Multidisciplinary Management of Craniopharyngiomas in Children: A Single Center Experience
Giada Del Baldo,
Sabina Vennarini,
Antonella Cacchione,
Dante Amelio,
Maria Antonietta De Ioris,
Francesco Fabozzi,
Giovanna Stefania Colafati,
Angela Mastronuzzi,
Andrea Carai
Affiliations
Giada Del Baldo
Department of Onco-Hematology, Cell and Gene Therapy, Bambino Gesù Children’s Hospital, Scientific Institute for Reasearch, Hospitalization and Healthcare (IRCCS), 00165 Rome, Italy
Sabina Vennarini
Pediatric Radiotherapy Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, 20133 Milan, Italy
Antonella Cacchione
Department of Onco-Hematology, Cell and Gene Therapy, Bambino Gesù Children’s Hospital, Scientific Institute for Reasearch, Hospitalization and Healthcare (IRCCS), 00165 Rome, Italy
Dante Amelio
Proton Therapy Center, Azienda Provinciale per i Servizi Sanitari (APSS), 38123 Trento, Italy
Maria Antonietta De Ioris
Department of Onco-Hematology, Cell and Gene Therapy, Bambino Gesù Children’s Hospital, Scientific Institute for Reasearch, Hospitalization and Healthcare (IRCCS), 00165 Rome, Italy
Francesco Fabozzi
Department of Onco-Hematology, Cell and Gene Therapy, Bambino Gesù Children’s Hospital, Scientific Institute for Reasearch, Hospitalization and Healthcare (IRCCS), 00165 Rome, Italy
Giovanna Stefania Colafati
Department of Diagnostic Imaging Oncological Neuroradiology Unit, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, Italy
Angela Mastronuzzi
Department of Onco-Hematology, Cell and Gene Therapy, Bambino Gesù Children’s Hospital, Scientific Institute for Reasearch, Hospitalization and Healthcare (IRCCS), 00165 Rome, Italy
Andrea Carai
Neurosurgery Unit, Department of Neurosciences, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, Italy
Background: Craniopharyngioma (CP) is a rare brain tumor involving the sellar region. The best management is still debated. Gross total resection (GTR) is considered the best option to improve recurrence-free survival, but considerable long-term sequelae with a significant impact on quality of life have been reported. Subtotal resection followed by radiotherapy achieves similar disease control compared to GTR with less complications. Methods: We retrospectively reviewed 10 pediatric patients affected by CP treated with partial resection and subsequent proton therapy (PBT). We reviewed visual, endocrinological, and neuropsychological data at baseline, after surgery, and after radiation for all patients. Results: At the time of diagnosis, visual impairment was detected in 70% of patients and endocrinological abnormalities in 50%. All patients were subject to one or more surgical procedures. Surgery had no impact on visual status; however, it caused a worsening of endocrine function in half of patients. After surgery, all patients underwent PBT, achieving a partial response in 7 out of 10 patients (70%), while stable disease was observed in the other three patients (30%) at a median follow-up of 78 months from the end of PBT. Both visual and endocrine deficits were stable after PBT, with neurocognitive performance scores unchanged from baseline. Conclusions: A conservative surgical approach followed by PBT represents a safe and effective strategy to manage CP and limit long-term sequelae.