Late sequela after treatment of childhood low-grade gliomas: a retrospective analysis of 69 long-term survivors treated between 1983 and 2003

Abstract

The aim of the present study was to evaluate the spectrum of late effects in a large cohort of pediatric patients with low-grade gliomas (WHO grade I and II) during an observation period of 20 years. Eighty-seven patients with low-grade gliomas grouped according to tumor location (cerebellum: n=28; cerebral hemispheres: n=21; central midline: n=15; brainstem: n=12; tectum: n=5; other locations: n=6) were evaluated for tumor- and/or treatment-related late effects by analysis of medical and computer records, and personal interviews. Seventy patients underwent neurosurgery, 29 patients received additional radiotherapy and 20 additional chemotherapy. Median follow-up of survivors is 96 months with an overall survival of 79% (cerebellum: 89%; cerebral hemispheres: 95%; central midline: 80%; brainstem: 25%; tectum: 100%; other locations: 66%). Chronic medical problems (mild ataxia to multiple severe neuroendocrine deficits) are observed in 100% of patients with brainstem/central midline tumors and in 40-50% of patients with low-grade gliomas of other locations. Endocrine deficiencies were observed in 15/17 (88%) of long-term survivors who received radiotherapy. In contrast, none of the patients who underwent surgery only had endocrine deficiencies. Seven long-term survivors (10.1%) are severely disabled with permanent need of medical help. Tumor- and treatment-related late effects are common in patients with low-grade gliomas with the most severe occurring in patients with brainstem or central midline tumors. As long-term survival is excellent in patients with low-grade gliomas except for tumors located in the brainstem, future treatment studies should focus on avoiding long-term late effects.

Clinical and treatment factors determining long‐term outcomes for adult survivors of childhood low‐grade glioma: A population‐based study

“…1 Although patients with PLGG may undergo multiple progressions, 1,2 the short-term (<5 years) and intermediate-term (5-10 years) overall survival (OS) is excellent, and most children survive to adulthood. [3][4][5][6][7] A lack of consistent follow-up poses a challenge for accurate data collection, and the long-term outcomes of adult survivors of PLGG are seldom reported. 5 Determinants of short-term tumor-related outcomes [3][4][5][6][7] include patient-related factors, such as age and an underlying diagnosis of neurofibromatosis type 1 (NF1), 8,9 and tumor-related factors, such as tumor extension and certain pathological subtypes.…”

“…[3][4][5][6][7] A lack of consistent follow-up poses a challenge for accurate data collection, and the long-term outcomes of adult survivors of PLGG are seldom reported. 5 Determinants of short-term tumor-related outcomes [3][4][5][6][7] include patient-related factors, such as age and an underlying diagnosis of neurofibromatosis type 1 (NF1), 8,9 and tumor-related factors, such as tumor extension and certain pathological subtypes. 6,10,11 Although complete surgical resection is consistently associated with improved survival, the extent of resection highly depends on the tumor location, and resection is feasible only for a minority of tumors.…”

Female Pelvis: Genital Organs

“…Acute and long-term functional outcome after neurosurgery largely depends on the anatomical site of the tumor, neurosurgical technique, expertise of the surgeon, as well as the patient's age and clinical condition prior to surgery (Müller 2013 ;Pillai et al 2012 ;Benesch et al 2006 ;Wendt et al 2014 ). Postoperative defi cits such as neurovascular events (stroke), seizures, palsies, oculomotor, visual and coordination impairments, as well as diencephalic damage with endocrinopathies and reduced neurocognitive function are not rare, and both adjuvant RT and chemotherapy may be covariates (Sterkenburg et al 2014 ;Hoffmann et al 2014 ;Özyurt et al 2014 ).…”

Approaches Toward Improving the Prognosis of Pediatric Patients With Glioma: Pursuing Mutant Drug Targets With Emerging Small Molecules

“…2 Adjuvant radiotherapy can contribute to significant neurological morbidity and conventional chemotherapy with alkylating agents has achieved limited improvement in progression free or overall survival. [3][4][5][6] Paediatric astrocytomas differ considerably from their adult counterparts in the spectrum and frequency of their mutations. Many of the common genetic abnormalities in adult glioma are not present in the paediatric tumours, particularly amplification and/or overexpression of EGFR and VEGRF and deletion/mutation of PTEN and hence, small tyrosine kinase inhibitors targeting these receptors are ineffective for treating paediatric astrocytoma.…”