Management of primary thalamic low-grade glioma in pediatric patients: results of the multicenter treatment studies HIT-LGG 1996 and SIOP-LGG 2004

Thalamic low-grade glioma (LGG) poses a special therapeutic challenge, as complete resection is often not possible. To determine the survival outcomes of mono- and bithalamic LGG, we analyzed a large cohort of pediatric patients.From 1996 until 2012, 2618 patients were registered in the HIT-LGG 1996 and the SIOP-LGG 2004 studies. A total of 102 of these 2618 patients (3.9%) were diagnosed with a thalamic LGG with a median age at diagnosis of 8.0 years (range, 0.4-17.5 years); 87 patients (85%) had monothalamic and 15 patients (15%) had bithalamic LGG.Ninety patients received at least one surgical procedure. Thirty-one patients received radiotherapy and 24 patients received chemotherapy as a first-line, nonsurgical treatment indicated by radiological tumor progression or severe/progressive clinical symptoms. Patients with monothalamic tumors showed a 10-year overall survival (OS) rate of 91%, whereas patients with bithalamic tumors only reached 65% (P = .001). Bithalamic tumors more frequently showed diffuse histology than monothalamic tumors. Patients with diffuse astrocytoma had a lower 10-year OS (68%) than those with pilocytic astrocytoma (93%). The 10-year progression-free survival rate after the start of first nonsurgical treatment was 53% in the radiotherapy group and 34% in the chemotherapy group.Thalamic glioma was manageable using a strategy that included surgery, observation, chemotherapy, and/or radiotherapy. Radiotherapy could be successfully deferred or obviated in a number of patients. Survival was high in among patients with monothalamic tumors. The worse prognosis associated with bithalamic tumors correlates with the higher rate of diffuse histology in this subgroup, precluding total or near-total resection.