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.
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.
Authors: Florian Ebel; Florian M Thieringer; Christoph Kunz; Andreas Klein-Franke; Katrin Scheinemann; Raphael Guzman; Jehuda Soleman Journal: Childs Nerv Syst Date: 2020-01-17 Impact factor: 1.475
Authors: Thiago Pastor da Silva Pinheiro; José Thiers Carneiro; Sérgio de Melo Alves; João de Jesus Viana Pinheiro; Fabrício Mesquita Tuji Journal: Head Face Med Date: 2013-11-25 Impact factor: 2.151