Role of radical surgery for intracranial melanotic neuroectodermal tumor of infancy: Case report

OBJECTIVE AND IMPORTANCE: Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, locally aggressive tumor that arises most commonly from the max illa or mandible. Infrequently, irt originates from the cranial vault, and recent reports have described a favorable outcome after radical surgery. So me lesions are particularly problematic, such as those located along the cr anial midline or cranial base and those with significant intracranial exten sion. Currently, there is no effective adjuvant therapy for MNTI; radiation is precluded by the patients' young age, and chemotherapy trials have not demonstrated long-term efficacy. CLINICAL PRESENTATION: A 2-month-old infant boy presented with a firm, immo bile subcutaneous mass behind the right ear. The mass had been present at b irth and enlarged with time. INTERVENTION: Initial resective surgery down to the dura resulted in massiv e tumor recurrence within weeks. Successful management required repeat surg ery including excision of the dura and dural venous sinuses. CONCLUSION: This patient's large MNTI of the cranial base was successfully managed by radical surgery. Although MNTI is a vapidly growing tumor that i s locally highly invasive, radical surgery may be associated with a favorab le outcome and offers the potential for long-term cure.