Broniscer Alberto, Laningham Fred H, Sanders Robert P, Kun Larry E, Ellison David W, Gajjar Amar
Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee 38105, USA.
Cancer. 2008 Aug 1;113(3):566-72. doi: 10.1002/cncr.23584.
Because diffuse pontine glioma (DPG) is rare among young children, the outcome of affected patients is unknown.
The authors reviewed clinical and radiologic characteristics of all children aged <3 years with DPG who were evaluated at their institution. Inclusion followed standard magnetic resonance imaging criteria for the diagnosis of DPG.
The median age at diagnosis in 10 patients was 2.2 years (range, 0.8-2.7 years). The median interval between the onset of symptoms and diagnosis was 2.5 months. All patients presented with cranial nerve palsy with (n = 7) or without (n = 3) other neurologic deficits attributable to brainstem involvement. All patients had pons-based tumors involving >50% of this brainstem segment. Histologic confirmation was attempted in 2 patients who had atypical radiologic features at diagnosis. Four patients initially were observed only. All patients received therapy, which consisted of radiation therapy (RT) (n = 2), RT and chemotherapy (n = 6), or chemotherapy only (n = 2). Four patients died of tumor progression after a median of 0.7 years (range, 0.5-3.7 years). Six patients have survived for a median of 2.3 years (range, 0.9-8 years). The 3-year progression-free and overall survival rates were 45% +/- 19% and 69% +/- 19%, respectively.
Children aged <3 years with DPG potentially may fare better than older patients with the same diagnosis despite the use of similar therapy. The current results suggested that DPG in younger children may be distinct biologically from similar tumors in older age groups.
由于弥漫性脑桥胶质瘤（DPG）在幼儿中较为罕见，因此受影响患者的预后尚不清楚。
作者回顾了在其机构接受评估的所有年龄小于3岁的DPG患儿的临床和放射学特征。纳入标准遵循诊断DPG的标准磁共振成像标准。
10例患者的诊断中位年龄为2.2岁（范围为0.8 - 2.7岁）。症状出现至诊断的中位间隔时间为2.5个月。所有患者均出现颅神经麻痹，伴有（n = 7）或不伴有（n = 3）其他因脑干受累导致的神经功能缺损。所有患者的肿瘤均位于脑桥，累及该脑干节段的50%以上。对2例诊断时具有非典型放射学特征的患者进行了组织学确诊尝试。4例患者最初仅接受观察。所有患者均接受了治疗，包括放射治疗（RT）（n = 2）、RT和化疗（n = 6）或仅化疗（n = 2）。4例患者在中位0.7年（范围为0.5 - 3.7年）后死于肿瘤进展。6例患者存活，中位生存期为2.3年（范围为0.9 - 8年）。3年无进展生存率和总生存率分别为45%±19%和69%±19%。
尽管采用了相似的治疗方法，但年龄小于3岁的DPG患儿的预后可能优于同诊断的年长患者。目前的结果表明，年幼儿童的DPG在生物学上可能与年龄较大组的相似肿瘤不同。
