A pilot study of risk-adapted radiotherapy and chemotherapy in patients with supratentorial PNET

Abstract

We undertook this study to estimate the event-free survival (EFS) of patients with newly diagnosed supratentorial primitive neuroectodermal tumor (SPNET) treated with risk-adapted craniospinal irradiation (CSI) with additional radiation to the primary tumor site and subsequent high-dose chemotherapy supported by stem cell rescue. Between 1996 and 2003, 16 patients with SPNET were enrolled. High-risk (HR) disease was differentiated from average-risk (AR) disease by the presence of residual tumor (M(0) and tumor size > 1.5 cm(2)) or disseminated disease in the neuraxis (M(1)-M(3)). Patients received risk-adapted CSI: those with AR disease received 23.4 Gy; those with HR disease, 36-39.6 Gy. The tumor bed received a total of 55.8 Gy. Subsequently, all patients received four cycles of high-dose cyclophosphamide, cisplatin, and vincristine with stem cell support. The median age at diagnosis was 7.9 years; eight patients were female. Seven patients had pineal PNET. Twelve patients are alive at a median follow-up of 5.4 years. The 5-year EFS and overall survival (OS) estimates for all patients were 68% +/- 14% and 73% +/- 13%. The 5-year EFS and OS estimates were 75% +/- 17% and 88% +/- 13%, respectively, for the eight patients with AR disease and 60% +/- 19% and 58% +/- 19%, respectively, for the eight with HR disease. No deaths were due to toxicity. High-dose cyclophosphamide-based chemotherapy with stem cell support after risk-adapted CSI results in excellent EFS estimates for patients with newly diagnosed AR SPNET. Further, this chemotherapy allows for a reduction in the dose of CSI used to treat AR SPNET without compromising EFS.

Pediatric Brain Tumors: Current Treatment Strategies and Future Therapeutic Approaches

“…61 Again, given the two different treatment regimens in these two protocols it remains difficult to answer which part of the therapy was most beneficial. Chintagumpala et al 62 investigated the use of reduced craniospinal irradiation (23.4 Gy) and high-dose chemotherapy with ASCR in children with nonmetastatic sPNET. In this study, patients with high-risk disease (defined as having either residual tumor Ͼ1.5 cm 2 or evidence of metastatic disease) received 36.0 to 39.6 Gy, and those with average risk disease only 23.4 Gy to the craniospinal axis.…”

“…These results are comparable with other studies and suggest that reduced craniospinal irradiation in combination with chemotherapy in averagerisk patients might not compromise outcome. 62 The information available today suggests that sPNETs are chemo-sensitive and that high-dose chemotherapy can be successfully used in newly diagnosed sPNET. Especially younger children (Ͻ3 years of age) might benefit from high-dose chemotherapy with ASCR as initial therapy to delay radiation therapy.…”

Molecular methods in diagnosis of poorly differentiated malignant brain tumors in children

“…Но при всем сходстве гистологии по биологиче-ским свойствам и подходам к лечению вышеописан-ные новообразования представляют собой абсолютно разные группы опухолей, требующие собственных протоколов лечения [5,6,8,12,14,19,20,24,50].…”

Small Supratentorial, Extraaxial Primitive Neuroectodermal Tumor Causing Large Intracerebral Hematoma -Case Report-

“…12) Treatment is often challenging and frustrating due to the poor prognosis, and depends on many risk factors such as localization of the lesion and grade of staging at diagnosis. 3,5) Compared to other embryonic malignant tumors such as medulloblastoma with better established treatment regimes, the optimum treatment of this rare and heterogeneous tumor group is still the subject of research and many clinical studies are ongoing. 10,14,19,22) Today, surgery is recommended for the care of PNET patients (À3 years of age) followed by radiation 14) and chemotherapy.…”