A case report of atypical malignant melanotic neuroectodermal tumor of infancy

Abstract

Melanotic neuroectodermal tumor of infancy is a rare neoplasm that predominantly involves cranial bones and tends to occur during the first year of life. About 500 cases have been described in the literature to date; 6% of them have been reported to be malignant. Treatment for these malignant tumors was not documented and often turned out to be ineffective.Here we report a case of a child aged 2 years and 4 months who presented with a rapidly growing mass in the maxillary region spreading through the orbit into the anterior cranial fossa. The patient’s parents gave consent to the use of their child’s data, including photographs, for research purposes and in publications. He was treated at the Russian Children’s Clinical Hospital from July 2018 to November 2019. The child underwent chemoradiation and staged surgical removal of the tumor. Treatment with ICE and radiation therapy led to a significant reduction of the tumor volume and enabled us to perform cytoreductive surgery with the removal of the mass in the maxilla. Further treatment according to the CWS 2009 guidance for high-risk patients with NRSTS (NonRhabdomyosarcoma Soft Tissue Sarcoma) and radiation therapy resulted in further regression of intraorbital and intracranial components of the tumor and we performed a radical resection of the residual tumor conglomerate. Investigations during the course of treatment revealed no signs of metastatic involvement. The behavior of malignant melanotic neuroectodermal tumors of infancy is unpredictable, that is why in case of the massive involvement of the facial bones when surgery is associated with a high risk of functional impairment or cosmetic deformity, one should consider preoperative chemotherapy to reduce tumor size and intraoperative blood loss. Moreover, chemotherapy in combination with resection makes it possible to minimize the risk of local relapse or metastasis.