• Proceedings of the KOR-BRONCHOESO Conference
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• 1979.05a
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• pp.9.5-9
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• 1979

Somewhat less than 100 cases of neurogenic tumors of the larynx are reported in the literature. This is slowly growing tumor and is mostly schwannomas and neurofibromas, and could affect any age group. Since the first description of Von Recklinghausen's disease in 1882, about 20 cases of laryngeal involvement have been reported in the world literature. In this paper two cases of laryngeal neurofibroma will be presented: one congenital laryngeal neurofibroma in a 2-month-old boy, and one large laryngeal neurofibroma in Von Recklinghausen's disease in a 28 year old woman. The importance of conservative surgical procedure is discussed with. case presentation and literature review.

• Clinical and Experimental Pediatrics
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• v.49 no.2
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• pp.203-207
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• 2006

Neurofibromatosis type 1 (von Recklinghausen disease, NF1) involves the central and peripheral nervous systems as well as the skin, bone, endocrine, gastrointestinal and vascular systems. The gastrointestinal neurofibroma associated with NF1 has been infrequently reported. We report our experience with a 15-year-old boy who had a gastric plexiform neurofibroma with upper gastrointestinal bleeding and underwent a tumorectomy because of massive upper gastrointestinal bleeding. We conclude that gastrointestinal bleeding and anemia in the setting of NF1 mandates complete endoscopic examination of the digestive tract to rule out neurofibromas. Surgical resection is the standard treatment.

• Journal of Korean Neurosurgical Society
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• v.29 no.4
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• pp.550-554
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• 2000

Most extracranial schwannomas are solitary, but neurofibromas are frequently associated with other manietations of neurofibromatosis. Schwannomas that occur within the context of neurofibromatosis tend to be multiple, but multiple schwannomas without manifestation of neurofibromatosis type 2 are very rare. The authors report a very rare case of multiple spinal intradural schwannomas in the absence of neurofibromatosis Type 2 maniestations. A 40-year-old man suffered from longstanding low back pain and left side sciatica which was treated with two stage operations. MRI showed multiple intradural mass lesions extending from L1 vertebral segment to S1 vertebral segment. There were no clinical and radiological manifestations of Type 2 neurofibromatosis. Histologically confirmed diagnosis was schwannoma.

• Korean Journal of Bronchoesophagology
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• v.15 no.1
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• pp.24-27
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• 2009

Background: Benign tumors of the esophagus are rare. They include leiomyomas, gastrointestinal stromal tumors, neurofibromas and lipomas. In this study we present our experience with enucleation of these 13 tumors for 10 years. Material and Method: A retrospective review of patients who underwent enucleation of benign esophageal tumors between 1995 and 2005 was conducted. Symptom, tumor location and size, operative approach and outcomes after surgery were recorded. Result Thirteen patients were identified(leiomyoma: n=12; GIST n=l). Eight patients were men, five patients were women. Most of them were 4rd and 5th decade. The tumors arose in the lower(7 patients) and middle(6 patients) thirds of the esophagus. Eleven patients underwent a thoracotomy; the remainder were resected using VATS. All of patients underwent simple enucleation. There were no complications and recurrences after surgery. Conclusion: We present our experience with enucleation of these 12 leiomyomas and one GIST for 10 years.

• Archives of Craniofacial Surgery
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• v.21 no.6
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• pp.380-383
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• 2020

Schwannomas, which originate from Schwann cells in the peripheral nervous system, are slowg-rowing and uncommon benign tumors. Most schwannomas (90%) occur in isolation, and multiple occurrences are a characteristic feature of neurofibromas. Schwannomas of the nose and nasal tip are particularly unusual. Although a few cases of schwannomas of the sinusoidal tract and nasal septum have been reported, schwannomas arising from the nasal dorsum area and tip are extremely rare. Sensory abnormalities are also a very rare symptom. We excised a schwannoma on the nasal dorsum through direct incision and a schwannoma on the nasal tip through open rhinoplasty. No postoperative complications involving recurrence, hematoma, or infection occurred. The possibility of neurological changes should be considered in cases of an abnormality in the peripheral nervous system. Schwannoma must be kept in mind as a possible cause of neurological changes localized to a specific dermatome, and should always be considered in the differential diagnosis of a mass on the nose.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.30 no.1
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• pp.56-59
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• 2004

The neurofibroma in oral cavity is typically associated with neurofibromatosis. The solitary neurofibroma is commonly observed in skin. It is relatively rare in oral cavity and usually observed in the tongue, buccal mucosa, and vestibule. The rare types of solitary neurofibromas have been reported as a case report and they were in the inferior alveolar nerve, infratemporal fossa, maxilla, and palatal ginviva. In our hospital, the presented case was the first case as reported as solitary neurofibroma in the oral cavity. The prognosis after excision and the review of literatures were presented.

• The Journal of the Korean bone and joint tumor society
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• v.6 no.2
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• pp.77-81
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• 2000

Malignant peripheral nerve sheath tumors(MPNSTs) are uncommon sarcomas that mostly arise in the soft tissue. They can develop from the pre-existing neurofibromas or schwannomas, or denovo from the peripheral nerves, or they can occur following the radiation therapy. We report a case of MPNST that developed in the sciatic nerve of the patient with neurofibromatosis type-1(NF-1). The patient was a 39-year-old man with the history of NF-1, who's main symptom was a rapidly enlarging painful mass in his posterior thigh. The well demarcated tumor, $6.5{\times}5{\times}4.5$cm in size, was composed of closely packed spindle cells. Since the patients with NF-1 have a high risk for developing a recurred MPNST, the importance of the clinical follow up is emphasized.

• Clinical and Experimental Pediatrics
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• v.48 no.1
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• pp.93-96
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• 2005

Neurofibromatosis type I is an autosomal dominant disorder with varied manifestations in bone, soft tissue, the nervous system and skin. This is characterized by cafe-au-lait spots, neurofibromas, Lisch nodules, optic glioma, bony displasia, and intertriginous freckling. One of the more serious aspect of the disease relates to the arterial involvement. Vascular changes in neurofibromatosis may occur in any arterial tree from the proximal aorta to the small arteries but these changes are most common in the renal arteries, aorta, celiac arteries and mesenteric arteries. Of the many complications observed in neurofibromatosis type I, cerebrovascular lesions may be the least appreciated. About 40 cases of neurofibromatosis type I associated with occlusive cerebrovascular disorders have been reported in the literature, but MRI and angiographic findings typical of moyamoya disease are rarely described. We experienced a case of moyamoya disease associated with neurofibromatosis type I in a 3-year-old girl who of complained gait disturbance and paraparesis and showed findings typical of moyamoya disease on MRI and carotid angiogram.

• Journal of Yeungnam Medical Science
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• v.30 no.2
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• pp.105-108
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• 2013

Type 1 neurofibromatosis (von Recklinghausen's disease, NF-1) is an autosomal-dominant neurocutaneous-disorder characterized by systemic cafe'-au-lait spots, multiple cutaneous neurofibromas, axillary or inguinal freckling, and Lisch nodules (pigmented iris hamartomas). Approximately 10-25% of NF1 patients have gastrointestinal neoplasms. Gastrointestinal stromal tumor (GIST) in patients with neurofibromatosis is most commonly found in the small bowel and the stomach, and approximately 60% of such patients have multiple tumors or multiple tumor sites. Although, the increased incidence of GIST in patients with neurofibromatosis is well documented in pathology literature in English, but has rarely been documented in Korea. Here, we report a case of multiple GISTs in a 48-year-old woman accompanied by NF1. She was admitted to Yeung-nam University Hospital with complaints of melena and dyspnea. A contrast-enhanced computed tomography (CT) scan revealed that multiple soft tissue masses were occupying the entire peritoneal cavity. An ultrasonogram- guided biopsy was performed and the tumors were found to have been composed of tumor cells that were positive for c-kit protein. The patient was put on Imatinib mesylate treatment, and further follow-up will be carried out.

• The Journal of Pediatrics of Korean Medicine
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• v.15 no.2
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• pp.69-73
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• 2001

The neurofibromatosis (NF) are a set of genetic disorders which cause tumors to grow along various types of nerves and, in addition, can affect the development of non-nervous tissues such as bones and skin. NF causes tumors to grow anywhere on or in the body. It also leads to developmental abnormalities. For example, individuals with NF have a higher incidence of learning disabilities. Neurofibromatosis(NF) has been classified into two distinct types: NF-I and NF-II. neurofibromatosis 1(NF-I), also known as von Recklinghausen NF or Peripheral NF, occurring in 1:4,000 births, is characterized by multiple cafe-au-lait spots and neurofibromas on or under the skin. Enlargement and deformation of bones and curvature of the spine (scoliosis) may also occur. Occasionally, tumors may develop in the brain, on cranial nerves, or on the spinal cord. About 50% of people with NF also have learning disabilities. Neurofibromatosis 2(NF-II), also known as Bilateral Acoustic NF(BAN), is much rarer occurring in 1:50,000 births. NF-II is characterized by multiple tumors on the cranial and spinal nerves, and by other lesions of the brain and spinal cord. Tumors affecting both of the auditory nerves are the hallmark. Hearing loss beginning in the teens or early twenties is generally the first symptom. We reported a 10-year-old female patient with NF-I, she has pain and edema in left leg, no symptoms of NF.