GROWTH AND DEVELOPMENT IN CHILDREN AND AD OLESCENTS AFTER SURGERY, IRRADIATION AND CHEMOTHERAPY FOR RETINOBLASTOMA

Objective: Retinoblastoma is the most frequent intraocular tumor in in fancy and childhood that in its bilateral form is frequently cured by radiotherapy. As a consequence of the proximity of the tumor-harbourin g organ to the radiosensitive hypothalamo-pituitary unit, external rad iation of the tumor may cause endocrine disturbances and growth retard ation. Aim of the study was to determine the prevalence of decreased a dult height and major impairment of the hypothalamo-pituitary-sometome din and -gonadal axis and to investigate whether the radiation risk to hypothalamus and pituitary differs between radiation techniques. Meth ods: We reexamined 92 patients with bilateral retinoblastoma 5.3-24.2 years following radiotherapy (n = 80) or surgical therapy (n = 12), wi th or without chemotherapy. Height, pubertal development, and serum co ncentrations of IGF I, gonadotropins, testosterone and estradiol were assessed. Radiation sources included a 200-300 kV X-ray source (group 1: n = 37), an external beam Co-60 or Cs-137 source (group 2: n = 12), or a 5.7 MeV linear accelerator (group 3: n = 31). Results: Height wa s normal in the majority (84/92) of patients, 1 girl had short stature . Median height SDS ranged from 0.22 to 0.71 and did not differ betwee n radiated and surgically treated patients. Lowest height SDS were obs erved in patients of group 2 treated with doses of greater than or equ al to 60 Gy (- 1.09 and - 2.22; n = 2). Adult height SDS of all patien ts of group 1 was in the normal or tall range (- 1.1 - 2.6). Within th at range a negative association of total radiation dose and final heig ht SDS (r = - 0.48, P < 0.01) or final height in % of target height (r = -0.58, P < 0.002,) was observed. IGF I concentrations were normal i n all patients. 1 girl (group 2) developed mild precocious puberty, 1 young man and a young woman (groups 1 and 3) developed mild to moderat e hy pogonadotropic hypogonadism. Conclusions: Growth of retinoblastom a patients treated with x-rays or on external beam Co-60 or Cs-137 sou rce is not compromised and final height of patients treated with x-ray s is normal. Until more information is available, growth of retinoblas toma patients exposed to high-dose radiotherapy from a linear accelera tor source should be followed up carefully. Disorders of pubertal deve lopment must be anticipated in all groups.