A clinicopathologic and immunohistochemical analysis of melanotic neuroectodermal tumor of infancy

Resident’s Name: Brian Schmid DMD
Author(s): A.W. Barrett et al.
Journal: Oral Surg Oral Med Oral Path
Month, Year: 2002
Major topic: A small retrospective, histological study of melanotic neuroectodermal tumors in infants (MNTI)
Type of Article: Retrospective
Findings: MNTI’s are quite rare and usually considered benign with only 250 reported cases, typically as a single or paired lesion but also in clusters of 5 or more. The membrane can be intact or ulcerated, blue, brown or red and can even resemble an eruption cyst. There is no sex predilection and presentation is typically less than 1y.o. The treatement of choice is surgical removal with a conservative lymph node dissection to rule out metastasis. Radiotherapy and chemotherapy are ineffective and would be contraindicated in such young patients anyhow.
Searching through pathology department archives, 8 cases of melanotic neuroectodermal tumors were found; 7 in males and 7 affected the maxilla. The cases ranged from 1963-1999. The age of presentation was 2.5-14 months and all were treated with excision. Three of the cases “involved the whole maxilla” and 2 crossed the midline but only 1 case required a radical bilateral maxillectomy. Radiographically the lesions could present a radiopaque or radiolucent state and moth single and multilocularity. Histologically the lesions present as small melanocyte-like cells mixed with smaller neuroblast-like cells. There is good evidence that these lesions are of neural crest origin. The purpose of this study as to elucidate a histological/immunological assay that could be performed to successfully predict aggression and malignancy of a given tumor. NB84 is a reliable marker for childhood neuroblastoma but was negative on all account in this study. While the most aggressive case was the only one positive for CD99 and Ki67, this is inconsistent with many previous findings. There is a small chance of recurrence which can be monitored with regular intra-oral exams.
Key points/Summary: While this study did not specify any immunohistological markers which could help identify the more malignant episodes of this tumor, further study is necessary and in the meantime it is in the best interest of the patient to perform proper soft tissue exams at regular intervals in all patients. Erythroplakic and pigmented lesions are of particular importance to note, monitor and treat.
Assessment of article: Chock full of biochemistry goodness but with some valuable clinical peppering. They included no photos of the study patients, which may not have been available, or even stock photos of an MNTI.