PURPOSE: Supratentorial primitive neuroectodermal tumors (S-PNET) are rare and have a grim prognosis, frequently taking an aggressive course with local relapse and metastatic spread. We report the results of a mono-institutional therapeutic trial. METHODS AND MATERIALS: We enrolled 15 consecutive patients to preradiation chemotherapy (CT) consisting of high-dose methotrexate, high-dose etoposide, high-dose cyclophosphamide, and high-dose carboplatin, craniospinal irradiation (CSI) with hyperfractionated accelerated radiotherapy (HART) plus focal boost, maintenance with vincristine/lomustine or consolidation with high-dose thiotepa followed by autologous stem-cell rescue. RESULTS: Median age was 9 years; 7 were male, 8 female. Site of disease was pineal in 3, elsewhere in 12. Six patients were had no evidence of disease after surgery (NED). Of those with evidence of disease after surgery (ED), 2 had central nervous system spread. Of the 9 ED patients, 2 had complete response (CR) and 2 partial response (PR) after CT, 4 stable disease, and 1 progressive disease. Of the 7 ED patients before radiotherapy, 1 had CR, 4 PR, and 2 minor response, thus obtaining a 44% CR + PR after CT and 71% after HART. Because of rapid progression in 2 of the first 5 patients, high-dose thiotepa was systematically adopted after HART in the subsequent 10 patients. Six of 15 patients relapsed (4 locally, 1 locally with dissemination, 1 with dissemination) a mean of 6 months after starting CT, 2 developed second tumors; 5 of 6 relapsers died at a median of 13 months. Three-year progression-free survival, event-free survival, and overall survival were 54%, 34%, and 61%, respectively. CONCLUSION: Hyperfractionated accelerated RT was the main tool in obtaining responses in S-PNET; introducing the myeloablative phase improved the prognosis (3/10 vs. 3/5 relapses), though the outcome remained unsatisfactory despite the adoption of this intensive treatment.
PURPOSE: Supratentorial primitive neuroectodermal tumors (S-PNET) are rare and have a grim prognosis, frequently taking an aggressive course with local relapse and metastatic spread. We report the results of a mono-institutional therapeutic trial. METHODS AND MATERIALS: We enrolled 15 consecutive patients to preradiation chemotherapy (CT) consisting of high-dose methotrexate, high-dose etoposide, high-dose cyclophosphamide, and high-dose carboplatin, craniospinal irradiation (CSI) with hyperfractionated accelerated radiotherapy (HART) plus focal boost, maintenance with vincristine/lomustine or consolidation with high-dose thiotepa followed by autologous stem-cell rescue. RESULTS: Median age was 9 years; 7 were male, 8 female. Site of disease was pineal in 3, elsewhere in 12. Six patients were had no evidence of disease after surgery (NED). Of those with evidence of disease after surgery (ED), 2 had central nervous system spread. Of the 9 ED patients, 2 had complete response (CR) and 2 partial response (PR) after CT, 4 stable disease, and 1 progressive disease. Of the 7 ED patients before radiotherapy, 1 had CR, 4 PR, and 2 minor response, thus obtaining a 44% CR + PR after CT and 71% after HART. Because of rapid progression in 2 of the first 5 patients, high-dose thiotepa was systematically adopted after HART in the subsequent 10 patients. Six of 15 patients relapsed (4 locally, 1 locally with dissemination, 1 with dissemination) a mean of 6 months after starting CT, 2 developed second tumors; 5 of 6 relapsers died at a median of 13 months. Three-year progression-free survival, event-free survival, and overall survival were 54%, 34%, and 61%, respectively. CONCLUSION: Hyperfractionated accelerated RT was the main tool in obtaining responses in S-PNET; introducing the myeloablative phase improved the prognosis (3/10 vs. 3/5 relapses), though the outcome remained unsatisfactory despite the adoption of this intensive treatment.
Authors: Carsten Friedrich; Klaus Müller; Katja von Hoff; Robert Kwiecien; Torsten Pietsch; Monika Warmuth-Metz; Nicolas U Gerber; Peter Hau; Joachim Kuehl; Rolf D Kortmann; André O von Bueren; Stefan Rutkowski Journal: J Neurooncol Date: 2014-01-10 Impact factor: 4.130
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Authors: M Kashif Ishaqi; A Jamil; M Khanani; M Baroudi; Omar Trad; M El-Hayek; Eric Bouffet Journal: J Neurooncol Date: 2009-08-23 Impact factor: 4.130
Authors: Murali Chintagumpala; Tim Hassall; Shawna Palmer; David Ashley; Dana Wallace; Kimberly Kasow; Thomas E Merchant; Matthew J Krasin; Robert Dauser; Frederick Boop; Robert Krance; Shiao Woo; Robyn Cheuk; Ching Lau; Richard Gilbertson; Amar Gajjar Journal: Neuro Oncol Date: 2008-09-16 Impact factor: 12.300
Authors: Teresa de Rojas; Francisco Bautista; Miguel Flores; Lucía Igual; Raquel Rubio; Eduardo Bardón; Lucía Navarro; Laura Murillo; Raquel Hladun; Adela Cañete; Miguel Garcia-Ariza; Carmen Garrido; Ana Fernández-Teijeiro; Eduardo Quiroga; Carlota Calvo; Anna Llort; Inmaculada de Prada; Luis Madero; Ofelia Cruz; Lucas Moreno Journal: J Neurooncol Date: 2017-12-16 Impact factor: 4.130
Authors: Bryan K Li; Alexandre Vasiljevic; Christelle Dufour; Fupan Yao; Ben L B Ho; Mei Lu; Eugene I Hwang; Sridharan Gururangan; Jordan R Hansford; Maryam Fouladi; Sumihito Nobusawa; Annie Laquerriere; Marie-Bernadette Delisle; Jason Fangusaro; Fabien Forest; Helen Toledano; Palma Solano-Paez; Sarah Leary; Diane Birks; Lindsey M Hoffman; Alexandru Szathmari; Cécile Faure-Conter; Xing Fan; Daniel Catchpoole; Li Zhou; Kris Ann P Schultz; Koichi Ichimura; Guillaume Gauchotte; Nada Jabado; Chris Jones; Delphine Loussouarn; Karima Mokhtari; Audrey Rousseau; David S Ziegler; Shinya Tanaka; Scott L Pomeroy; Amar Gajjar; Vijay Ramaswamy; Cynthia Hawkins; Richard G Grundy; D Ashley Hill; Eric Bouffet; Annie Huang; Anne Jouvet Journal: Acta Neuropathol Date: 2019-12-09 Impact factor: 17.088