• 대한두경부종양학회지
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• 제13권1호
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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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• 제49권2호
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• pp.203-207
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• 2006

위장관계의 신경섬유종의 발생빈도는 드물다고 알려져 있다. 저자들은 제1형 신경섬유종증의 15세 남자 환아에서 상부위장관 출혈로 발현한 위에 생기는 신경섬유종을 내시경 및 상부위장관 조영술을 통해 진단을 내리고, 수술적 제거를 시행하였던 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Journal of Korean Neurosurgical Society
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• 제30권sup1호
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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.

• 대한두경부종양학회지
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• 제39권2호
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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.

• Tuberculosis and Respiratory Diseases
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• 제50권1호
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• pp.122-126
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• 2001

저자들은 신경섬유종증 환자에서 우측 내유동맥에 생긴 가성동맥류의 파열로 인한 자연 혈흉을 진단하고 혈관색전술로 치료한 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제48권4호
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• pp.448-452
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• 2005

본 저자들은 전형적인 신경섬유종증 환자에서 이소성 신장을 동반한 환자를 최초로 경험하였기에 문헌고찰과 함께 보고한다.

• Archives of Plastic Surgery
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• 제33권6호
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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.

• Nuclear Medicine and Molecular Imaging
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• 제43권4호
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• pp.361-362
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• 2009

We present a patient with high $^{18}$F-fluorodeoxyglucose (FDG) uptake detected in a neurofibroma that was confused with sarcomatous transformation on a positron emission tomography/computed tomography (PET/CT) scan. A 39-year-old male patient with a 20-year history of neurofibromatosis-1 (NF-1) performed FDG PET/CT scan for the evaluation of lesions with sarcomatous transformation. The FDG PET/CT images demonstrated varying degrees of increased FDG uptake in the multiple nodules throughout whole body. The left pelvic mass with the highest FDG uptake had a maximum standardized uptake values (maxSUV) 5.0 and surgical resection was performed. Histological analysis confirmed the presence of a benign neurofibroma infiltrated with inflammatory cells.

• Tuberculosis and Respiratory Diseases
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• 제65권5호
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• pp.426-429
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• 2008

신경종은 흔한 후종격동 종양으로, 신경집종은 종격동에 발생하는 신경종의 40%를 차지한다. 신경집종은 대개 무증상으로 우연히 발견된다. 일부 환자에서 종양이 신경이나 기도를 눌러서 증상이 나타날 수 있다. 그러나, 제1형 신경섬유종증이 아닌 단일 신경집종에 의한 자발성 혈흉은 매우 드물다. 저자들은 자발성 대량 혈흉을 동반한 단일 신경집종을 경험하여 보고한다.

• 대한두경부종양학회지
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• 제9권1호
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• pp.49-55
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• 1993

Neurofibromatosis is an autosomal deminant neurocutaneous systemic disease characterized by multiple Cafe-au-lait spots. generalized cutaneous neurofibromatosis. central nervous system tumors. skeletal defects and a myriad of somatic and endocrinologic abnormalities. It occurs in about 1:3000 live births and both sexes are equally affected. From 1989 to 1992, 21 patients who admitted to our department were evaluated in clinical aspects. Then we conclude as follows: 1) The onset was usually before 10 years of age. and the incidence between the sexes was approximately the same. 2) The familial tendency was approximately 48%. 3) The head and neck were the usual sites of the disease. 4) Bone changes occurred in about 24%. 5) Most of the tumor could be removed through early diagnosis to prevent recurrence. bone changes and malignant changes. And at the same time, trough reconstructive surgery, results both functionally as well as aesthetically satisfactory could be achieved.