title: Childhood craniopharyngioma in Macedonia: incidence and outcome after subtotal resection and cranial irradiation.
abstract: BACKGROUND
  craniopharyngioma is a frequent tumor in children with challenging surgical, endocrine, and visual consequences. We evaluated our experience in treating craniopharyngioma and its incidence in Macedonia.
  
  
  METHODS
  Thirteen children (9 male and 4 female) with craniopharyngioma (age 9.55 ± 3.74 years; range 2.90-15.11) who had been treated between 1989 and 2008 in Macedonia were reviewed.
  
  
  RESULTS
  initial signs were vision disturbances (10 children), seizures (1), growth retardation (13), and diabetes insipidus (DI) (2). All children were subjected to subtotal surgical removal. Cranial irradiation was performed in 12 of the 13 children, and intracystic bleomycin was given to one child. The patients were followed up for 6-229 months (mean ± SD: 107.00 ± 74.04 months). All children had multiple pituitary deficiencies after surgical removal of the tumor. Body mass index increased from 16.93 ± 6.34 standard deviation scores (SDS) at diagnosis to 26.33 ± 5.91 SDS (P>0.005) at the last follow-up. DI was permanent in 9 of the 13 children, and multiple pituitary deficiencies were seen in all children. Treatment with growth hormone resulted in normalization of adult height from -1.27 ± 1.52 SDS at the start of the treatment to -0.13 ± 1.39 SDS at the last followup. The final height was not significantly lower than the genetic target height (P>0.005). The permanent deficit was visual impairment: blindness in one or both eyes in 4 children, bitemporal hemianopsia in 4, and other defects in 2. Recurrence of the disease was ruled out in one child after 31 months. No mortality was observed in the observation period of 104.92 ± 76.11 months.
  
  
  CONCLUSIONS
  the overall incidence of craniopharyngioma in the period of 1989-2008 in Macedonia was 1.43 per 1 000 000 person-years. Subtotal resection and systematic irradiation showed good life quality of survivors.
authors.affiliation: Medical Faculty Skopje, 50 Divizija BB, 1000 Skopje, Macedonia. gucevz@gmail.com
authors.forename: Zoran S
authors.initials: ZS
authors.lastname: Gucev
authors.forename: Dragan
authors.initials: D
authors.lastname: Danilovski
authors.forename: Velibor
authors.initials: V
authors.lastname: Tasic
authors.forename: Jovica
authors.initials: J
authors.lastname: Ugrinovski
authors.forename: Vesna
authors.initials: V
authors.lastname: Nastova
authors.forename: Aleksandra
authors.initials: A
authors.lastname: Jancevska
authors.forename: Marina
authors.initials: M
authors.lastname: Krstevska-Konstantinova
authors.forename: Nada
authors.initials: N
authors.lastname: Pop-Jordanova
authors.forename: Ilija
authors.initials: I
authors.lastname: Kirovski
mesh_terms: D000293:Adolescent; D000903:Antibiotics, Antineoplastic / Q000008:administration & dosage; D001761:Bleomycin / Q000008:administration & dosage; D002648:Child; D002675:Child, Preschool; D003397:Craniopharyngioma / Q000150:complications / Q000453:epidemiology / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D003919:Diabetes Insipidus / Q000188:drug therapy / Q000209:etiology; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D006130:Growth Disorders / Q000188:drug therapy / Q000209:etiology; D019382:Human Growth Hormone / Q000627:therapeutic use; D006801:Humans; D015994:Incidence; D015552:Injections, Intralesional; D008297:Male; D010911:Pituitary Neoplasms / Q000150:complications / Q000453:epidemiology / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D011788:Quality of Life; D018714:Radiotherapy, Adjuvant / Q000379:methods; D018843:Republic of North Macedonia / Q000453:epidemiology; D012189:Retrospective Studies; D012640:Seizures / Q000209:etiology; D016896:Treatment Outcome; D014786:Vision Disorders / Q000209:etiology
chemical_list: D000903:Antibiotics, Antineoplastic; D001761:Bleomycin; D019382:Human Growth Hormone