• Clinical and Experimental Pediatrics
• /
• 제49권2호
• /
• pp.203-207
• /
• 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.

• 대한영상의학회지
• /
• 제82권5호
• /
• pp.1321-1327
• /
• 2021

신경섬유종증 1형은 비교적 흔한 유전 질환이며 신경섬유종 형성, 피부의 색소 이상, 홍채의 리쉬결절 및 골격 이상들을 특징으로 한다. 다발성 피부 신경섬유종은 양성 신경초 종양이며 신경섬유종증 1형의 특징적인 병변이다. 신경섬유종증 1형과 관련된 심장 신경섬유종은 매우 드물며 문헌에 몇 가지 사례가 보고되었다. 이에 저자들은 신경섬유종증 1형을 가진 32세 여성에서 수술로 확진된 좌심실 신경섬유종의 컴퓨터단층촬영 및 자기공명영상 소견을 보고한다.

• 대한두경부종양학회지
• /
• 제39권2호
• /
• pp.45-48
• /
• 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
• /
• 제30권1호
• /
• pp.56-59
• /
• 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.

• 대한소아치과학회지
• /
• 제35권3호
• /
• pp.556-561
• /
• 2008

신경섬유종증은 상염색체 우성 유전성 질환으로, 17번 염색체의 장완에 위치한 종양 억제 유전자의 변성에 의해 발생한다. 이는 두가지 형이 있으며 신경섬유종의 발생은 제1형 신경섬유종증의 임상적 진단 기준 중 하나이다. 신경섬유종증의 임상적 증상으로는 피부병소, 골변형, 중추신경계의 종양 등이 있으며 환자의 25%가 구강내 신경섬유종을 보인다. 악골내 신경섬유종은 드물며 방사선학적으로 하악공, 하악관, 이공을 포함하고 단방성으로 잘 경계된 방사선 투과성으로 나타난다. 신경섬유종은 하나 또는 그 이상의 병소에서 신경육종으로 전이된다는 점에서 임상적으로 중요하며 현재 특이한 치료법은 없으나, 외과적 절제술이 좋은 치료법으로 여겨지고 있다. 본 증례는 제1형 신경섬유종증 진단을 받은 환아로, 구강내 신경섬유종의 외과적 절제 후 양호한 결과를 보여 이를 보고하는 바이다.

• Archives of Plastic Surgery
• /
• 제38권4호
• /
• pp.398-400
• /
• 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
• /
• 제21권2호
• /
• pp.403-407
• /
• 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
• /
• 제31권1호
• /
• pp.82-85
• /
• 1998

기관의 양성종양은 드물고 증상과 증후가 비슷하므로 대개 기관지 천식으로 잘못 진단된다. 원발성 신경섬유종은 슈반세포(Schwann cell)에서 기원하며 예후는 좋으나 재발할 수도 있고 악성으로 변할 수도 있기 때문에 기관 분절절제와 단단문합술이 권장되어진다. 최근 기관에 발생한 원발성 신경섬유종을 기관 분절절제와 단단문합술로 성공적으로 치험하였기에 문헌고찰과 함께 보고하는 바이다.

• Journal of Korean Neurosurgical Society
• /
• 제39권6호
• /
• pp.459-463
• /
• 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
• /
• 제1권1호
• /
• pp.199-206
• /
• 1984

저자들은 19세 남자 환자에서 정중신경에 발생한 신경섬유종을 신경학적 손상없이 제거하였다. 전자현미경을 통한 초미형태학적으로는 종양성 신경주막세포, 섬유아세포 및 교원섬유로 이 종양이 구성되었고 주된 세포는 신경주막세포로써 종양의 발생기전에 중요한 역할을 할 것으로 사료되어 문헌고찰과 함께 보고하는 바이다.