Abstract
BackgroundTo identify prognostic factors and define the optimal management of patients with supratentorial primitive neuroectodermal tumors (sPNETs), we investigated treatment outcomes and explored the prognostic value of specific molecular markers.MethodsA total of 47 consecutive patients with pathologically confirmed sPNETs between May 1985 and June 2012 were included. Immunohistochemical analysis of LIN28, OLIG2, and Rad51 expression was performed and correlated with clinical outcome.ResultsWith a median follow-up of 70 months, 5-year overall survival (OS) and progression-free survival (PFS) was 55.5% and 40%, respectively, for all patients. Age, surgical extent, and radiotherapy were significant prognostic factors for OS and PFS. Patients who received initially planned multimodal treatment without interruption (i.e., radiotherapy and surgery (≥subtotal resection), with or without chemotherapy) showed significantly higher 5-year OS (71.2%) and PFS (63.1%). In 29 patients with available tumor specimens, tumors with high expression of either LIN28 or OLIG2 or elevated level of Rad51 were significantly associated with poorer prognosis.ConclusionsWe found that multimodal treatment improved outcomes for sPNET patients, especially when radiotherapy and ≥subtotal resection were part of the treatment regimen. Furthermore, we confirmed the prognostic significance of LIN28 and OLIG2 and revealed the potential role of Rad51 in sPNETs.
Highlights
Primitive neuroectodermal tumors (PNETs) are the most frequent type of malignant pediatric brain tumors, which primarily consist of undifferentiated round neuroepithelial cells
In 29 patients with available tumor specimens, tumors with high expression of either LIN28 or OLIG2 or elevated level of Rad51 were significantly associated with poorer prognosis
We found that multimodal treatment improved outcomes for sPNET patients, especially when radiotherapy and subtotal resection were part of the treatment regimen
Summary
Primitive neuroectodermal tumors (PNETs) are the most frequent type of malignant pediatric brain tumors, which primarily consist of undifferentiated round neuroepithelial cells. Certain factors, including young age, tumor dissemination, no radiotherapy, and no grossly total resection, are related to poorer prognosis, emphasizing the importance of multimodal treatment or tailored therapeutic strategies for each patient [3,4,5]. Expression of Rad, which plays a central role in the repair of DNA double-strand breaks, has been recently examined in many types of tumors [13] but not sPNETs. It is generally suggested that Rad overexpression increases cellular resistance to radiation and some chemotherapeutic drugs [14]. We suggested that this molecular marker could be used to predict prognosis but may serve as potential therapeutic targets for sPNETs. To identify prognostic factors and define the optimal management of patients with supratentorial primitive neuroectodermal tumors (sPNETs), we investigated treatment outcomes and explored the prognostic value of specific molecular markers
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