Management of Melanotic Neuroectodermal Tumor of Infancy Ma ´ rcia Gaiger De Oliveira, DDS, Lester D.R. Thompson, MD, Anna Cecı ´lia Moraes Chaves, DDS, Pantelis Varvaki Rados, DDS, Isabel da Silva Lauxen, Biol, and Manoel Sant’Ana Filho, DDS Melanotic neuroectodermal tumor of infancy is a rare congenital neoplasm involving the head and neck in young patients. The clinical assessment, histologic diagnosis, and management is reviewed, with an emphasis on different treatment alternatives in two new case reports. Ann Diagn Pathol 8: 207-212, 2004. © 2004 Elsevier Inc. All rights reserved. Index Words: Melanotic neuroectodermal tumor of infancy, clinical, pathology, prognosis, differential diagnosis M ELANOTIC neuroectodermal tumor of in- fancy (MNTI) is a rare, usually benign neo- plasm of neural crest origin composed of relatively primitive pigment-producing cells. MNTI usually arises in infants within the first 6 months of life. Because of the uncommon occurrence of this neo- plasm, past terms used to describe the tumor in- clude “pigmented epulis,” “retinal anlage tumor,” “congenital melanocarcinoma,” “melanotic epithe- lial odontoma,” “melanotic ameloblastoma,” and “melanotic progonoma,” to name just a few. It usually arises in the head and neck region and predominantly affects the maxilla, 1-3 although other sites, such as brain, epididymis, mediastinum, femur, and ovary have also been reported. 2,4-10 The tumor is usually nonulcerated and presents as a soft tissue swelling, frequently affecting bone. Although the tumor cells produce melanin, pig- mentation may not be clinically evident. 2,4,9,10 MNTI is a benign tumor, but can be locally aggres- sive, growing rapidly and resulting in tooth dis- placement as tumor cells invade bone. 1,11-13 With plain radiographs, MNTI appear as intrabony ex- pansive areas of radiolucency, usually with poorly demarcated margins, probably as a result of rapid tumor growth and a tendency to be locally invasive. Extensive tumor calcification may be identified. 5 Teeth are usually displaced and appear within the radiolucent area of the tumor. 8,9 Computed tomog- raphy (CT) scans provide important information regarding the extent of the lesion, thereby assisting in the development of a surgical plan. 9 We report the clinical and histopathologic fea- tures of two cases of MNTI treated surgically, with and without incisional biopsy. Case Reports Case 1 A small mass was noted within the maxilla of a male patient at birth, with associated feeding prob- lems developing during the ensuing 2 months. During this time the mass started growing rapidly, resulting in a referral at 2 months to the School of Dentistry, Universidade Federal do Rio Grande do Sul (Porto Alegre, Brazil). No other physical, clin- ical, or laboratory abnormalities were identified, and there were no congenital anomalies. Clinical examination revealed a fluctuant blue mass cov- ered by an intact mucosa with expansion of the left maxillary alveolar ridge (Fig 1). Radiographic ex- amination revealed a poorly defined osteolytic ra- diolucency associated with a left upper deciduous central incisor that was displaced anteriorly. CT showed a radiolucent lesion with expansion of the From the School of Dentistry, Oral Pathology, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil; and Southern California Permanente Medical Group, Woodland Hills, CA. Address reprint requests to Lester D. R. Thompson, MD, Southern California Permanente Medical Group, Department of Pathology, 5601 De Soto Ave, Woodland Hills, CA 91367. © 2004 Elsevier Inc. All rights reserved. 1092-9134/04/0804-0003$30.00/0 doi:10.1053/j.anndiagpath.2004.04.003 207 Annals of Diagnostic Pathology, Vol 8, No 4 (August), 2004: pp 207-212