Thompson Willard Darrell, Kosnik Edward J
Section of Pediatric Neurosurgery, Department of Surgery, Children's Hospital of Columbus, Ohio, USA.
J Neurosurg. 2005 Jan;102(1 Suppl):65-71. doi: 10.3171/ped.2005.102.1.0065.
The authors present two cases of diffuse brainstem lesions that regressed without treatment. Two newborns presented with cranial nerve palsies and limb weakness at birth. Magnetic resonance (MR) images obtained in the 1st week of life revealed a large, expansive pontomedullary lesion in each patient. Findings of clinical and imaging examinations were highly consistent with the characteristics of diffuse brainstem glioma. After consultation with the parents of both infants, all parties agreed to forgo the treatment modalities available at the time. Neither patient underwent surgery, radiation treatment, or chemotherapy; both underwent routine neurological and MR imaging examinations. Within weeks the patient in Case 1 started to improve clinically and at 4 years of age has reached nearly all developmental milestones. Serial MR images demonstrated a steady decrease in the size of the lesion. The patient in Case 2 improved in a similar manner and is now 10 years old. The findings from these two cases should encourage families and clinicians to consider that a subcategory of diffuse lesions may exist, particularly in the neonatal period. It must be stressed, however, that nearly all patients with diffuse brainstem lesions experience a poor outcome, regardless of tumor grade or treatment. Brainstem gliomas, spontaneous regression of central nervous system tumors, and the differential diagnoses of brainstem lesions are discussed.
作者报告了两例未经治疗而消退的弥漫性脑干病变病例。两名新生儿出生时均出现颅神经麻痹和肢体无力。出生后第1周获得的磁共振(MR)图像显示,每名患者均有一个巨大的、扩展性的脑桥延髓病变。临床和影像学检查结果与弥漫性脑干胶质瘤的特征高度一致。在与两名婴儿的父母协商后,各方均同意放弃当时可用的治疗方式。两名患者均未接受手术、放射治疗或化疗;均接受了常规神经学检查和MR成像检查。数周内,病例1的患者临床症状开始改善,4岁时几乎达到了所有发育里程碑。系列MR图像显示病变大小稳步减小。病例2的患者也以类似方式改善,现10岁。这两个病例的发现应促使家庭和临床医生考虑可能存在一类特殊的弥漫性病变,尤其是在新生儿期。然而,必须强调的是,几乎所有弥漫性脑干病变患者的预后都很差,无论肿瘤分级或治疗情况如何。文中讨论了脑干胶质瘤、中枢神经系统肿瘤的自发消退以及脑干病变的鉴别诊断。