title: Treatment of peripheral neuroepithelioma in children and young adults.
abstract: Seventeen patients with peripheral neuroepithelioma were treated with an intensive chemotherapy regimen of vincristine, Adriamycin (Adria Laboratories, Columbus, OH), and cyclophosphamide (VADRIAC) in combination with radiation therapy. Fifteen patients with stage III (seven) or stage IV (eight) at presentation were treated on a more intensive regimen including total body irradiation (TBI) (8 Gy). Two patients with stage I (one) or II (one) disease received a less intensive chemotherapy regimen of VADRIAC. Therapy was completed within 6 to 7 months in all patients. The disease arose in the chest wall in 12 patients, pelvis in three patients, and extremity in two patients. Sixteen of the 17 (94%) patients achieved a complete remission. With a median follow-up of 18 months, ten patients remain in complete remission with an actuarial survival of 68% and an actuarial relapse-free survival of 56% at 12 months. On the basis of our initial experience with this tumor, we believe that peripheral neuroepithelioma is a chemoresponsive and radioresponsive tumor.
authors.affiliation: National Cancer Institute, Bethesda, MD.
authors.forename: J S
authors.initials: JS
authors.lastname: Miser
authors.forename: T J
authors.initials: TJ
authors.lastname: Kinsella
authors.forename: T J
authors.initials: TJ
authors.lastname: Triche
authors.forename: R
authors.initials: R
authors.lastname: Steis
authors.forename: M
authors.initials: M
authors.lastname: Tsokos
authors.forename: R
authors.initials: R
authors.lastname: Wesley
authors.forename: K
authors.initials: K
authors.lastname: Horvath
authors.forename: J
authors.initials: J
authors.lastname: Belasco
authors.forename: D L
authors.initials: DL
authors.lastname: Longo
authors.forename: E
authors.initials: E
authors.lastname: Glatstein
mesh_terms: D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000008:administration & dosage; D003609:Dactinomycin / Q000008:administration & dosage; D005121:Extremities; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000188:drug therapy / Q000532:radiotherapy / Q000628:therapy*; D010386:Pelvic Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000628:therapy*; D011379:Prognosis; D011879:Radiotherapy Dosage; D012512:Sarcoma, Ewing / Q000628:therapy; D013899:Thoracic Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000628:therapy*; D013997:Time Factors; D014750:Vincristine / Q000008:administration & dosage; D014916:Whole-Body Irradiation
chemical_list: D003609:Dactinomycin; D014750:Vincristine; D003520:Cyclophosphamide