Clinical Course of Optic Nerve Sheath Meningioma

PURPOSE: To evaluate the clinical course of optic nerve sheath meningioma (ONSM) in Korean patients. METHODS: A retrospective study of 11 eyes of 11 patients with a diagnosis of ONSM between 2002 and 2015 at Severance Hospital. RESULTS: The mean age at symptom onset was 47.6 years. Ten females and 1 male participated in the study and all tumors were unilateral. Patients typically presented with visual loss and proptosis. Three patients complained of limited extraocular movements and seven patients exhibited visual field defects. Three patients who had a greater growth rate with intracranial involvement and two patients who had decreased vision received treatments. Five patients maintained good vision and visual field during the follow-up period. However, one patient who underwent surgical treatment presented significant visual loss and deterioration of visual field defect. One out of two patients who received three-dimensional conformal radiotherapy (3D-CRT) experienced improvement in visual field, and the other showed no change in visual field defect but remained stable with decreased tumor size. One out of two patients who underwent gamma-knife surgery showed aggravated visual field defect and the other presented with visual loss. CONCLUSIONS: ONSM is typically a slow-growing tumor. Deterioration of visual loss and visual field defect can occur after treatment of ONSM. Therefore, management should be considered carefully and should be limited to cases in which progression of the disease is advanced or tumor growth is fast. 3D-CRT can be considered in patients in need of treatment.

A Case of Trochlear Nerve Schwannoma Presenting with Binocular Diplopia

PURPOSE: To report a case of unilateral trochlear nerve schwannoma in a patient without neurofibromatosis. CASE SUMMARY: A 58-year-old male presented with acute onset of diplopia which developed 10 days prior. Alternate prism cover test, ductions and versions and Bielschowsky three-step test were compatible with left superior oblique muscle palsy. High-resolution magnetic resonance imaging showed a 6-mm-sized lobulated mass in the cisternal segment of the left trochlear nerve passing lateral to the brainstem. An additional thin-section gadolinium-enhanced orbit magnetic resonance imaging showed definite enhancement in the entire portion of the lobulated mass, compatible with a trochlear nerve schwannoma. Diplopia was managed conservatively with prism glasses and regular follow-up examinations were recommended without further treatment. CONCLUSIONS: A trochlear nerve tumor should be considered in adults who develop diplopia associated with acquired superior oblique muscle palsy.

An Intramuscular Neurofibroma Presenting as a Thenar Mass

PURPOSE: Neurofibromas may present as multiple or solitary lesions. Although there is no predilection site for solitary lesions, they are rare on the hand. In addition, solitary intramuscular neurofibromas are a very rare pathological type. Here, we report a rare solitary intramuscular neurofibroma in the hand. This paper examines the clinical characteristics of intramuscular neurofibroma arising from the lumbricalis in order to enable a correct diagnosis and treatment. METHODS: A 32-year-old male presented with a painless mass on the palm. The physical examination revealed a 3 x 2cm protruding mass that was non-tender to palpation. The vascular and sensory examinations were unremarkable, while the motor examination showed mild difficulty with flexion and extension. Magnetic resonance imaging demonstrated an enhancing solid mass between the thenar eminence and second metacarpophalangeal joint. The diagnosis of an intramuscular neurofibroma was confirmed following surgical excision and histological evaluation. RESULTS: The pathological examination was consistent with a neurofibroma, with delicate fascicles and loose fusiform cells in a fibrous stroma, with oval or spindle-shaped nuclei and scant cytoplasm. The background matrix was pale staining and had focal myxoid stroma. There was no significant nuclear pleomorphism and no mitoses. Immunohistochemistry with S-100 was slightly positive. At the 6-month follow-up, motor and sensory function were intact and the range of motion was full. CONCLUSION: A neurofibroma is a rare tumor of the hand, especially the intramuscular type. Hand surgeons should consider the diagnosis of this tumor based on the examination and imaging.

Clinical experience in facial nerve tumors:A review of 27 cases / 临床耳鼻咽喉头颈外科杂志

Objective:To analyze the clinical manifestations and the diagnosis of the facial nerve tumor according to the clinical information, and evaluate the different surgical approaches depending on tumor location.Method:Twenty-seven cases of facial nerve tumors with general clinical informations available from 1999.9 to 2006.12 in the Shanghai EENT Hospital were reviewed retrospectively.Result:Twenty(74.1%) schwannomas,4 (14.8%)neuofibromas ,and 3(11.1%)hemangiomas were identified with histopathology postoperatively.During the course of the disease,23 patients(85.2%)suffered facial paralysis,both hearing loss and tinnitus affected 11 (40.7%)cases,5(18.5%)manifested infra-auricular mass and the others showed some of otalgia or vertigo or ear fullness or facial numbness/twitched. CT or/and MRI results in 24 cases indicated that the tumors originated from the facial nerve.Intra-operative findings showed that 24(88.9%)cases involved no less than 2 segments of the facial nerve,of these 24 cases 87.5%(21/24)involved the mastoid protion,70.8%(17/24)involved the tympanic protion, 62.5%(15/24)involved the geniculate ganglion, only 4.2%(1/24)involved the internal acoustic canal(IAC),and 3 cases (11.1%)had only one segments involved. In all of these 27 cases ,the tumors were completely excised,of which 13 were resected followed by an immediate facial nerve reconstruction,including 11 sural nerve cable graft,1 facial nerve end-to-end anastomosis and 1 hypoglossal-facial nerve end-to-end anastomosis.Tumors were removed with preservation of facial nerve continuity in 2 cases.Conclusion:Facial nerve tumor is a rare and benign lesion,and has numerous clinical manifestations.CT and MRI can help surgeons to make a right diagnosis preoperatively.When and how to give the patients an operation depends on the patients individually.

Tumor do VIII nervo com apresentação incomum / An uncommon presentation of an VIII nerve tumor

Os Schwannomas vestibulares são responsáveis por 80 a 90 por cento dos tumores do ângulo ponto-cerebelar. A atual incidência é estimada em 0,8 por cento a 2,5 por cento da população mundial. A hipoacusia unilateral e progressiva é o sintoma mais precoce e freqüente, sendo o tinido a segunda queixa mais comum. Estudos demonstram que apenas 5 por cento dos pacientes com schwannoma vestibular têm exames audiométricos normais. No caso em foco é relatado hipoestesia da hemiface com diminuição do reflexo córneo palpebral ipsilateral, hipoestesia da porção póstero-superior do pavilhão auditivo (sinal de Hitzelberger positivo), diminuição do lacrimejamento, Romberg sensibilizado positivo. Observava-se discreto desvio da rima labial para a esquerda, não apresentando outras alterações nos demais pares cranianos. À acumetria, não havia alteração da sensibilidade auditiva em ambas as vias aéreas.

Vestibular Shwannomas are responsible for 80-90 percent of the cerebelar-pontine angle tumors and their incidence is of 0.8 to 20.5 percent of all tumors. Unilateral and progressive hearing loss is the most frequent and premature symptom, and tinnitus is the second most common complaint. Only 5 percent of the patients have normal audiograms. In this case the patient complained of ipsilateral facial numbness and weak blink, posterior pinna hypoesthesia (Hitzelberger +), tear reduction and positive Romberg test. He also had mouth twisting but no other involvement of other cranial nerves. Hearing acuity was normal.

Gastrointestinal Autonomic Nerve Tumor (GA NT) in Small Intestine

Gastrointestinal autonomic nerve tumors (GANTs) represent a newly described entity, which are stromal tumors of the gastrointestinal tract, with neuronal differentiation. They are CD117 (c-kit) immunoreactive neoplasms and are often immunoreactive for S100 protein, synaptophysin, and CD34. Ultrastructural examination provides the definitive distinguishing features for diagnosing a GANT. The presence of electron-dense granules, or small vesicles, suggests the presence of postganglionic neurotransmitter vesicles. We experienced a 65-year-old female patient who complained of bloody diarrhea, and a 75-year-old male patient who complained of melena. They were diagnosed with a gastrointestinal stromal tumor by an abdominal CT and operation, and were diagnosed as GANT pathologically. Thus we report two cases of a gastrointestinal autonomic nerve tumor, with a review of the relevant literatures.

Metastatic Gastrointestinal Autonomic Nerve Tumor in Liver

Gastrointestinal autonomic nerve tumors (GANT) are uncommon neoplasms of the gastrointestinal tract. The GANT is a specialized form of stromal neoplasm with the feature of myenteric plexus derivation. We report a patient with a GANT of the small intestine and liver metastasis. The patient was admitted for evaluation of hematochezia and a palpable abdominal mass. A CT of the abdomen showed an exophytic mass arising from the distal ileum and exhibiting deep ulceration and air-cavity formation, and a huge cystic mass in the right lobe of the liver. The gross appearance of a surgical specimen of the liver showed a huge cystic mass, containing liquified hemorrhagic fluid, with an irregular nodular peripheral portion. Its capsule was intact. An ileal mass, measuring 9x5 cm and arising from the submucosal layer, with deep ulceration was seen. Histological examination of the liver and the ileal mass revealed a malignant stromal tumor with neural differentiation on immunohistochemical staining.

2 Cases of Peripheral Nerve Tumors of Anterior Neck Simulating Thyroid Nodule / 대한내분비학회지

Peripheral nerve tumors are mostly benign and can arise on any nerve trunk or twig. Although peripheral nerve tumors can occur anywhere in the body, including the spinal roots and cauda equina, many cases are subcutaneous in location and present as a soft swelling, sometimes with a purplish discoloration of skin. There are two major catagories, schwannoma(neurilemmoma), and neurofibroma. Schwannomas are usually solitary and grow in the nerve sheath, rendering them relatively easy to dissect free. In contrast, neurofibromas tend to be multiple, grow in the endoneural substance, which renders them difficult to dissect, may undergo malignant changes, and are the hallmark of von Recklinghausens neurofibromatosis. Masses in the anterior part of neck may be initially thought to be thyroid nodule and then other cervical masses should be considered. The diagnosis rests on clinical suspicion and diagnostic support may be obtained by CT scan, magnetic resonance imaging(MRI) and substraction angiography in the literature. After imaging, fine needle aspiration for cytology may be helpful. If they are resected unrecognized and/or without regard to their nerve origin, major and permanent nerve defects can unnecessarily occur. We experienced 2 cases of peripheral nerve tumors of anterior neck simulating a thyroid nodule.