ESPE Abstracts (2019) 92 P1-106

Management and Treatment Outcome of Craniopharyngiomas in Young Children Before 4 Years of Age in Italy: Multicentre Collection of 16 Cases

Federico Baronio1, Mino Zucchelli2, Tommaso Aversa3, Daniela Driul4, Chiara Guzzetti5, Lorenzo Iughetti6, Patrizia Matarazzo7, Maria Parpagnoli8, Stefania Pedicelli9, Gabriella Pozzobon10, Mariacarolina Salerno11, Stefano Zucchini1


1Endo Unit, Dept of Woman, Child and Urologic Diseases, AOU S.Orsola-Malpighi H, Bologna, Italy. 2IRCCS Institute of Neurological Science, Pediatric Neurosurgery, Bellaria Hospital, Bologna, Italy. 3Dept of Human Pathology in Adulthood and Childhood, University of Messina, Messina, Italy. 4Division of Ped Endocrinology, Dept of Pediatrics, University of Udin, Udine, Italy. 5Pediatric Endocrinology Unit, Microcitemico H, Cagliari, Italy. 6Pediatric Unit, Modena and Reggio Emilia University, Modena, Italy. 7SSD Endocrinology-O.I.R.M.-Città della Salute, Torino, Italy. 8Auxend Ped Ginecology, AOU Meyer, Firenze, Italy. 9Endo Unit Bambino Gesù Children's Hospital, IRCCS, Roma, Italy. 10Dept of Pediatrics, San Raffaele Scientific Institute, Milano, Italy. 11Dept of Pediatrics, University Federico II, Napoli, Italy


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.