Boesten Tineke, Gerber Nicolas U, Kandels Daniela, Azizi Amedeo A, Schmidt Rene, Warmuth-Metz Monika, Pietsch Torsten, Kortmann Rolf-Dieter, Gnekow Astrid, Grotzer Michael A
Department of Oncology, University Children's Hospital, Zurich, Switzerland (T.B., N.U.G., M.A.G.); Hospital for Children and Adolescents, Klinikum Augsburg, Augsburg, Germany (D.K., A.G.); Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria (A.A.A.); Institute of Biostatistics and Clinical Research, University of Muenster, Germany (R.S.); Department of Neuroradiology, University of Wuerzburg, Germany (M.W.); Institute of Neuropathology, University of Bonn, Germany (T.P.); Department of Radiation Oncology, University of Leipzig, Leipzig, Germany (R.K.).
Neurooncol Pract. 2017 Mar;4(1):29-39. doi: 10.1093/nop/npw007. Epub 2016 Dec 9.
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% ( = .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.
丘脑低级别胶质瘤（LGG）带来了特殊的治疗挑战，因为通常无法实现完全切除。为了确定单侧和双侧丘脑LGG的生存结果，我们分析了一大群儿科患者。
从1996年到2012年，2618名患者登记参加了HIT-LGG 1996和SIOP-LGG 2004研究。这2618名患者中共有102名（3.9%）被诊断为丘脑LGG，诊断时的中位年龄为8.0岁（范围0.4 - 17.5岁）；87名患者（85%）为单侧丘脑LGG，15名患者（15%）为双侧丘脑LGG。
90名患者接受了至少一次手术。31名患者接受了放疗，24名患者接受了化疗作为一线非手术治疗，这是由放射学肿瘤进展或严重/进行性临床症状所指示的。单侧丘脑肿瘤患者的10年总生存率（OS）为91%，而双侧丘脑肿瘤患者仅为65%（P = 0.001）。双侧丘脑肿瘤比单侧丘脑肿瘤更常表现为弥漫性组织学。弥漫性星形细胞瘤患者的10年OS（68%）低于毛细胞型星形细胞瘤患者（93%）。在开始首次非手术治疗后的10年无进展生存率，放疗组为53%，化疗组为34%。
丘脑胶质瘤可通过包括手术、观察、化疗和/或放疗的策略进行管理。在许多患者中可以成功推迟或避免放疗。单侧丘脑肿瘤患者的生存率较高。与双侧丘脑肿瘤相关的较差预后与该亚组中较高的弥漫性组织学发生率相关，这使得完全或接近完全切除成为不可能。
