Abstract

Primitive neuroectodermal tumors (PNET) are rare malignancies of presumedneural crest origin, most often presenting as bone or soft tissue masses in the trunk or axial skeleton,in children and young adults. Treatment of advanced PNET in adults is not clearly defined in the literature.Data concerning dose-intensive chemotherapy regimens for poor-risk patients with those tumors are sparse,due to rarity of PNET in adults, their diverse presentation, the variable treatment procedures appliedand the absence of direct comparisons. On the other hand, the role of anthracyclines in the treatmentof advanced soft tissue sarcomas is well known and substantial. Six advanced PNET patients were treatedat the Institute for Oncology and Radiology of Serbia, during last five years, with high-doses of doxorubicinor epidoxorubicin combined with cisplatin. The paper reviews each of our patients, discussing how doeschemotherapy influence the outcome in these patients, in context of the feasibility of high-doses ofanthracyclines in advanced settings. High dose anthracyclines (epidoxorubicin 150 mg/m2) in combinationwith cisplatin 120 mg/m2 induced a complete response lasting for 63+ months in a patient with desmoplasticmedulloblastoma of the cerebellum metastatic to bones and bone marrow. The same treatment but with theepidoxorubicin dose of 180 mg/m2 induced a complete response in a patient with olfactory neuroblastoma.Administration of high dose Doxorubicin (75 mg/m2) seems feasible in association with irradiation treatmentin patients with extraosseal Ewing sarcoma/PNET but the place of high dose chemotherapy within this settingremains to be determined.

Keywords: Antibiotics, Antineoplastic, Cerebellar Neoplasms, Disease-Free Survival, Doxorubicin, Epirubicin, Medulloblastoma, Neuroblastoma, Neuroectodermal Tumors, Primitive