Introduction: Craniopharyngiomas (CP) are rare pediatric intracranial tumors (1.2-4%) with a peak of incidence between 5-14 years . We retrospectively reviewed data of 16 cases (M/F 4/12) diagnosed before 4 years of age (median follow up 7.2 years) from a cohort of 117 patients (pts) (M/F 56/41) diagnosed after 01/01/2000, followed-up in 14 Italian centres of pediatric endocrinology belonging to the Italian Society for Pediatric Endocrinology and Diabetology.
Results: The pts were treated in 8 different centers across Italy. Median age at diagnosis was 2.7 years (yrs) (range 0.1-3.7) with a delay of 6 months (0-2.3 yrs) from the symptoms onset: visual impairment (5 pts), headache (6 pts),vomiting (4 pts), polyuria/polydipsia (2 pts), ataxia (1pt) fatigue (1pt). One pt was diagnosed prenatally. Neuroimaging showed 2 solid, 5 cystic and 9 mixed tumors, localized intrasellar (3 pts) and suprasellar (13 pts), with 3rd ventricle involvement in 8 pts. Median tumor size was 34,5 mm ( range 30-70). The surgery approach was transsphenoidal (TS) in 5 pts ( 31%), craniotomic (CT) in 11 pts ( 69%). Radical tumor removal was obatined in 10/16 pts (62%). Histology confirmed adamantinous CP in 15/16 cases and papillary CP in one case. 12/16 pts early after surgery started the substitutive treatment with l-thyroxin, hydrocortisone and DDAVP for multiple pituitary hormone deficiencies.
11/16 cases started hGH treatment ( median dose 0.07-0.2 mg/kg/wk) after 1.28 (0.5-2.6) years after diagnosis of CP for GH deficiency and growth impairment.
Recurrences occurred in 11/16 pts (69%), 1 in 4 pts, 2 in 6, 3 in 1. The first relapse occurred 6 months (range 0.4-4 yrs) after surgery, in 5/11 pts despite radical tumor removal. Recurrences were treated with surgical reintervention, associated with radiotherapy in 5 cases (1 γ-knife, 2 proton, 2 conventional). In the 5 patients who underwent TS radical surgery no CP relapses arose until last control. 3 /11 pts relapsed during the first 2 years of GH treatment.4/16 (3 CT/1 TS) pts showed overweight at last control with BMI SDS 1.8-2.6, 3/4 with hypotalamic syndrome (HS).
Conclusion: CP in our children showed an aggressive behavior, with severe symptoms at diagnosis, relapsed in 69% of cases even in those with radical removal. In all cases at least 3 pituitary hormone deficiency arose after treatment, hGH seems not to increase the incidence of recurrences. HS is a frequent complication irrespective of type of surgery.
19 - 21 Sep 2019
European Society for Paediatric Endocrinology