Orbital apex syndrome (OAS) has been described as malfunction of cranial nerve II in the optic canal and cranial nerves III, IV, V1, and VI in the superior orbital fissure due to vascular compromise, compression, or infiltration. Symptoms include ophthalmoplegia, ptosis, decreased corneal sensation, and vision loss. There are many potential causes of OAS including inflammation, infection, iatrogenic, neoplasm, vascular disease, or trauma. Among these, however, fungal sinusitis is one of the more rarely reported etiologies. Infections of the sphenoid sinuses typically occur in conjunction with other paranasal sinuses, while isolated sphenoid sinusitis is uncommon. In this case report, we describe OAS due to isolated sphenoid fungal sinusitis in a 67-year old man who presented with periorbital pain, acute vision loss, and ophthalmoplegia. He was treated surgically and was given postoperative antifungal agents as well as steroids for three months. We present this rare case along with a literature review of OAS.
Acute Pain ; Antifungal Agents ; Cranial Nerves ; Inflammation ; Ophthalmoplegia ; Optic Nerve ; Orbit* ; Paranasal Sinuses ; Sensation ; Sinusitis* ; Sphenoid Sinus ; Sphenoid Sinusitis ; Steroids ; Vascular Neoplasms

Orbital apex syndrome (OAS) has been described as malfunction of cranial nerve II in the optic canal and cranial nerves III, IV, V1, and VI in the superior orbital fissure due to vascular compromise, compression, or infiltration. Symptoms include ophthalmoplegia, ptosis, decreased corneal sensation, and vision loss. There are many potential causes of OAS including inflammation, infection, iatrogenic, neoplasm, vascular disease, or trauma. Among these, however, fungal sinusitis is one of the more rarely reported etiologies. Infections of the sphenoid sinuses typically occur in conjunction with other paranasal sinuses, while isolated sphenoid sinusitis is uncommon. In this case report, we describe OAS due to isolated sphenoid fungal sinusitis in a 67-year old man who presented with periorbital pain, acute vision loss, and ophthalmoplegia. He was treated surgically and was given postoperative antifungal agents as well as steroids for three months. We present this rare case along with a literature review of OAS.
Acute Pain ; Antifungal Agents ; Cranial Nerves ; Inflammation ; Ophthalmoplegia ; Optic Nerve ; Orbit* ; Paranasal Sinuses ; Sensation ; Sinusitis* ; Sphenoid Sinus ; Sphenoid Sinusitis ; Steroids ; Vascular Neoplasms

We describe a rare case of malignant transformation in a vestibular schwannoma in a 33-yr-old woman. She presented herself with headache, tinnitus, and hearing loss and underwent posterior fossa explorations three times during the short period of 3 months. The clinicopathological features of the original tumor were typical of benign vestibular schwannoma. Despite a comlpete microsurgical excision, two months later, the tumor recurred locally with a rapid increase in size causing a progressive worsening of neurological symptoms. A diagnosis of malignant schwannoma was made for the recurrent tumor on the basis of the microscopic findings of high cellularity, moderate pleomorphism, and the presence of mitotic cells. Repeat magnetic resonance imaging performed a month after the second surgery unexpectedly showed definite tumor enlargement. She remained clinically stable following the third debulking of the tumor and adjuvant radiotherapy. We propose that this recurrent tumor represent malignant transformation from a benign vestibular schwannoma which was an unusual occurrence in a patient without neurofibromatosis.
Adult ; Case Report ; Cell Transformation, Neoplastic ; Cranial Nerve Neoplasms/*pathology ; Female ; Human ; Magnetic Resonance Imaging ; Neoplasm Recurrence, Local ; Nerve Sheath Tumors/*pathology ; Neuroma, Acoustic/*pathology

Adult ; Cranial Nerve Neoplasms ; Female ; Humans ; Hypoglossal Nerve ; Paraganglioma

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

From 1957 to Jun 1987, authors experienced 8 cases of pediatric brain tumor located in the cerebellopontine angle(CPA) which were operated upon at the Department of Neurosurgery of Seoul National University Hospital. The proportion of the pediatric CPA tumors in the total pediatric brain tumors was 4% (8/197), and that in the pediatric posterior fossa tumors was 8% (8/100). The pathologic diagnoses of the 8 cases were ependymoma (2 cases), medulloblastoma (2 cases), astrocytoma ( 1 case), primitive neuroectodermal tumor (1 case), trigeminal schwannoma (1 case) and Masson's hemangioendothelioma (1 case). The proportion of CPA location in the posterior fossa ependymomas was 13% (2/16), and those of the posterior fossa medulloblastomas and astrocytomas were 4% (2/50 and 1/28, respectively). The initial presentations were symptoms of increased intracranial pressure in 5 cases, and those of cranial nerve dysfunction in 3 cases. On admission, all of 8 cases showed signs of increased intracranial pressure and among the cranial nerve dysfunctions, facial nerve dysfunction was most common (5 cases). It seemed that it was due to close relationship between these tumors and fourth ventricle, and the patients' age which was too young to complain of subtle subjective sensory dysfunctions. The operative findings showed medial displacement and compression of the cerebellum, brain stem and cranial nerves, enlargement of surrounding subarachnoid space or arachnoid cyst formation, tumor growth into the cranial nerve canals, and encasement of nerves or vessels by the tumor. The sites of origin were lateral recess of fourth ventricle in 4 cases (2 ependymomas and 2 medulloblastomas), and surrounding brain tissue in the other 2 glioma cases. And a representative case was presented.
Arachnoid ; Astrocytoma ; Brain ; Brain Neoplasms ; Brain Stem ; Cerebellopontine Angle* ; Cerebellum ; Cranial Nerves ; Diagnosis ; Ependymoma ; Facial Nerve ; Fourth Ventricle ; Glioma ; Hemangioendothelioma ; Infratentorial Neoplasms ; Intracranial Pressure ; Medulloblastoma ; Neurilemmoma ; Neuroectodermal Tumors, Primitive ; Neuroma, Acoustic* ; Neurosurgery ; Seoul ; Subarachnoid Space

Adult ; Cranial Nerve Neoplasms ; diagnosis ; Facial Nerve ; pathology ; Female ; Glomus Jugulare Tumor ; diagnosis ; Humans ; Neurilemmoma ; diagnosis

Adolescent ; Cranial Nerve Neoplasms ; Facial Nerve ; Humans ; Male ; Neurilemmoma ; Parotid Region ; pathology

Adult ; Cranial Nerve Neoplasms ; diagnosis ; Diagnostic Errors ; Facial Nerve ; pathology ; Hemangioma ; diagnosis ; Humans ; Male