Introduction: Craniopharyngiomas (CP) are benign, dysontogenic supra or intra-sellar tumours. They are locally aggressive with severe endocrine, neurological and ophthalmological implications. Somatotroph deficiencies rise therapeutic management problems due to increased risk of tumour growth and recurrence.
Case report: Male patient, aged 18 years 4 months, born naturally, late-term (42 weeks, 3200 g, Apgar 7), third child in a brotherhood of four (apparently healthy parents and brothers), with slightly delayed developmental acquisitions, was firstly evaluated in the Endocrinology Department at age 13 years 6 months for growth retardation. Investigations pleaded for pituitary dwarfism (short stature −2.5 S.D., absence of puberty signs, delayed bone age of 10 years, somatotroph axis: low basal GH with no stimulation to arginine, low IGF-1) and rhGH therapy was initiated. After just 5 months he was admitted to Neurosurgery Department due to severe headache, Jacksonian seizures and optochiasmatic syndrome determined by rapid developing para- and suprasellar compressive craniopharyngioma identified at MRI. Minimally invasive surgery was performed (partial ablation). On follow-up patient sustained panhypopituitarism and received corticoid and thyroid substitutive treatment. The evolution comprised of two other surgeries performed for tumour recurrence and external radiation (54 Gy/30 ft/CTV), associating further ophthalmological complications. At current evaluation, severe growth retardation (−5.11 S.D.), delayed bone age (11 years 6 months, fertile cartilages) and somatotroph deficiency raised the question of usefulness of rhGH treatment. Given frequent tumour recurrences and the presence of tumour residue, the histological type of CP determination becomes crucial for better adjustment in treatment strategy.
Discussions: Regarding published data on patients treated surgically and with rhGH, CP recurrence is reported at approximatively 4 years after surgery and two after rhGH therapy. Although GH receptors exist in some craniopharyngiomas, many authors consider rhGH safe. We emphasize the importance of close follow-ups of rhGH treatment, given the rapidly evolving tumour in our patient case.
10 - 12 Sep 2016
European Society for Paediatric Endocrinology