J Korean Assoc Oral Maxillofac Surg.  2010 Oct;36(5):434-437.

Multicentric melanotic neuroectodermal tumor of infancy: a case report

• Choi BH
• Park SW
• Jang SM
• Park BC
• Son HN
• Son JH
• Sung IY
• Kim JR
• Cho YC

Abstract

A melanotic neuroectodermal tumor of infancy (MNTI) is a uncommon osteolytic pigmented neoplasm that primarily affects the jaws of newborn infants. Most patients (> 90%) present with the tumor in the first year of life. Approximately 65% form in the maxilla, 11% in the mandible, 5% in the brain and elsewhere. MNTI is normally benign, but up to 15% may recur and a few have metastasized. Approximately 200 cases of MNTI have been reported but only 2 of them presented as multifocal. A case of MNTI in a 7 month old boy was encountered. The chief complaint was maxillary anterior ridge swelling. The incisional biopsy findings were MNTI. Two months after the first operation, mild swelling of another site was observed. The infant was examined periodically since undergoing two procedures with no recurrence. This case demonstrates the possibility of a multicentric MNTI. We report a multicentric MNTI with a review of the relevant literature.

Keyword

Melanotic neuroectodermal tumor of infancy (MNTI); Multicentric

MeSH Terms

Biopsy
Brain
Humans
Infant
Infant, Newborn
Jaw
Mandible
Maxilla
Neuroectodermal Tumor, Melanotic
Polyenes
Recurrence
Polyenes

Figure

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  Fig. 1. Clinical photograph showing swelling on left anterior alveolus.

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  Fig. 2. Axial computed tomography (CT) of patient showing radiolucent lesion expanding into labial and palatal direction.

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  Fig. 3. Intraoperative photograph of the resected tumor.

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  Fig. 4. Clinical photograph showing on right anterior alveolus at 2 months after first operation.

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  Fig. 5. Clinical photograph after second operation. There was no recurrence.

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  Fig. 6. Histopathologic findings of the primary operation showing tumor cells composed of small neuroblastic cells and larger melanin containing epithelial cells.(H&E staining, original magnification ×100)

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  Fig. 7. Histopathologic findings of the secondary operation showing similar features with primary operation.(H&E staining, original magnification ×100)

Reference

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