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.