Naylor Ryan M, Wohl Anton, Raghunathan Aditya, Eckel Laurence J, Keating Gesina F, Daniels David J
1Mayo Medical School.
3Mayo Medical Scientist Training Program; and.
J Neurosurg Pediatr. 2018 Oct;22(4):397-403. doi: 10.3171/2018.4.PEDS17638. Epub 2018 Jul 6.
The aim of this study was to describe the clinical presentation, imaging appearance, and differential outcomes based on tumor location in 7 patients with desmoplastic infantile astrocytoma and desmoplastic infantile gangliogliomas (DIA/DIG).
Data of 7 patients with histopathology-proven DIA/DIGs and preoperative imaging were retrospectively reviewed, and age, sex, clinical presentation, imaging characteristics, tumor location, surgical procedure, postoperative morbidity, and overall mortality were recorded.
Two subgroups of patients with DIA/DIGs were found to exist based on whether their tumor was located in the cerebral hemispheres or suprasellar region. Nearly all patients presented with rapidly enlarging head circumference regardless of tumor location. However, ocular abnormalities, including nystagmus and preference for downward gaze, were specific for patients with suprasellar disease. These patients experienced significant postoperative complications and had poor long-term outcomes. In contrast, patients with hemispheric tumors underwent more extensive resection than patients with suprasellar tumors, had uneventful postoperative courses, and had no documented long-term comorbidities.
Postoperative course and long-term outcome for patients with DIA/DIGs were correlated to the anatomical location and radiographic appearance of their tumor at presentation, despite having histologically and molecularly indistinguishable, WHO grade I tumors.
本研究旨在描述7例促纤维增生性婴儿星形细胞瘤和促纤维增生性婴儿节细胞胶质瘤（DIA/DIG）患者的临床表现、影像学表现以及基于肿瘤位置的不同预后。
回顾性分析7例经组织病理学证实的DIA/DIG患者的数据及术前影像学资料，记录年龄、性别、临床表现、影像学特征、肿瘤位置、手术过程、术后发病率及总死亡率。
根据肿瘤位于大脑半球还是鞍上区域，发现DIA/DIG患者存在两个亚组。几乎所有患者无论肿瘤位置如何均表现为头围迅速增大。然而，包括眼球震颤和向下凝视偏好在内的眼部异常是鞍上疾病患者所特有的。这些患者术后出现严重并发症且长期预后较差。相比之下，半球肿瘤患者比鞍上肿瘤患者接受了更广泛的切除，术后病程平稳，且无长期合并症记录。
尽管DIA/DIG患者的肿瘤在组织学和分子学上难以区分且均为WHO I级肿瘤，但其术后病程和长期预后与肿瘤出现时的解剖位置及影像学表现相关。
