Malignant optic nerve glioma in a young woman with 7 year follow up without recurrence.

PURPOSE: To report diagnostic and management challenges of a case of WHO Grade III glioma of the optic nerve occurring in an unusually young patient with more than 7 years of survival without recurrence. OBSERVATIONS: An 18-year-old woman reported rapidly progressive vision loss in the right eye in the setting of a right optic nerve lesion, central retinal artery occlusion, central retinal vein occlusion, and neovascularization of the optic disc. An orbital MRI with contrast demonstrated enhancement of the intraocular, intraorbital, and intracanalicular portion of the right optic nerve. Biopsy of a portion of the intraorbital optic nerve was negative, however, biopsy of the intracranial optic nerve confirmed WHO Grade III glioma (anaplastic astrocytoma). Although the tumor was excised, there remained positive margins at the optic chiasm. The patient was then managed with a combination of radiation and temozolomide. Postoperatively, the initial neovascularization of the optic nerve that had resolved, re-emerged with gliosis. In this setting a concern for intraorbital tumor arose and the globe was enucleated, definitively ruling out neoplasm. The patient has remained tumor free seven years after resection. CONCLUSIONS AND IMPORTANCE: Malignant optic pathway glioma is rare and carries a high 5-year mortality rate. Diagnosis can be elusive given orbital MRI with contrast often appears to be non-specific. Inflammatory changes can be confounding such that a biopsy in the respective area will yield a negative pathologic result. Repeat biopsy is recommended if clinical suspicion is high. Combination treatment of optic nerve tumor resection, temozolomide and radiation has been effective in treating this patient who continues to be followed closely and has had no clinical or radiographic evidence of recurrence in over 7 years. The re-emergence of neovascularization with gliosis/fibrosis of the optic nerve, was driven by ischemia and further precipitated by radiation. To our knowledge this patient represents the youngest reported case of malignant optic nerve glioma with the longest reported survival in the literature to date (over seven years).

Interesting case of subependymoma of the spinal cord.

BACKGROUND CONTEXT: Subependymomas are rare, slow-growing, and usually noninvasive/nonaggressive World Health Organization Grade I tumors that tend to occur in the ventricles. Their most common site of occurrence is the fourth ventricle followed by the lateral ventricles. Spinal cord subependymomas typically manifest as cervical and cervicothoracic intramedullary or, rarely, extramedullary mass lesions. They often present clinically with pain and neurologic symptoms, including motor, sensory, urinary, and sexual dysfunction. Histologically, there are hypocellular areas with occasional clusters of cells and frequent microcystic changes, calcifications, and hemorrhage. Radiologically, subependymomas generally manifest as eccentric well circumscribed nodular lesions with mild-to-moderate enhancement. PURPOSE: To highlight an interesting and rare presentation for subependymoma of the spinal cord. STUDY DESIGN: This is a case report of a single patient in whom a subependymoma was resected from the cervical spinal cord with return to normal functioning. METHODS: Clinical examination, magetic resonance imaging evaluation, surgical resection, and histological analysis were performed for diagnosis and treatment of this patient. RESULTS: The patient experiencing myelopathy symptoms underwent a surgical resection of cervical spinal cord subependymoma that resulted in return to normal function. CONCLUSIONS: Subependymoma should be included in the differential diagnosis of atypical presentations for myelopathy, as discrete surgical resection can result in good outcome.