title: Choroid plexus carcinoma in children: the Head Start experience.
abstract: BACKGROUND
  Choroid plexus carcinoma (CPC) is a rare aggressive intracranial neoplasm with a predilection for young children and a historically poor outcome. Currently, no defined optimal therapeutic strategy exists. The Head Start (HS) regimens have included irradiation-avoiding strategies in young children with malignant brain tumors using high dose chemotherapy to improve survival and minimize neurocognitive sequelae.
  
  
  METHODS
  Three sequential HS studies have been conducted from 1991 to 2009. HS treatment strategy has consisted of maximal surgical resection followed by five cycles of intensive induction followed by consolidation myeloablative chemotherapy with autologous hematopoietic stem cell rescue (AuHCR). Irradiation was given following recovery from consolidation based on the patient's age and evidence of residual disease.
  
  
  RESULTS
  Twelve children with CPC (median age of 19.5 months) have been treated with HS regimens. Ten patients had >95% resection. Three patients had disseminated disease at diagnosis. Ten patients completed consolidation of whom five are alive, irradiation and disease free at 29, 43, 61, 66 and 89 months from diagnosis. Seven patients experienced tumor recurrence/progression at a median time of 13 months (range 2-43 months). Five patients received irradiation, one for residual disease and four upon progression or recurrence, of whom one is alive at 61 months. The 3- and 5-year progression-free survivals are 58% and 38% and overall survivals 83% and 62% respectively. Late deaths from disease beyond 5 years were also noted.
  
  
  CONCLUSIONS
  Head Start strategies may produce long-term remission in young children with newly diagnosed CPC with avoidance of cranial irradiation.
authors: Division of Pediatric Hematology and Oncology, Department of Pediatrics, The University of Texas MD Anderson Cancer Center, Houston, Texas.
authors: Wafik
authors: W
authors: Zaky
authors: Girish
authors: G
authors: Dhall
authors: Soumen
authors: S
authors: Khatua
authors: Robert J
authors: RJ
authors: Brown
authors: Kevin F
authors: KF
authors: Ginn
authors: Sharon L
authors: SL
authors: Gardner
authors: Vedat O
authors: VO
authors: Yildiz
authors: Maxim
authors: M
authors: Yankelevich
authors: Jonathan L
authors: JL
authors: Finlay
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001932:Brain Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D016190:Carboplatin / Q000008:administration & dosage; D002277:Carcinoma / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D059248:Chemoradiotherapy; D002675:Child, Preschool; D016545:Choroid Plexus Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D002945:Cisplatin / Q000008:administration & dosage; D016371:Cranial Irradiation; D003520:Cyclophosphamide / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007390:International Agencies; D008297:Male; D009367:Neoplasm Staging; D011379:Prognosis; D011446:Prospective Studies; D015996:Survival Rate; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D014750:Vincristine; D005047:Etoposide; D003520:Cyclophosphamide; D016190:Carboplatin; D002945:Cisplatin
keywords: CNS tumors; chemotherapy; choroid plexus carcinoma; clinical trials; stem cell transplantation