BACKGROUND: Intracranial peripheral primitive neuroectodermal tumors (P-PNET) are extremely rare. They can be easily misdiagnosed as central nervous system primitive neuroectodermal tumors (CNS-PNET) or meningiomas. Little is known about the optimal treatment and prognosis of these tumors. PATIENTS AND METHODS: We evaluated the treatment and outcome of 17 patients with intracranial, nonmetastatic, genetically confirmed P-PNET. Three patients were treated at our institutions. Thirteen other cases providing sufficient treatment and follow-up information were extracted from the literature. RESULTS: The median age at diagnosis was 17 years. All patients underwent initial surgery. Complete resection was achieved in 9 of the 17 cases (53 %). Combined adjuvant treatment consisting of radiotherapy (focal, n = 10; craniospinal, n = 1) and chemotherapy was administered to 11 of the 17 patients (59 %). The median follow-up time was 1.4 years. In 8 of the 17 patients (47 %), the disease progressed; 4 of the 17 patients (24 %) died. The 2-year progression-free and overall survival rates were 64 % and 76 %, respectively. CONCLUSION: The differential diagnosis for intracranial, meningeal-based, small, round-cell tumors should include P-PNET. It is highly probable that complete resection has a positive impact on survival--as previously reported for extracranial P-PNET--but this cannot be shown by our data. Intensive adjuvant treatment consisting of radiotherapy and chemotherapy seems to be essential. A statistically grounded recommendation for the appropriate target volume and radiation dose is not yet possible. However, in most case reports of primary intracranial P-PNET published to date, patients were treated with focal irradiation. The optimal chemotherapy regimen has yet to be established, with both the Ewing tumor and CNS-PNET protocols being promising candidates for effective treatment.
BACKGROUND: Intracranial peripheral primitive neuroectodermal tumors (P-PNET) are extremely rare. They can be easily misdiagnosed as central nervous system primitive neuroectodermal tumors (CNS-PNET) or meningiomas. Little is known about the optimal treatment and prognosis of these tumors. PATIENTS AND METHODS: We evaluated the treatment and outcome of 17 patients with intracranial, nonmetastatic, genetically confirmed P-PNET. Three patients were treated at our institutions. Thirteen other cases providing sufficient treatment and follow-up information were extracted from the literature. RESULTS: The median age at diagnosis was 17 years. All patients underwent initial surgery. Complete resection was achieved in 9 of the 17 cases (53 %). Combined adjuvant treatment consisting of radiotherapy (focal, n = 10; craniospinal, n = 1) and chemotherapy was administered to 11 of the 17 patients (59 %). The median follow-up time was 1.4 years. In 8 of the 17 patients (47 %), the disease progressed; 4 of the 17 patients (24 %) died. The 2-year progression-free and overall survival rates were 64 % and 76 %, respectively. CONCLUSION: The differential diagnosis for intracranial, meningeal-based, small, round-cell tumors should include P-PNET. It is highly probable that complete resection has a positive impact on survival--as previously reported for extracranial P-PNET--but this cannot be shown by our data. Intensive adjuvant treatment consisting of radiotherapy and chemotherapy seems to be essential. A statistically grounded recommendation for the appropriate target volume and radiation dose is not yet possible. However, in most case reports of primary intracranial P-PNET published to date, patients were treated with focal irradiation. The optimal chemotherapy regimen has yet to be established, with both the Ewing tumor and CNS-PNET protocols being promising candidates for effective treatment.
Authors: Holcombe E Grier; Mark D Krailo; Nancy J Tarbell; Michael P Link; Christopher J H Fryer; Douglas J Pritchard; Mark C Gebhardt; Paul S Dickman; Elizabeth J Perlman; Paul A Meyers; Sarah S Donaldson; Sheila Moore; Aaron R Rausen; Teresa J Vietti; James S Miser Journal: N Engl J Med Date: 2003-02-20 Impact factor: 91.245
Authors: Ramon Navarro; Astrid Laguna; Carmen de Torres; Juan Cruz Cigudosa; Mariona Suñol; Ofelia Cruz; Jaume Mora Journal: J Neurosurg Date: 2007-11 Impact factor: 5.115
Authors: Arnold C Paulino; Daniel T Cha; Jerry L Barker; Simon Lo; Ricarchito B Manera Journal: Int J Radiat Oncol Biol Phys Date: 2004-03-15 Impact factor: 7.038
Authors: Vivek Subbiah; Pete Anderson; Alexander J Lazar; Emily Burdett; Kevin Raymond; Joseph A Ludwig Journal: Curr Treat Options Oncol Date: 2009-06-17
Authors: Katherine A VandenHeuvel; Rami N Al-Rohil; Michael E Stevenson; Jiang Qian; Naina L Gross; Rene McNall-Knapp; Shibo Li; Eric P Wartchow; Gary W Mierau; Kar-Ming Fung Journal: Int J Clin Exp Pathol Date: 2015-01-01
Authors: Sophie Pietschmann; André O von Bueren; Guido Henke; Michael Josef Kerber; Rolf-Dieter Kortmann; Klaus Müller Journal: J Neurooncol Date: 2014-08-27 Impact factor: 4.130