• 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 the Korean Society of Radiology
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• v.82 no.5
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• pp.1321-1327
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• 2021

Neurofibromatosis type 1 (NF1) is a relatively common inherited disorder characterized by the formation of neurofibromas, pigmentary abnormalities of the skin, Lisch nodules of the iris, and skeletal abnormalities. Multiple cutaneous neurofibromas are benign nerve sheath tumors and the main manifestation of NF1. Cardiac neurofibroma associated with NF1 is very rare, and few cases have been reported in the literature. Herein, we present the CT and MRI findings of a surgically confirmed left ventricular neurofibroma in a 32-year-old female with NF1.

• Korean Journal of Head & Neck Oncology
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• v.39 no.2
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• pp.45-48
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• 2023

Malignant peripheral nerve sheath tumor (MPNST) is one of the soft tissue sarcoma believed to originate from neural crest cells. The patients with neurofibromatosis type I (NF1) have about 8-13% of the lifetime risk of the malignant transformation. Neurofibroma on patients with NF1 can be surgically resected and has good prognosis if approach to the tumor is possible. We experienced a case of a 50-year-old woman with NF who had incompletely resected neurofibroma, which presented a rapid malignant transformation to MPNST, 3 months after the first surgery. We reported this case with a brief review of literature.

• 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.

• Journal of the korean academy of Pediatric Dentistry
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• v.35 no.3
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• pp.556-561
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• 2008

Neurofibromatosis is an autosomal dominant disorder caused by a mutation of a tumor supressor gene on the long arm of chromosome 17. There are two types of neurofibromatosis, and development of neurofibroma is one of clinical diagnostic criteria for neurofibromatosis. The clinical signs of neurofibromatosis include as skin lesions, bone deformities, and tumors involving central nervous system. About 25% of neurofibromatosis involves oral neurofibroma. Radiographically, oral neurofibroma is well-defined unilocular radiolucency, which involves mandibular canal, mandibular foramen and mental foramen. When a lesion is small and approachable, complete resection, including 1cm of marginal connective tissue, is feasible. However, there are studies reporting that the recurrence rate after surgical resection is high and frequent recurrence may even increase the risk of malignant transformation. This case reports a patient with neurofibromatosis type I, accompanying oral neurofibroma, who shows a favorable result after surgical resection of the oral lesion.

• Archives of Plastic Surgery
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• v.38 no.4
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• pp.398-400
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• 2011

Purpose: Subungual tumors are a common cause of nail plate deformity, and may be caused by fibrokeratoma, Koene's tumor and glomus tumors. Neurofibromas, either as part of neurofibromatosis or as a solitary tumor are exceptionally rare in the digits. Methods: A 44-year-old man presented with painless onychodystrophy and nail plate elevation of the right thumb due to a small subungual mass that had started growing 3 years ago. Sensory evaluation of the distal phalanx was normal, and no discoloration nor infection signs were seen. The nail plate was extracted under local anesthesia, and the mass was delicately removed without injury to the nail bed. The nail matrix was repaired with primary closure. Results: Histopathology shows a well circumscribed, cellular tumor with myxoid stroma. Tumor cells were S-100 protein positive, and the patient was diagnosed with myxoid neurofibroma. There has been no sign of recurrence to date, 14 months after the operation. Conclusion: Presentation of cutaneous neurofibromas in the digits is an uncommon finding. They may occur as a manifestation of neurofibromatosis or as a solitary tumor. Subungual neurofibromas are exceptionally rare. To our knowledge, there are only ten reports of solitary subungual neurofibroma unrelated to neurofibromatosis to date. We report a rare case of solitary subungual myxoid neurofibroma of the thumb, that was treated through total excision, with preservation of the nail matrix.

• Journal of Chest Surgery
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• v.21 no.2
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• pp.403-407
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• 1988

Primary tumors of the diaphragm are very rare, From a clinical point of view, they do not present a specific symptomatology and the radiologic aspect is not characteristic. Their etiology is often obscure. The authors experienced one case of primary neurofibroma of the diaphragm. The patient was 52 year old male, and detected preoperatively abnormal round mass shadow in the middome portion of the left diaphragm in routine chest X-ray. A left posterolateral thoracotomy through the 7th ICS was performed. The mass and surrounding tissues were completely removed, and diagnosed as a primary neurofibroma by the histopathologic findings. Postoperative course was uneventful.

• Journal of Chest Surgery
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• v.31 no.1
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• pp.82-85
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• 1998

Benign tumors of the trachea are rare and are usually misdiagnosed as bronchial asthma because of the similarity of the symptoms and signs. Although the prognosis of neurofibroma which originats from Schwann cells is good, it may recur or undergo malignant change, so segmental resection of the trachea is recommended. Recently, we experienced a case of primary neurofibroma of the trachea treated successfully by segmental resection of the trachea and end-to-end anastomosis. We report it with a brief review of literatures.

• Journal of Korean Neurosurgical Society
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• v.39 no.6
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• pp.459-463
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• 2006

This report describes a case of diffuse neurofibroma[DNF] that presented with spontaneous intratumoral hemorrhage. A 31-year-old man with cutaneous manifestations typical of neurofibromatosis type 1[NF1] was referred to hospital with a progressively expanding scalp swelling. Magnetic resonance imaging[MRI] showed an acute hematoma surrounded by a dense mass on the parietooccipital scalp. Plain skull radiographs and a computed tomography[CT] scan revealed bony defects in the occipital bone around the lambdoid suture and the skull base, including the sphenoid and petrous bones. The tumor extended to the upper cervical region and infiltrated through the fascia of the musculature. The tumor and adjacent soft tissue were highly vascular, which made surgical resection difficult. We describe the clinical, radiological and pathological features of this patient md discuss the surgical methods used to avoid a life-threatening hemorrhage during surgery.

• Journal of Yeungnam Medical Science
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• v.1 no.1
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• pp.199-206
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• 1984

With respect to the histogenesis of the neurofibroma, there are many controversies, since von Recklinghausen postulated that neurofibromas are mainly fibrous tumors arising from the connective tissue of the nerve sheaths. According to the recent studies through the electron microscope, the neoplastic perineurial cells are regarded as the main components of the tumor. So, the neurofibroma enucleated from the median nerve at the level of proximal arm without resulting neurologic sequale was examined through the light- and electron microscope. Ultrastructurally, this tumor is composed of the perineurial cells, fibroblasts, and collagen fibers. The predominant perineurial cells show distinct basal lamina, and contain abundant microfilaments.