[Melanotic neuroectodermal tumors of infancy: Current state of knowledge]. / Les tumeurs neuroectodermiques mélanotiques infantiles : état actuel des connaissances.

ABSTRACT

Melanotic Neuroectodermal Tumors of Infancy (MNTI), also known as melanotic progonoma are rare tumors affecting young children. The main locations are primarily head, neck and cranial vault. Complete surgical resection remains the standard treatment for these tumors leading to healing in the majority of cases. However, recurrent, metastatic or locally advanced forms require other treatments. The literature since 1980 reported 27 cases of patients who received treatment with chemotherapy and/or radiation therapy. Among the 24 patients who received chemotherapy, a reduction or stabilization of tumor volume was observed in 14 observations. Nine patients received radiation therapy and one patient experiences a tumor improvement. The information provided by this review can evoke the chemosensitivity of this rare tumor type but are insufficient to conclude about their radiosensitivity.