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Glioneuronal tumors in childhood - Before and after surgery. A long-term follow-up study
Uppsala universitet, Medicinska och farmaceutiska vetenskapsområdet, Medicinska fakulteten, Institutionen för kvinnors och barns hälsa, Forskargrupper (Inst. för kvinnor och barns hälsa), Barnneurologi/Barnonkologi.
Uppsala universitet, Medicinska och farmaceutiska vetenskapsområdet, Medicinska fakulteten, Institutionen för kirurgiska vetenskaper, Radiologi.
Uppsala universitet, Medicinska och farmaceutiska vetenskapsområdet, Medicinska fakulteten, Institutionen för immunologi, genetik och patologi, Klinisk och experimentell patologi.ORCID-id: 0000-0001-7376-7331
Uppsala universitet, Medicinska och farmaceutiska vetenskapsområdet, Medicinska fakulteten, Institutionen för kvinnors och barns hälsa, Forskargrupper (Inst. för kvinnor och barns hälsa), Barnneurologi/Barnonkologi.
Vise andre og tillknytning
2017 (engelsk)Inngår i: Epilepsy & Behavior, ISSN 1525-5050, E-ISSN 1525-5069, Vol. 72, s. 82-88Artikkel i tidsskrift (Fagfellevurdert) Published
Abstract [en]

Aim: To give a detailed description of the long-term outcome of a cohort of children with glioneuronal tumors regarding pre-and postsurgical factors, including "dual" and "double" pathology, seizure freedom, and psychosocial outcome.

Methods: During a fifteen-year period (1995-2009), all patients (age 0-17.99 years) with a glioneuronal brain tumor diagnosed and treated at Uppsala University Children's Hospital were identified from the National Brain Tumor Registry and the National Epilepsy Surgery Registry. Hospital medical records were reviewed and neuroradiological and neuropathological findings were re-evaluated. A cross-sectional long-term follow-up prospective evaluation, including an interview, neurologic examination, and electroencephalogram, was accomplished in patients accepting participants in the study.

Results: A total of 25 out of 28 (89%) eligible patientswere included. The M: F ratiowas 1.5: 1. Mean follow-up time after surgery was 12.1 years (range 5.0-19.3). Twenty patients were adults (N18 years) at follow-up. Seizure freedomwas achieved in 64%. Gross total resection (GTR) was the only preoperative factor significantly correlating to seizure freedom (p= 0.027). Thirty-eight percent were at some time postoperatively admitted for a psychiatric evaluation. There was a trend towards both higher educational level and employment status in adults who became seizure free.

Conclusion: Long-termoutcome is good regarding seizure freedom if GTR can be achieved, but late seizure recurrence can occur. "Dual" and "double" pathology is uncommon and does not influence seizure outcome. Obtaining seizure freedomseems to be important for psychosocial outcome, but there is a risk for psychiatric comorbidities and long-term follow-up by a multi-professional team is advisable.

sted, utgiver, år, opplag, sider
ACADEMIC PRESS INC ELSEVIER SCIENCE , 2017. Vol. 72, s. 82-88
Emneord [en]
Glioneuronal tumor, Childhood, "Dual" pathology, "Double" pathology, Seizure outcome, Psychosocial outcome
HSV kategori
Identifikatorer
URN: urn:nbn:se:uu:diva-333399DOI: 10.1016/j.yebeh.2017.02.012ISI: 000406321300015PubMedID: 28575773OAI: oai:DiVA.org:uu-333399DiVA, id: diva2:1157478
Tilgjengelig fra: 2017-11-16 Laget: 2017-11-16 Sist oppdatert: 2019-01-07bibliografisk kontrollert
Inngår i avhandling
1. Glioneuronal tumours in childhood: Clinical picture, long-term outcome and possible new treatments
Åpne denne publikasjonen i ny fane eller vindu >>Glioneuronal tumours in childhood: Clinical picture, long-term outcome and possible new treatments
2019 (engelsk)Doktoravhandling, med artikler (Annet vitenskapelig)
Abstract [en]

Background: Glioneuronal tumours are a subgroup of low-grade tumours of the central nervous system (CNS), often causing epilepsy. Overall survival is excellent, but data regarding long-term seizure outcome and late effects are scarce.

Aims: The overall aim was to gather data about pre- and postsurgical factors of importance and long-term outcomes to improve standards of care. Another aim was to explore the expression of somatostatin receptor (SSTR) subtypes and mTOR pathway markers.

Methods: This thesis, based on four population-based studies with both retrospective and cross-sectional parts, was performed through a long-term follow-up of a Swedish cohort of children with glioneuronal tumours in the Uppsala-Örebro health region. Patients were identified from the National Brain Tumour Registry and the National Epilepsy Surgery Registry. Various methods were used: reviews of hospital medical records, patient interviews, health-related quality of life (HRQoL) assessments with generic (Short Form 36version2) and disease specific (Quality of Life in Epilepsy-31) questionnaires, neuropsychological evaluations with Wechsler Intelligence Scale for Children-IV or Wechsler Adult Intelligence Test-IV and Reys Complex Figure Test and evaluation for possible depression with Hospital Anxiety Depression Scale. Immunohistochemical analyses for SSTR subtypes 1, 2a, 3 and 5 and mTOR pathway components ezrin-radixin-moesin and pS6 were performed on tumour specimens.

Results: Glioneuronal tumours seem to be more frequent than previously reported, accounting for 13.5% of all childhood CNS tumours. They often cause medically refractory epilepsy resulting in cognitive impairment. Neurosurgery was often delayed; mean time from symptom debut to lesionectomy was 4.6 years. Long-term seizure freedom was achieved in 84% of patients who had a gross total resection (GTR) and is important for long-term cognitive restitution, HRQoL, educational and vocational outcomes. SSTR2a and SSTR3 expression was a frequent finding in glioneuronal tumours. Signs of mTOR pathway activation were abundant in ganglioglioma.

Conclusions: A safe GTR should be striven for and considered a first-line treatment. Long-term clinical follow-up should be offered to all patients and for those with an inoperable tumour/tumour remnant causing tumour growth and/or medically refractory epilepsy, somatostatin analogues and/or mTOR inhibitors might represent a therapeutic alternative worth exploring further.

sted, utgiver, år, opplag, sider
Uppsala: Acta Universitatis Upsaliensis, 2019. s. 66
Serie
Digital Comprehensive Summaries of Uppsala Dissertations from the Faculty of Medicine, ISSN 1651-6206 ; 1530
Emneord
Glioneuronal tumour, ganglioglioma, dysembryoplastic neuroepithelial tumour, childhood, cognition, psychosocial, HRQoL, outcome, mTOR pathway, somatostatin receptor
HSV kategori
Forskningsprogram
Pediatrik
Identifikatorer
urn:nbn:se:uu:diva-371907 (URN)978-91-513-0549-3 (ISBN)
Disputas
2019-03-01, Rosénsalen, Akademiska Barnsjukhuset, ingång 95/96 nbv., Uppsala, 09:15 (svensk)
Opponent
Veileder
Tilgjengelig fra: 2019-02-06 Laget: 2019-01-07 Sist oppdatert: 2019-02-18

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