ESPE Abstracts (2019) 92 P1-401

Pituitary, Neuroendocrinology and Puberty (2)

Management and Treatment Outcome of Childhood-Onset Craniopharyngioma (CP) in Italy: Multicentre Collection of 117 Cases

Stefano Zucchini1, Jacopo Fantini2, Diego Mazzatenta3, Gabriella Pozzobon4, Cristina Partenope4, Stefania Pedicelli5, Graziamaria Ubertini5, Maria Parpagnoli6, Lorenzo Genitori7, Rachele Menardi8, Daniela Driul9, Patrizia Matarazzo10, Gerdi Tuli10, Chiara Guzzetti11, Lorenzo Iughetti12, Tommaso Aversa13, Raffaella Di Mase14, Irene Rutigliano15, Maria Laura Iezzi16, Valentino Cherubini17, Anna Grandone18, Alessandra Cassio1,2


1Endo Unit Dept of Woman Child Health and Urologic Diseases AOU S.Orsola H, Bologna, Italy. 2University of Bologna, Bologna, Italy. 3Dept of Neurosurgery, DIBINEM, University of Bologna, Bologna, Italy. 4Dept of Pediatrics, San Raffaele Scientific Institute, MIlan, Italy. 5Endo Unit Bambino Gesù Children's Hospital, IRCCS, Rome, Italy. 6Auxend Ped Ginecology, AOU Meyer, Florence, Italy. 7Neuro-Oncology Unit, Dept of Pediatric Oncology, Meyer Children's Hospital, Florence, Italy. 8DAME, University of Udine, Udine, Italy. 9ASUIUD, Department of Pediatrics, Udine, Italy. 10SSD Endocrinology-O.I.R.M.-Città della Salute, Turin, Italy. 11Microcitemico H, Cagliari, Italy. 12UO Pediatria, Modena and Reggio Emilia University, Modena, Italy. 13Dept of Human Pathology in Adulthood and Childhood, University of Messina, Messina, Italy. 14DAI materno infantile AOU Federico II, Naples, Italy. 15Dept of Pediatrics IRCCS Casa Sollievo della Sofferenza, S.Giovanni Rotondo, Italy. 16Ped Clinic, Hospital District 'San Salvatore', L'Aquila, Italy. 17Dept of Women's and Children's Health, AOU Ospedali Riuniti, G. Salesi H, Ancona, Italy. 18Dept of Woman, Child and of General and Specialized Surgery, University of Campania Luigi Vanvitelli, Naples, Italy

In Italy, treatment of children with CP is not centralized. We collected data of 117 patients (pts) (M/F 56/41) with CP diagnosed after 01/01/2000, followed-up in 14 centres of paediatric endocrinology belonging to the I.S.P.E.D. Five centres provided data on more than 10 pts (range 12-19), while the remaining on 1-9. 46 pts were diagnosed between 2000-2010 and 71 afterwards. Follow-up was 7.5±4.1 yrs.

Results. Histology was adamantinomatous in 107 pts (91.4%), papillary in 6 pts (5.1%) and Rathke cyst in 4 (3.5%). The lesion was suprasellar in 92 pts (79%), involved the 3rd ventricle in 72 pts (61%). Age at intervention was 8.5±4 yrs (1 mo-18.6 yrs), with 16 pts operated before age 4 yrs. Surgery was mainly performed in Florence (26 pts), Milan (18 pts), Rome (16 pts), Udine (11 pts) and Bologna (10 pts). Craniotomic surgery was chosen in 75 pts (64%), transsphenoidal in 39 pts (33%) (3 pts missing). In Bologna 8/10 pts were operated transsphenoidally, whereas in Florence (13t/13c), Rome (0t/16c), Milan (4t/18c) and Udine (2t/9c) transsphenoidal surgery ranged from 0 to 50 % of pts. Surgery changed over time (2000-2010 vs 2011-2018) with craniotomic approach declining, not significantly, from 76% (35/46) to 58% (56/71) with transsphenoidal surgery increasing from 20% (9/46) to 43% (30/71) (P=0.01). Complete resection remained stable, from 64% (29/46) to 55% (39/71) between the 2 periods. Post-surgery complications occurred in 58 pts (50%) (8 pts missing) with Na electrolytes disorders in 19 pts followed by SAH in 12 pts and liquoral fistula (7 cases). They were similar between those operated cranially (37/75; 49%) vs transsphenoidally (20/39, 51%). Radiotherapy was used in 40 pts: 10 pts underwent γknife and 6 proton therapy. Recurrence occurred in 49 pts (42%): 1 in 30 pts, 2 in 15, 3 in 2, 4 in 1 and 6 in 1. In 98 pts hypothalamic syndrome was evaluated and occurred in 27 pts (27.5%). 9 pts also developed severe obesity (BMI > 2 SDS): this group (27+9 pts) had either craniotomic (26/64, 41%) or transsphenoidal surgery (10/34, 29%) (P=ns).

Conclusion. Our children with craniopharyngioma were diagnosed and treated at all ages in several centres around the country. The proportion of pts undergoing craniotomic vs transsphenoidal surgery varied significantly among the various centres. We confirm that recurrence occurred in about 40% of pts and hypothalamic disturbances in more than 1/3 of pts irrespective of type of surgery.