SIOP-E-BTG and GPOH Guidelines for Diagnosis and Treatment of Children and Adolescents with Low Grade Glioma

Abstract

Low grade gliomas (LGGs) constitute the largest, yet clinically and (molecular-) histologically heterogeneous group of pediatric brain tumors of WHO grades I and II occurring throughout all pediatric age groups and at all central nervous system (CNS) sites. The tumors are characterized by a slow growth rate and may show periods of growth arrest. Around 40% of all LGG patients can be cured by complete neurosurgical resection and are followed by close observation. In case of relapse, second resection often is possible. Following incomplete resection observation is recommended, as long as there is no radiologic tumor growth and the patient does not suffer from significant, tumor-related symptoms. This also applies to patients with a diagnosis of LGG on the basis of radiological criteria. By contrast, clinical worsening and / or radiologic progression are an indication to treatment with either chemo- or radiotherapy. Overall survival is around 90%, and many patients survive with residual tumor, i. e. they suffer from chronic disease. All patients need comprehensive neuro-oncological care, the principles and details of which are summarized in the current guidelines. These represent standard of care for diagnostic work-up (including neuroimaging and neuropathology), and for therapeutic decisions (including the indications to non-surgical treatment) as well as concepts for neurosurgical intervention, chemotherapy and radiotherapy as well as surveillance and rehabilitation. The current treatment algorithm was compiled by members of the LGG working group of the SIOP-E brain tumor group (SIOP-E-BTG) and is based upon the results of previous European LGG studies and international reports.

Loss of efficacy of subsequent nonsurgical therapy after primary treatment failure in pediatric low‐grade glioma patients—Report from the German SIOP‐LGG 2004 cohort

“…Extent of resection was confirmed by early postoperative scanning (obtained within the first 48 hours after surgery, up to 72 hours in exceptional circumstances) and combined surgical and radiological judgement. 26 Additional surgery for regulation of increased cranial pressure (ie, ventriculostomy, external cerebrospinal fluid drainage or ventricular-peritoneal shunting) was not considered for analysis.…”

The Visual Acuity Outcome and Relevant Factors Affecting Visual Improvement in Pediatric Sporadic Chiasmatic–Hypothalamic Glioma Patients Who Received Surgery

“…Current treatments for progressive symptomatic OPG include chemotherapy, radiation, and surgery for select patients (8). The aim of treatments for OPG increasingly focuses on improving the patient's QOL, such as VA rescue, but information on OPG long-term VA outcome is scant (13,14). The largest study of longterm VA outcomes of OPG was reported by Rakotonjanahary et al (15).…”

“…"Maintained" was used when the LVA level was equal to the IVA level, and "deteriorated" was used when the LVA level was lower than the IVA level. Tumor volume was calculated using the ellipsoid volume formula "A × B × C × π/6" (Figure 1) (13). Tumors were divided into small, medium, and large size groups by tertiles.…”

Prognostic impact of distinct genetic entities in pediatric diffuse glioma WHO‐grade II—Report from the German/Swiss SIOP‐LGG 2004 cohort

“…For radiological response assessment, contrast‐enhanced MRI was performed at defined intervals at week 24, 54 and 85 after the start of nonsurgical therapy. Complete, partial and objective responses (regression) as well as stable disease (SD) were considered positive responses 19,26 …”