title: Phase II TPDCV protocol for pediatric low-grade hypothalamic/chiasmatic gliomas: 15-year update.
abstract: To report long-term results for children with low-grade hypothalamic/chiasmatic gliomas treated on a phase II chemotherapy protocol. Between 1984 and 1992, 33 children with hypothalamic/chiasmatic LGGs received TPDCV chemotherapy on a phase II prospective trial. Median age was 3.0 years (range 0.3-16.2). Twelve patients (36%) underwent STRs, 14 (42%) biopsy only, and seven (21%) no surgery. Twenty patients (61%) had pathologic JPAs, nine (27%) grade II gliomas, and four (12%) no surgical sampling. Median f/u for surviving patients was 15.2 years (range 5.3-20.7); 20 of the 23 surviving patients had 14 or more years of follow-up. Fifteen-year PFS and OS were 23.4 and 71.2%, respectively. Twenty-five patients progressed, of whom 13 are NED, two are AWD, and 10 have died. All children who died were diagnosed and first treated at age three or younger. Age at diagnosis was significantly associated with relapse and survival (P = 0.004 for PFS and P = 0.037 for OS). No PFS or OS benefit was seen with STR versus biopsy/no sampling (P = 0.58 for PFS, P = 0.59 for OS). For patients with JPAs and WHO grade II tumors, the 15-year PFS was 18.8 and 22.2% (P = 0.95) and 15-year OS was 73.7 and 55.6% (P = 0.17), respectively. Upfront TPDCV for children with hypothalamic/chiasmatic LGGs resulted in 15-year OS of 71.2% and 15-year PFS of 23.4%. No survival benefit is demonstrated for greater extent of resection. Age is a significant prognostic factor for progression and survival.
authors.affiliation: Department of Radiation Oncology, University of California, San Francisco, 1600 Divisadero, San Francisco, CA 94143, USA.
authors.forename: Kavita K
authors.initials: KK
authors.lastname: Mishra
authors.forename: Sarah
authors.initials: S
authors.lastname: Squire
authors.forename: Kathleen
authors.initials: K
authors.lastname: Lamborn
authors.forename: Anuradha
authors.initials: A
authors.lastname: Banerjee
authors.forename: Nalin
authors.initials: N
authors.lastname: Gupta
authors.forename: William M
authors.initials: WM
authors.lastname: Wara
authors.forename: Michael D
authors.initials: MD
authors.lastname: Prados
authors.forename: Mitchel S
authors.initials: MS
authors.lastname: Berger
authors.forename: Daphne A
authors.initials: DA
authors.lastname: Haas-Kogan
mesh_terms: D000293:Adolescent; D000367:Age Factors; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D018572:Disease-Free Survival; D005260:Female; D005910:Glioma / Q000188:drug therapy* / Q000401:mortality; D006801:Humans; D007029:Hypothalamic Neoplasms / Q000188:drug therapy* / Q000401:mortality; D007223:Infant; D008130:Lomustine / Q000627:therapeutic use; D008137:Longitudinal Studies; D008297:Male; D008936:Mitolactol / Q000627:therapeutic use; D011237:Predictive Value of Tests; D011344:Procarbazine / Q000627:therapeutic use; D012189:Retrospective Studies; D016879:Salvage Therapy / Q000379:methods; D013866:Thioguanine / Q000627:therapeutic use; D016896:Treatment Outcome; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D011344:Procarbazine; D014750:Vincristine; D008130:Lomustine; D013866:Thioguanine; D008936:Mitolactol