System, New Hyde Park, New York, USA.
authors.forename: Douglas K
authors.initials: DK
authors.lastname: Frank
authors.affiliation: Division of Oral and Maxillofacial Surgery, Hofstra North-Shore Long Island Jewish Health System, New Hyde Park, New York, USA.
authors.forename: Michael E
authors.initials: ME
authors.lastname: Lessin
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D001706:Biopsy / Q000379:methods; D004210:Dissection; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008437:Maxilla / Q000473:pathology / Q000601:surgery; D008441:Maxillary Neoplasms / Q000473:pathology / Q000628:therapy; D063166:Maxillary Osteotomy / Q000379:methods*; D008447:Maxillofacial Prosthesis; D019931:Maxillofacial Prosthesis Implantation / Q000379:methods*; D020360:Neoadjuvant Therapy / Q000379:methods; D017600:Neuroectodermal Tumor, Melanotic / Q000473:pathology / Q000628:therapy; D016896:Treatment Outcome
keywords: infancy; maxilla; tumor