OBJECTIVE: Compared with other neural structures, optic apparatus are particularly sensitive to radiation. If tumors are adjacent to or in contact with optic apparatus, a number of limitations need to be addressed for planning radiosurgery. To avoid radiation induced optic neuropathy, we treated these lesions with fractionated stereotactic radiosurgery (FSRS). This study is undertaken to assess the efficacy of FSRS for sella and parasella tumors adjacent to or in contact with optic apparatus. METHODS: We treated 19 sellar region tumors located adjacent to or in contact with optic apparatus with fractionated stereotactic radiosurgery using the Novalis system. Seventeen patients who could be followed were included in this study. They consisted of 8 pituitary adenomas, 4 optic gliomas, 3 meningiomas, 2 craniopharyngiomas. The mean tumor volume was 16.1cc(range 1-61.1). When planning FSRS, the prescribed fractionation dose to optic apparatus below 200cGy. Follow up examinations consisted of neurological, neuroradiological, and neuroopthalmological evaluations. RESULTS: Follow-up ranged from 2 to 34 months(mean 15 months). Serial magnetic resonance imaging revealed no increase in volume of tumor in all 17 patients. No patients had radiation induced optic neuropathy. CONCLUSION: Fractionated stereotactic radiosurgery is proper innovative treatment modality for sella or parasella tumors adjacent to or in contact with optic apparatus to avoid radiation induced optic neuropathy. We need further follow-up and clinical experiences.
Craniopharyngioma ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Meningioma ; Optic Nerve Diseases ; Optic Nerve Glioma ; Pituitary Neoplasms ; Radiosurgery ; Tumor Burden

Neurofibromatosis type 1(NF1) is one of the most common inherited disorders, clinically characterized by cafe-au-lait spots, Lisch nodules and neurofibromas. In addition, the affected individuals usually develop benign and malignant tumors of the nervous system. One of the most common tumors is the optic nerve glioma. NF1-associated glioma, however, rarely occurs in the cerebellum. Recently, we experienced a NF1-associated cerebellar pilocytic astrocytoma in an 11 years old girl. She has a family history of NF1 and multiple cafe-au-lait spots over her whole body. We report herewith a case of NF1-associated cerebellar pilocytic astrocytoma with a brief review of related literature.
Astrocytoma* ; Cafe-au-Lait Spots ; Cerebellum ; Child ; Female ; Glioma ; Humans ; Nervous System Neoplasms ; Neurofibroma ; Neurofibromatoses* ; Neurofibromatosis 1* ; Optic Nerve Glioma

No abstract available.
Dose-Response Relationship, Radiation ; Glaucoma, Neovascular/*etiology ; Glioma/*surgery ; Humans ; Optic Nerve Neoplasms/*surgery ; Radiosurgery/*adverse effects

Neurofibromatosis type 1 (NF1) is an autosomal dominantly inherited familial tumor syndrome. Benign tumors such as pilocytic astrocytoma, optic glioma make up the majority of intracranial neoplasms in patients with NF1. There have only been a handful of cases in which adult glioblastoma presented with NF1. A 32-year-old male presented with headache and radiological studies showing a high grade intra-axial tumor. The patient underwent gross total surgical excision and the pathology revealed glioblastoma. After the surgery, he received concomitant chemo-radiotherapy with temozolomide and adjuvant temozolomide chemotherapy. We report a NF1 patient who developed glioblastoma and reviewed related articles.
Adult ; Astrocytoma ; Brain Neoplasms ; Drug Therapy ; Glioblastoma* ; Hand ; Headache ; Humans ; Male ; Neurofibromatosis 1* ; Optic Nerve Glioma ; Pathology

Intrathird ventricular tumors that mainly occupy the ventricle cavity without extending to the neighbouring structures are rare. These tumors are developed from the choroids plexus, tela, ependyma, subjacent neuroglia and embryonic cell rest. The authors experienced 11 cases of rare intrathird ventricular tumors during the past 10 years. During the same period, number of surgically proven brain tumors were 990 and intrathird ventricular tumors comprised 1.1%. These are two cases of choroids plexus papillomas, three craniopharyngiomas, two germ cell tumors, one meningioma, one glioependymal cyst, one ependymoma, one astrocytoma. Suprasellar craniopharyngiomas, thalamic gliomas, optic and hypothalamic gliomas were excluded. The age distribution was from 6 years old to 59 years old. The clinical manifestation was characteristic in that they usually had the sudden intermittent headache without specific localizing signs. Surgical approach to this area is by transcallosal or transcortical approach. Surgical total removal was quite feasible by either approach and outcome was rather favorable.
Age Distribution ; Astrocytoma ; Brain Neoplasms ; Child ; Choroid ; Craniopharyngioma ; Ependyma ; Ependymoma ; Glioma ; Headache ; Humans ; Meningioma ; Middle Aged ; Neoplasms, Germ Cell and Embryonal ; Neuroglia ; Optic Nerve Glioma ; Papilloma ; Papilloma, Choroid Plexus

Intrathird ventricular tumors that mainly occupy the ventricle cavity without extending to the neighbouring structures are rare. These tumors are developed from the choroids plexus, tela, ependyma, subjacent neuroglia and embryonic cell rest. The authors experienced 11 cases of rare intrathird ventricular tumors during the past 10 years. During the same period, number of surgically proven brain tumors were 990 and intrathird ventricular tumors comprised 1.1%. These are two cases of choroids plexus papillomas, three craniopharyngiomas, two germ cell tumors, one meningioma, one glioependymal cyst, one ependymoma, one astrocytoma. Suprasellar craniopharyngiomas, thalamic gliomas, optic and hypothalamic gliomas were excluded. The age distribution was from 6 years old to 59 years old. The clinical manifestation was characteristic in that they usually had the sudden intermittent headache without specific localizing signs. Surgical approach to this area is by transcallosal or transcortical approach. Surgical total removal was quite feasible by either approach and outcome was rather favorable.
Age Distribution ; Astrocytoma ; Brain Neoplasms ; Child ; Choroid ; Craniopharyngioma ; Ependyma ; Ependymoma ; Glioma ; Headache ; Humans ; Meningioma ; Middle Aged ; Neoplasms, Germ Cell and Embryonal ; Neuroglia ; Optic Nerve Glioma ; Papilloma ; Papilloma, Choroid Plexus

PURPOSE: Diencephalic syndrome is an uncommon cause of failure to thrive in early childhood that is associated with central nervous system neoplasms in the hypothalamic-optic chiasmatic region. It is characterized by complex signs and symptoms related to hypothalamic dysfunction; such nonspecific clinical features may delay diagnosis of the brain tumor. In this study, we analyzed a series of cases in order to define characteristic features of diencephalic syndrome. METHODS: We performed a retrospective study of 8 patients with diencephalic syndrome (age, 5-38 months). All cases had presented to Seoul National University Children's Hospital between 1995 and 2013, with the chief complaint of poor weight gain. RESULTS: Diencephalic syndrome with central nervous system (CNS) neoplasm was identified in 8 patients. The mean age at which symptoms were noted was 18+/-10.5 months, and diagnosis after symptom onset was made at the mean age of 11+/-9.7 months. The mean z score was -3.15+/-1.14 for weight, -0.12+/-1.05 for height, 1.01+/-1.58 for head circumference, and -1.76+/-1.97 for weight-for-height. Clinical features included failure to thrive (n=8), hydrocephalus (n=5), recurrent vomiting (n=5), strabismus (n=2), developmental delay (n=2), hyperactivity (n=1), nystagmus (n=1), and diarrhea (n=1). On follow-up evaluation, 3 patients showed improvement and remained in stable remission, 2 patients were still receiving chemotherapy, and 3 patients were discharged for palliative care. CONCLUSION: Diencephalic syndrome is a rare cause of failure to thrive, and diagnosis is frequently delayed. Thus, it is important to consider the possibility of a CNS neoplasm as a cause of failure to thrive and to ensure early diagnosis.
Astrocytoma ; Brain Neoplasms ; Central Nervous System ; Central Nervous System Neoplasms ; Diagnosis ; Diarrhea ; Drug Therapy ; Early Diagnosis ; Failure to Thrive* ; Follow-Up Studies ; Head ; Humans ; Hydrocephalus ; Hypothalamic Diseases ; Infant* ; Optic Nerve Glioma ; Palliative Care ; Retrospective Studies ; Seoul ; Strabismus ; Vomiting ; Weight Gain

A 13-year-old girl with a right intraorbital optic nerve glioma (ONG) was referred to our glaucoma clinic because of uncontrolled intraocular pressure (IOP) in her right eye. The IOP reached as high as 80 mmHg. Several months earlier, she had undergone stereotactic image-guided robotic radiosurgery using the CyberKnife for her ONG; the mass had become smaller after treatment. Her visual acuity was no light perception. Slit lamp examination revealed rubeosis iridis, a swollen pale optic disc, and vitreous hemorrhage. After medical treatment, the IOP decreased to 34 mmHg, and no pain was reported. Although the mass effect of an ONG can cause neovascular glaucoma (NVG), this case shows that stereotactic radiosurgery may also cause NVG, even after reducing the mass of the tumor. Patients who undergo radiosurgery targeting the periocular area should be followed carefully for complications.
Adolescent ; Diagnosis, Differential ; Female ; Fluorescein Angiography ; Follow-Up Studies ; Fundus Oculi ; Glaucoma, Neovascular/diagnosis/*etiology/physiopathology ; Glioma/diagnosis/*surgery ; Gonioscopy ; Humans ; Intraocular Pressure ; Magnetic Resonance Imaging ; Optic Nerve Neoplasms/diagnosis/*surgery ; Radiosurgery/*adverse effects

We histopathologically analysed 221 consecutive cases of lid(97 cases ) and orbital(124 cases) tumors from 1992 through 1996 to investigate types and incidence of tumors. In cases of eyelid tumor, cystic tumors accounted for 32 cases (33.0%), basal cell carcinomas 15 cases, nevus 11 cases, sebaceous gland carcinomas 10 cases, cavernous hemangiomas 9 cases, squamous cell carcinomas 8 cases, papilloma 6 cases, pseudotumors 3 cases and lipoma 3 cases. The most common tumors were basal cell carcinoma(24.6%) in adults and cystic tumors(64.4%) in children. In cases of orbital tumor, cystic tumors were the most frequent type of lesions, responsible for 22 of the 124 cases, followed by secondary tumor 16 cases, lacrymal lesions 15 cases, pseudotumors 15 cases, lymphomas 15 cases, hemangioma 11 cases, lipoma 10 cases, peripheral nerve tumors 6 cases, optic nerve gliomas and meningiomas 5 cases, fibromas 5 cases and rhabdomyosarcomas 4 cases. The most common tumors were lacrimal tumors(17.3%) in adults and cystic tumors(50.0%) in children.
Adult ; Carcinoma, Basal Cell ; Carcinoma, Squamous Cell ; Child ; Classification* ; Eyelids* ; Fibroma ; Hemangioma ; Hemangioma, Cavernous ; Humans ; Incidence* ; Lipoma ; Lymphoma ; Meningioma ; Nevus ; Optic Nerve Glioma ; Orbit* ; Papilloma ; Peripheral Nervous System Neoplasms ; Rhabdomyosarcoma ; Sebaceous Glands

Neurofibromatosis is an autosomal dominant hereditary disorder with an overall incidence of one in 3,000~4,000, and type 1 (Von Recklinghausen's neurofibromatosis) characterized by the presence of multiple cutaneous neurofibromas, axillary and groin freckling, and cafe- au-lait spot. The neurofibromatosis type 1 gene is a tumor suppressor gene. Patients with the neurofibromatosis type 1 are at increased risk of developing nervous system neoplasm, including plexiform neurofibromas, optic gliomas, ependymomas, meningiomas, astrocytomas, and pheochromocytomas. Neurofibromas may undergo secondary malignant degeneration and sarcomatous changes. Patients with neurofibromatosis type 1 show a high incidence of Wilm's tumor, rhabdomyosarcoma, nonlymphocytic leukemia, and pheochromocytoma but the gastrointestinal involvement appears to be relatively rare and usually consists of neurofibroma, ganglioneuroma, and leiomyoma. We have identified a case of early gastric adenocarcinoma and intraabdominal schwannoma in a 65-year-old man afflicted with neurofibromatosis type 1.
Adenocarcinoma* ; Aged ; Astrocytoma ; Ependymoma ; Ganglioneuroma ; Genes, Tumor Suppressor ; Groin ; Humans ; Incidence ; Leiomyoma ; Leukemia ; Meningioma ; Nervous System Neoplasms ; Neurilemmoma* ; Neurofibroma ; Neurofibroma, Plexiform ; Neurofibromatoses* ; Neurofibromatosis 1* ; Optic Nerve Glioma ; Pheochromocytoma ; Rhabdomyosarcoma ; Wilms Tumor