Wilson J T, Wald S L, Aitken P A, Mastromateo J, Vieco P T
Department of Neurosurgery, University of Vermont, Burlington, 05401, USA.
Pediatr Neurosurg. 1995;23(1):1-5; discussion 6. doi: 10.1159/000120927.
Primary germ cell tumors confined to the optic nerves and chiasm without suprasellar extension are uncommon. These tumors appear similar to chiasmatic gliomas on both computed tomography and magnetic resonance imaging, potentially resulting in treatment errors if the diagnosis is based on radiologic criteria alone. Unlike chiasmatic gliomas, suprasellar germinomas characteristically present with a clinical triad of endocrine abnormalities, diabetes insipidus, and visual complaints. We report the case of a 9-year-old boy who presented with a 5-month history of fatigue, 16-pound weight gain, polydipsia, polyuria, visual complaints, and intermittent headache. Imaging studies demonstrated findings consistent with a glioma of the chiasm with infiltration into the optic tracts. At surgery, the chiasm and optic tracts were diffusely enlarged with no other suprasellar abnormalities. Biopsy specimens were characteristic of germ cell tumor. Based on this result, the patient received a treatment regimen different from that used at our institution for chiasmatic gliomas. We feel it is imperative to biopsy chiasmatic lesions that radiologically appear to be gliomas if symptoms do not adhere to the classical clinical presentation.
局限于视神经和视交叉且无鞍上扩展的原发性生殖细胞肿瘤并不常见。这些肿瘤在计算机断层扫描和磁共振成像上与视交叉胶质瘤相似，如果仅根据放射学标准进行诊断，可能会导致治疗失误。与视交叉胶质瘤不同，鞍上生殖细胞瘤的典型表现为内分泌异常、尿崩症和视觉症状三联征。我们报告了一名9岁男孩的病例，他有5个月的疲劳史、体重增加16磅、多饮、多尿、视觉症状和间歇性头痛。影像学检查结果与视交叉胶质瘤伴视神经束浸润一致。手术中，视交叉和视神经束弥漫性增大，无其他鞍上异常。活检标本具有生殖细胞肿瘤的特征。基于这一结果，患者接受了与我们机构用于视交叉胶质瘤的治疗方案不同的治疗方案。我们认为，如果症状不符合典型临床表现，对于放射学上看似胶质瘤的视交叉病变进行活检是至关重要的。
