Adult height after cranial irradiation for optic pathway tumors: relationship with neurofibromatosis.
OBJECTIVE
Optic pathway tumors decrease adult height by central precocious puberty (PP) or hypothalamic-pituitary disorders, particularly growth hormone (GH) deficiency caused by the tumor, management of the tumor, or associated neurofibromatosis 1. The objective of this study was to evaluate the respective roles of these factors on disorders and adult height.


METHODS
Thirty-five patients with optic pathway tumors diagnosed at 6.4 +/- 0.6 years treated by cranial irradiation (30-56 Gy) reached adult height after treatment with GH alone (n = 16), gonadotropin hormone-releasing hormone analogue alone (n = 2), or both (n = 16).


RESULTS
Central precocious puberty occurred before irradiation in four cases and after irradiation in 18. Eleven of the 17 patients with neurofibromatosis 1 had PP. Before irradiation, five of 21 patients lacked GH, zero of 21 lacked thyroid-stimulating hormone, and zero of 13 lacked adrenocorticotropin. After irradiation, 35 of 35 lacked GH, 28 of 35 lacked thyroid-stimulating hormone, and six of 35 lacked adrenocorticotropin; puberty was delayed in 15 patients. Adult height was -0.8 +/- 0.2 SD, below target height (0.2 +/- 0.2 SD, P <.0001), similar in patients with and without PP, but lower in those with neurofibromatosis 1 (-1.4 +/- 0.4 SD) than in those without (-0.3 +/- 0.3 SD, P =.04).


CONCLUSIONS
Optic pathway tumors cause PP, but cranial irradiation causes most of the other hypothalamic-pituitary disorders. Adult height of patients given GH or gonadotropin hormone-releasing hormone analogue seems to depend on neurofibromatosis 1.
Huguenin
Marion
M
Université René Descartes, Paris, France.
Trivin
Christine
C
Zerah
Michel
M
Doz
François
F
Brugieres
Laurence
L
Brauner
Raja
R
D001827:Body Height / Q000502:physiology / Q000528:radiation effects; D002648:Child; D016371:Cranial Irradiation / Q000009:adverse effects*; D005260:Female; D006130:Growth Disorders / Q000503:physiopathology*; D006801:Humans; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications / Q000503:physiopathology; D019574:Optic Nerve Neoplasms / Q000503:physiopathology / Q000532:radiotherapy*; D010910:Pituitary Irradiation / Q000009:adverse effects; D011629:Puberty, Precocious / Q000150:complications / Q000503:physiopathology; D011879:Radiotherapy Dosage