Robertson P L, Muraszko K M, Brunberg J A, Axtell R A, Dauser R C, Turrisi A T
Department of Pediatrics, University of Michigan Medical Center, Ann Arbor 48109-0570, USA.
Pediatr Neurosurg. 1995;22(2):65-73. doi: 10.1159/000120879.
The presentation, radiographic findings and course of 17 children with MRI-documented intrinsic midbrain lesions are reviewed. The anatomic centers of all the lesions were tectal, peritectal, or tegmental. Lesions centered at the pineal gland were excluded. Signs of increased intracranial pressure from hydrocephalus requiring shunt placement were present in 14 patients. Histopathological diagnosis was confirmed in three tumors; these were low grade astrocytomas and all received focal irradiation, as did one unbiopsied tumor. The remaining 13 patients with no histopathological diagnosis received no therapy other than shunt placement in 11. All but one of the lesions have remained clinically and radiographically stable, with a 4-year progression-free and total survival of 94 and 100%, respectively. We conclude that mass lesions originating in the upper midbrain are a subset of intrinsic brainstem tumors with a relatively benign course, usually presenting with hydrocephalus after infancy. They may remain stable for considerable periods and may require no further therapy after treatment of hydrocephalus. Surgical biopsy and/or resection can usually be reserved for progressive or atypical lesions which may also require further adjuvant therapy.
对17例经MRI证实存在中脑实质病变的儿童的临床表现、影像学检查结果及病程进行了回顾。所有病变的解剖中心均位于顶盖、顶盖周围或被盖区。以松果体为中心的病变被排除。14例患者因脑积水导致颅内压升高，需要进行分流手术。3例肿瘤经组织病理学确诊；这些均为低级别星形细胞瘤，所有患者均接受了局部放疗，1例未活检的肿瘤也接受了放疗。其余13例未进行组织病理学诊断的患者，除11例进行了分流手术外，未接受其他治疗。除1例病变外，所有病变在临床和影像学上均保持稳定，4年无进展生存率和总生存率分别为94%和100%。我们得出结论，起源于中脑上部的肿块病变是脑干实质肿瘤中的一个亚组，病程相对良性，通常在婴儿期后出现脑积水。它们可能在相当长的一段时间内保持稳定，在治疗脑积水后可能无需进一步治疗。手术活检和/或切除通常可保留用于进行性或非典型病变，这些病变可能还需要进一步的辅助治疗。
