Review ArticleManagement of Unresectable Metastatic Primitive Myxoid Mesenchymal Tumor of Infancy A Case Report and Systematic Review of the LiteratureAsaftei, Sebastian D. MD; Campello, Anna MD; Tirtei, Elisa MD; Colombo, Sara MD; Vallero, Stefano MD; Boffano, Michele MD; Fagioli, Franca MDAuthor Information Pediatric Oncology Department, OIRM Hospital, AOU Città della Salute e della Scienza Torino, Torino, Italy The authors declare no conflict of interest. Reprints: Sebastian D. Asaftei, MD, Pediatric Oncology Department, OIRM Hospital, AOU Città della Salute e della Scienza Torino, Piazza Polonia 94, Torino 10126, Italy (e-mail: email@example.com). Received August 23, 2019 Accepted January 27, 2020 Online date: February 28, 2020 Journal of Pediatric Hematology/Oncology: April 2020 - Volume 42 - Issue 3 - p 163-169 doi: 10.1097/MPH.0000000000001764 Buy Metrics Abstract Primitive myxoid mesenchymal tumor of infancy is a rare soft tissue tumor. The present case is one of the most invasive primitive myxoid mesenchymal tumor of infancy reported to date. To our knowledge, it is the first case described with extensive involvement of pelvis and the third described developing metastasis and with an invasion of the spinal canal without evidence of transformation into undifferentiated sarcoma. The patient failed to respond to chemotherapy (CHT). According to the few available data, CHT seems to be more effective in the presence of metastatic disease or increased cellularity. However, CHT, including high-dose ifosfamide, resulted ineffective even after lung metastasis development with pathologic evidence of increased mitotic rate. The management of this case and the data in the literature confirm surgery as the gold standard treatment in this pathology. Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.