Title
Melanotic Neuroectodermal Tumor of Infancy: A Wolf in Sheep's Clothing
Publication Date
2015
Journal Title
Ann Otol Rhinol Laryngol
Abstract
INTRODUCTION: 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. DISCUSSION: 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.
Volume Number
124
Issue Number
2
Pages
97-101
Document Type
Article
EPub Date
2014/07/18
Status
Faculty, Northwell Researcher
Facility
School of Medicine; Northwell Health
Primary Department
Otolaryngology
Additional Departments
Dental Medicine; Molecular Medicine
PMID
DOI
10.1177/0003489414543070