• Journal of Chest Surgery
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• v.30 no.10
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• pp.1051-1053
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• 1997

Malignant schwannoma is rare tumor which is derived from schwas cells or nerve sheath cells, and it is frequently associated with Von Rechlinghausen's disease. We experienced one case of malignant schwannoma on the right chest wall without Von Rechlinghausen's disease. Patient was 64-year-old man who presented painless palpable mass on the right lower -chest wall for about 2 months. On chest computed tomography, the mass which was 6$\times$6 cm in size and had central necrosis, involved 11th rib with destruction, invaded the diaphragm and displaced the kidney anteriorlly. He underwent on-bloc resection of the tumor and discharged without any problem after 20 days.

• Journal of Chest Surgery
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• v.29 no.1
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• pp.120-124
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• 1996

We report three cases of posterior mediastinal tumor in Von Recklinghausen's disease on 12-yearsy old boy, 8-years-old girl and 6-years-old boy. Especially the last case was combinded with "Dumbbell" neurilemmoma of cervical spine. In the first case, preoperative CT scan showed a homogeneous large mass In the posterior mediastinum. But the mass did not show intraspinal invasion on the chest MRI scan. In the last case, cervical spine CT with thecal enhancement shows dumbbell shaped neurofibroma, compressed spinal cord and thecal sac In three cases we removed all the masses successfully and the patients were discharged safely without significant problem.t problem.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.14 no.4
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• pp.315-321
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• 1992

Tumors arising from nerve tissue are uncommon in the oral maxillofacial regions. Neurofibroma, a benign neurogenic tumor, can occur as circumscribed solitary or multiple lesions. A solitary neurofibroma is seldom undergo sarcomatous transformation, but fibromatosis is common. Therefore, it is important that we observe the oral and radiographic changes to help minimize the tremendous potential of the disease for facial disfigurement and altered function. The tumors were excised and there are no evidence of recurrence. The patients are satisfied with function and esthetic aspects.

• Journal of Chest Surgery
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• v.12 no.3
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• pp.165-169
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• 1979

Neurofibromatosis [Von Recklinghausen`s disease] is a rare Mendelian dominant disease, which shows multiple generalized symptoms and signs at various sites [Ex Skin, Bone, Nerve, Endocrine, Mediastinum rarely Lung, etc.]. We experienced one case of neurofibromatosis which has typical skin lesions [cafe-au-lait, multiple nodules, axillary freckling] with neurofibrosarcoma [malignant change from mediastinal lesion]. Patient was admitted our department because of recently developed severe dyspnea which was probably due to main tracheal compression by mediastinal neurofibrosarcoma. After successful removal of mediastinal mass dyspnea disappeared completely. Patient`s postoperative course was uneventful, and the patient was discharged 14 days after operation.

• Korean Journal of Head & Neck Oncology
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• v.13 no.1
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• pp.86-89
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• 1997

Neurofibromatosis, now termed neurofibromatosis type I, is known as a congenital and familial disease presenting abnormalities of the skin, nervous system, bones, and soft tissue. We experienced a case of extremely large neurofibromatosis which developed on the orbital and temporal region of a 24-year-old man. The tumor was widely excised including normal skin margin, outer table of cranium, a part of zygoma and maxilla. Bony defect was reconstructed by rib bone graft and secondary cosmetic correction of blepharoptosis was performed using supratarsal fixation in postoperative 6 months.

• 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.30 no.sup1
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• pp.140-143
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• 2001

We report a case of huge trigeminal schwannoma in a 10-year-old boy with neurofibromatosis type II, extending into the three spaces of the left middle, posterior, and infratemporal fossa. Initially we thought the child had a solitary trigeminal schwannoma, and most of the mass was resected successfully through one-stage operation, cranio-orbito-zygomatic intradural approach. However during the follow-up of the patient we found radiographically other multiple intracranial tumors of bilateral acoustic schwannomas, right trigeminal schwannoma, and foramen magnum tumor. Eventually the patient was diagnosed as neurofibromatosis type II presenting multiple intracranial tumors. We think childhood trigeminal schwannoma, even though in the case of solitary tumor, should be considered as possible initial manifestation of neurofibromatosis type II and that careful follow-up for the possibility of occurrence of other brain tumors such as schwannomas or meningiomas is necessary.

• Tuberculosis and Respiratory Diseases
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• v.50 no.1
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• pp.122-126
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• 2001

A non-traumatic, spontaneous hemothorax is rare. The most common causes are coagulopathy, due to anticoagulation treatment, and cancers with a metastasis to the pleural surface. Other unusual causes include thoracic endometriosis, ruptured aortic aneurysm, pulmonary arterio-venous malformation, coagulopathy, Osler-Rendeu-Weber syndrome, Ehlers-Danlos syndrome et cetera. A type 1 neurofibromatosis(Von Recklinghausen's disease) is an autosomal dominant disease that is characterized by multiple skin tumors(neurofibroma) and abnormal skin pigmentation(caf$\acute{e}$-au-lait spots). Some are accompanied by vasculopathy, and are present with a spontaneous hemothorax. Such cases are unusual but fatal. We have recently experienced a case where a young male patient with neurofibromatosis initially presented with hypovolemic shock due to a spontaneous hemothorax. Later, aortography revealed that the cause of the hemothorax was a rupture of a pseudoaneurysm of the right internal mammary artery and as a result, an embolization was performed. Here we report this case with a review of the appropriate literature.

• Clinical and Experimental Pediatrics
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• v.48 no.4
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• pp.448-452
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• 2005

Neurofibromatosis(NF) is an autosomal dominant disorder, probably of neural crest origin that affects all three germinal layers. It is a group of heterogeneous multisystemic neurocutaneous disorders involving both neuroectodermal and mesenchymal derivatives. Type 1(von Recklinghausen disease) is the most common neurocutaneous disorder among the eight subtypes. Previous reports showed various involvements in the renal organ. Renovascular hypertension is the most common major manifestation of renal involvement in this disease. However, we experienced a case of ectopic kidney concurrent with neurofibromatosis type 1. The diagnosis of neurofibromatosis had been made by typical skin manifestation on physical examination, and ectopic kidney was discovered accidentally during routine abdominal sonography. The etiological basis of this association is not clear. We report a rare case of coexisting neurofibromatosis and ectopic kidney in a 7-year-old girl with a brief review.

• Archives of Plastic Surgery
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• v.33 no.6
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• pp.761-763
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• 2006

Purpose: Malignant peripheral nerve sheath tumors most often arise from the anatomically discernible peripheral nerve or neurofibroma. Methods: A 55-year-old man had a rapidly growing pedunculated large mass on the sacrolumbar junction for 2 years. He has congenital neurofibromatosis type I. He had multiple caf-au-lait spots and multiple neurofibromas on the entire body. The mass developed from a subcutaneous nodule on the sacrolumbar junction and grew rapidly. The preoperative punch biopsy revealed a malignant peripheral nerve tumor. The mass was completely excised with 1 cm free margin above the deep fascial plane. Results: There was no evidence of recurrence of tumor for 19 months of follow-up examination. Conclusion: Malignant peripheral nerve sheath tumor is very rare and has unique feature. We report a successful case of malignant peripheral nerve sheath tumor with the review of the literatures.