We describe herein a malignant glioma arising at the site of the resected hemangioblastoma after irradiation in a patient with von Hippel-Lindau disease (VHL). The patient was a 25 year-old male with multiple hemangioblastomas at the cerebellum and spinal cord, multiple pancreatic cysts and a renal cell carcinoma; he was diagnosed as having VHL disease. The largest hemangioblastoma at the right cerebellar hemisphere was completely removed, and he received high-dose irradiation postoperatively. The tumor recurred at the same site 7 years later, which was a malignant glioma with no evidence of hemangioblastoma. The malignant glioma showed molecular genetic profiles of radiation-induced tumors because of its diffuse p53 immunostaining and the loss of p16 immunoreactivity. The genetic study to find the loss of heterozygosity (LOH) of VHL gene revealed that only the cerebellar hemangioblastoma showed allelic losses for the gene. To the best of our knowledge, this report is the first to show a malignant glioma that developed in a patient with VHL disease after radiation therapy at the site of an excised hemangioblastoma. This report also suggests that radiation therapy should be performed very carefully in VHL patients with hemangioblastomas.
Adult ; Cerebellar Neoplasms/complications/*surgery ; Glioma/*diagnosis/*etiology ; Hemangioblastoma/complications/*surgery ; Humans ; Male ; Radiotherapy/*adverse effects ; von Hippel-Lindau Disease/complications/*radiotherapy

We report a case of acute comitant esotropia in a child with a cerebellar tumor. A 3-year-old boy was referred for management of a 9 month history of acute acquired comitant esotropia. On first presentation, the patient's angle of esodeviation was 50 prism-diopters (PD) at distance and near fixation without any lateral incomitance. The cycloplegic refraction revealed +0.75 diopters in both eyes. Very mild bilateral papilledema was found on the fundus examination, but the neurological examination did not reveal any other pathological findings. Brain MRI showed a 5 cm mass located in the midline of the cerebellum as well as hydrocephalus. The mass was completely excised and histological examination confirmed the diagnosis of pilocytic astrocytoma. Despite neurosurgery, the patient's strabismus remained unresolved. One year after neurosurgery, both medial rectus muscles were surgically recessed by 6 mm, resulting in esotropia of 8PD at distant and near fixation without restoration of bifoveal fusion at follow-up 2 years after the eye muscle surgery. Therefore, acute onset comitant esotropia in a child can be the first sign of a cerebellar tumor, even without any other neurological signs and symptoms.
Acute Disease ; Astrocytoma/*complications/surgery ; Brain/pathology ; Cerebellar Neoplasms/*complications/diagnosis/surgery ; Child, Preschool ; Esotropia/*etiology/pathology/physiopathology/surgery ; Humans ; Magnetic Resonance Imaging ; Male ; Neurosurgical Procedures ; Oculomotor Muscles/surgery ; Refraction, Ocular ; Time Factors