Squires L A, Constantini S, Miller D C, Epstein F
Department of Pediatrics, DeVos Children's Hospital, Michigan State University, Grand Rapids 49503, USA.
Pediatr Neurosurg. 1997 Sep;27(3):153-9. doi: 10.1159/000121243.
Recent imaging and neurosurgical techniques have led to an improvement in the surgical management of children with brainstem tumors (BSTs). Many children with tumors previously considered 'inoperable' can now benefit from surgery. Increased experience has brought about new theories concerning the growth pattern, natural history, classification and optimal management of these tumors. Cervicomedullary (CM) tumors commonly have an indolent presentation reflecting either medullary or cervical spinal cord dysfunction and tend to arise in the upper cervical cord growing into the medulla in a posterior exophytic fashion. Intrinsic BSTs often present acutely with cranial nerve dysfunction and generally arise in the pons with a diffuse infiltrating growth pattern. A 21-month-old patient had developed feeding difficulty and reactive airway disease at approximately 8 months of age. MRI showed a diffuse, nonenhancing tumor in the CM region. Following radical resection, and an unremarkable perioperative course, he aspirated, developed pulmonary insufficiency and expired. Postmortem examination revealed a low-grade diffuse fibrillary astrocytoma extending from C6 to the medulla. The medullary portion arose in a paramedian location and infiltrated dorsally into the fourth ventricle, the obex, the leptomeninges, and the adjacent cerebellum. This case demonstrates the growth pattern of a distinct subset of CM tumors that behave in a manner similar to intrinsic diffuse BST. Future identification of these subsets by a careful analysis of the clinical presentation and MRI images will enable better operative planning and optimal management.
近期的影像学和神经外科技术已使脑干肿瘤（BSTs）患儿的手术治疗得到改进。许多以前被认为“无法手术”的肿瘤患儿现在能从手术中获益。经验的增加带来了关于这些肿瘤的生长模式、自然病程、分类及最佳治疗方法的新理论。颈髓肿瘤常在临床上惰性发展，反映延髓或颈段脊髓功能障碍并倾向于起源于颈髓上段，以向后方外生性生长方式长入延髓。脑干内肿瘤常急性起病，伴有脑神经功能障碍，一般起源于脑桥，呈弥漫浸润性生长模式。一名21个月大的患者在约8个月大时出现喂养困难和反应性气道疾病。MRI显示颈髓区域有一个弥漫性、无强化的肿瘤。在根治性切除术后，围手术期过程顺利，但他出现误吸、肺功能不全并死亡。尸检显示为低级别弥漫性纤维型星形细胞瘤，从C6延伸至延髓。延髓部分起源于中线旁位置，背侧浸润至第四脑室、闩部、软脑膜及相邻小脑。该病例展示了颈髓肿瘤一个独特亚组的生长模式，其表现与脑干内弥漫性肿瘤相似。未来通过仔细分析临床表现和MRI图像来识别这些亚组，将有助于更好地进行手术规划和优化治疗。
