Stefano Zucchini1, Natascia Di Iorgi2,3, Gabriella Pozzobon4, Stefania Pedicelli5, Maria Parpagnoli6, Daniela Driul7, Patrizia Matarazzo8, Federico Baronio1, Marco Crocco2,9, Giovanna Iudica2, Cristina Partenope4, Beatrice Nardini6, Graziamaria Ubertini5, Rachele Menardi10, Chiara Guzzetti11, Lorenzo Iughetti12, Tommaso Aversa13, Raffaella Di Mase14, Alessandra Cassio1. 1. Pediatric Endocrine Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Endo-ERN Center for Rare Endocrine Conditions, 40138 Bologna, Italy. 2. Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genova, 16147 Genoa, Italy. 3. Department of Pediatrics, IRCCS Istituto Giannina Gaslini, 16147 Genoa, Italy. 4. Department of Paediatrics, IRCCS San Raffaele Scientific Institute, Vita Salute San Raffaele University, Endo-ERN Center for Rare Endocrine Conditions, 20132 Milan, Italy. 5. Dipartimento Pediatrico Universitario Ospedaliero, IRCCS "Bambino Gesù" Children's Hospital, 00165 Rome, Italy. 6. Auxo-Endocrinology and Gynecology Meyer Children's University Hospital, 50139 Florence, Italy. 7. Division of Pediatrics, University Hospital Santa Maria della Misericordia, ASUFC, 33100 Udine, Italy. 8. Department of Pediatric Endocrinology, Regina Margherita Children's Hospital, University of Turin, 10126 Turin, Italy. 9. Neuro-Oncology Unit, IRCCS Istituto Giannina Gaslini, 16147 Genoa, Italy. 10. Division of Paediatrics, Department of Medicine (DAME) University of Udine, 33100 Udine, Italy. 11. Pediatric Endocrine Unit, Pediatric Hospital Microcitemico Antonio Cao, AO Brotzu, 09121 Cagliari, Italy. 12. Pediatric Unit, Department of Medical and Surgical Sciences for Mothers, Children and Adults, University of Modena and Reggio Emilia, 41124 Modena, Italy. 13. Unit of Paediatrics, Department of Human Pathology of Adulthood and Childhood, University of Messina, 98124 Messina, Italy. 14. University Federico II, Department of Translational Medical Sciences, 80131 Naples, Italy.
Abstract
CONTEXT: Nationwide data on children diagnosed with craniopharyngioma (CP) are not available in Italy. OBJECTIVE: This work aimed to identify patients' characteristics, type of surgical approach, complications and recurrences, number of pituitary deficits, and number of patients starting growth hormone (GH) treatment. METHODS: A retrospective multicenter collection took place of 145 patients aged 0 to 18 years who underwent surgery for CP between 2000 and 2018, and followed up in 17 Italian centers of pediatric endocrinology. RESULTS: Age at diagnosis was 8.4 ± 4.1 years. Duration of symptoms was 10.8 ± 12.5 months and headache was most frequent (54%), followed by impaired growth (48%) and visual disturbances (44%). Most lesions were suprasellar (85%), and histology was adamantinomatous in all cases but two. Surgical approach was transcranial (TC) in 67.5% of cases and transsphenoidal (TS) in 31.%. The TC approach was prevalent in all age groups. Postsurgery complications occurred in 53% of cases, with water-electrolyte disturbances most frequent. Radiotherapy was used in 39% of cases. All patients but one presented with at least one hormone pituitary deficiency, with thyrotropin deficiency most frequent (98.3%), followed by adrenocorticotropin (96.8%), arginine vasopressin (91.1%), and GH (77.4%). Body mass index (BMI) significantly increased over time. A hypothalamic disturbance was present in 55% of cases. GH therapy was started during follow-up in 112 patients at a mean age of 10.6 years, and 54 developed a recurrence or regrowth of the residual lesion. CONCLUSION: CP is often diagnosed late in Italy, with TC more frequent than the TS surgical approach. Postsurgery complications were not rare, and hypopituitarism developed almost in all cases. BMI shows a tendency to increase overtime.
CONTEXT: Nationwide data on children diagnosed with craniopharyngioma (CP) are not available in Italy. OBJECTIVE: This work aimed to identify patients' characteristics, type of surgical approach, complications and recurrences, number of pituitary deficits, and number of patients starting growth hormone (GH) treatment. METHODS: A retrospective multicenter collection took place of 145 patients aged 0 to 18 years who underwent surgery for CP between 2000 and 2018, and followed up in 17 Italian centers of pediatric endocrinology. RESULTS: Age at diagnosis was 8.4 ± 4.1 years. Duration of symptoms was 10.8 ± 12.5 months and headache was most frequent (54%), followed by impaired growth (48%) and visual disturbances (44%). Most lesions were suprasellar (85%), and histology was adamantinomatous in all cases but two. Surgical approach was transcranial (TC) in 67.5% of cases and transsphenoidal (TS) in 31.%. The TC approach was prevalent in all age groups. Postsurgery complications occurred in 53% of cases, with water-electrolyte disturbances most frequent. Radiotherapy was used in 39% of cases. All patients but one presented with at least one hormone pituitary deficiency, with thyrotropin deficiency most frequent (98.3%), followed by adrenocorticotropin (96.8%), arginine vasopressin (91.1%), and GH (77.4%). Body mass index (BMI) significantly increased over time. A hypothalamic disturbance was present in 55% of cases. GH therapy was started during follow-up in 112 patients at a mean age of 10.6 years, and 54 developed a recurrence or regrowth of the residual lesion. CONCLUSION: CP is often diagnosed late in Italy, with TC more frequent than the TS surgical approach. Postsurgery complications were not rare, and hypopituitarism developed almost in all cases. BMI shows a tendency to increase overtime.