Childhood craniopharyngioma in Macedonia: incidence and outcome after subtotal resection and cranial irradiation
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Childhood craniopharyngioma in Macedonia: incidence and outcome after subtotal resection and cranial irradiation Zoran S Gucev, Dragan Danilovski, Velibor Tasic, Jovica Ugrinovski, Vesna Nastova, Aleksandra Jancevska, Marina Krstevska-Konstantinova, Nada Pop-Jordanova, Ilija Kirovski Original article
Skopje, Macedonia 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.
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.
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.
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.
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
World J Pediatr 2011;7(1):74-78
Author Affiliations: Medical Faculty Skopje, 50 Divizija BB, 1000 Skopje, Macedonia (Gucev ZS, Danilovski D, Tasic V, Ugrinovski J, Nastova V, Jancevska A, Krstevska-Konstantinova M, Pop-Jordanova N, Kirovski I) Corresponding Author: Zoran S Gucev, Medical Faculty Skopje, 50 Divizija BB, 1000 Skopje, Macedonia (Tel: 00 389 70 279 742; Fax: 00 389 2 3111 366; Email: [email protected]) doi:10.1007/s12519-011-0250-3 ©Children's Hospital, Zhejiang University School of Medicine, China and Springer-Verlag Berlin Heidelberg 2011. All rights reserved.
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Key words: craniopharyngioma; growth; incidence; outcome; partial resection
Introduction
C
raniopharyngioma accounts for 5%-15% of intracranial neoplasms in children.[1] The tumor may be detected at any age including the prenatal and neonatal period.[1] Hormonal deficiency, growth failu
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