Melanotic neuroectodermal tumor of infancy in the skull: case report and review of the literature.

BACKGROUND

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm that develops during the first year of life and grows rapidly. Early diagnosis and radical surgery are critical for a long-term cure. We report a rare case of MNTI in the skull and discuss the importance of the radical surgery and the long-term follow-up results.

CASE PRESENTATION

We describe a case of a 4-month-old girl with an MNTI in the skull who underwent the operation 11 years ago. The mass in the frontotemporosphenoid region grew rapidly after birth. The patient underwent a craniotomy. By referring to the histological findings of frozen section during surgery, a total excision of the tumor including its adjacent hypertrophic bone was performed. The patient has remained well without evidence of recurrence or neurological abnormality for 11 years.

CONCLUSION

Radical surgery for MNTI provides complete cure. According to the literature including our case, there should be follow-up for at least 2 years after surgery. Especially in cases in which tumors recur, follow-up should be for longer periods because of the possibility of its malignant change.