• Archives of Plastic Surgery
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• 제38권5호
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• pp.679-682
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• 2011

Purpose: Neurofibromas of neuroectodermal origin are commonly found in Von Recklinghausens disease or neurofibormatosis type 1. It is an autosomal dominant disease caused by mutation of the long arm of chromosome 17. It can present from small nodules to disfiguring giant tumor. Plexiform neurofibroma is benign in most cases, but it could be transformed into malignant tumor, which requires surgical excision. To cover the defects after the excision, a number of surgical correction methods are available. This study is to report a surgical correction of disfiguring plexiform neurofibroma using anterolateral thigh free flap for extensive defects after surgical excision of neurofibrona. Methods: Data of five neurofibroma patients with an average age of 39 including medical history, physical examination, computed tomography, and magnetic resonance imaging were checked. No disease other than neurofibroma were detected. Biopsy on the excised tissues was performed. The follow-up period was 7 to 27 months. Results: The average size of defects after complete excision of neurofibroma was $13{\times}10{\sim}25{\times}15$ cm. Defects were covered by anterolateral thigh free flap, while donor sites were covered by local flap, split thickness skin graft and regional flap. Throughout follow-up, there were no complication, relapse, or any abnormalities. Conclusion: Despite various surgical correction methods are applicable to defects after excision on disfiguring plexiform neurofibroma, coverage of massive defects is still challenging in plastic and reconstructive surgeon. We have made five successful cases of surgical correction of disfiguring plexiform neurofibroma using anterolateral thigh free flap.

• 대한골관절종양학회지
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• 제11권1호
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• pp.82-87
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• 2005

신경초 세포에서 기원하는 양성 종양에는 신경초종과 신경섬유종이 있다. 이 중 총상의 형태를 가지는 경우는 대부분 신경섬유종이며, 신경초종의 경우는 매우 드물다. 총상 신경초종은 신경섬유종증을 동반하지 않고, 악성화하지 않는다는 점에서 반드시 신경섬유종과 감별이 필요하다. 저자들은 각 각 좌측 상완부와 우측 족부에 발생한 총상 신경초종 2례를 치험하였기에 보고하는 바이다.

• 대한약침학회지
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• 제17권3호
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• pp.74-77
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• 2014

Objectives: The purpose of this study is to report a case of a plexiform neurofibroma (PNF) in the pelvic region treated with sweet bee venom (SBV) and mountain ginseng pharmacopuncture (MGP). Methods: A 16-year-old girl was diagnosed as having PNFs, neurofibromatosis type 1, 10 years ago and she had surgery three times to remove the benign tumors, but the growth of the PNFs continued. She has been treated in our clinic with SBV and MGP two times per month from March 2010 to April 2014. SBV was injected intra-subcutaneously at the borders of the PNFs in the pelvic region, and MGP was administrated intravenously each treatment time. Results: The growths of the PNFs occurred rapidly and continued steadily before treatment. Since March 2010, she has been treated in our clinic, and the growths of the PNFs have almost stopped; further-more, the discomfort of hip joint pain has been reduced, and her general condition has improved. Conclusion: We cautiously conclude that SBV and MGP treatment has some effects that suppress the growth and the spread of the PNFs in this patient.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제49권3호
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• pp.152-156
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• 2023

Plexiform neurofibroma is a rare benign tumor and a special subtype of neurofibromatosis 1. This report is a literature review with a case of patient with facial hemorrhage observed at the site of neurofibroma removal in the right lower face due to minor trauma. Through PubMed search, using terms ((facial hematoma) OR (facial bleeding)) AND (neurofibromatosis), 86 articles were identified, and five related articles (six patients) were finally selected. Of the six patients, two had previously undergone embolization. However, as a result, all patients received open surgery to remove hematomas. The hemostatic methods mentioned were vascular ligation (five patients), hypotensive anesthesia (two patients), and postoperative blood transfusion (four patients). In conclusion, spontaneous or minimally traumatic bleeding is possible in neurofibromatosis patients. In most cases, it can be resolved by vascular ligation under hypotensive anesthesia. Optionally, prior embolization and supplementary tissue adhesive may be used.

• 대한후두음성언어의학회지
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• 제26권2호
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• pp.137-140
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• 2015

Neurofibroma is characterized as a benign, slow growing neoplasm, originating from Schwann cells or fibroblast in peripheral nerve sheaths. It may appear as a solitary tumor or have multiple localizations in von Recklinghausen disease. They are commonly found in the gastrointestinal tract and laryngeal neurofibromas are extremely rare, accounting for only 0.03 to 0.1% of benign tumors of the larynx. The aryepiglottic fold and arytenoid are the common site of occurrence for laryngeal neurofibroma, because the branch of the superior laryngeal nerve is involved. We present a case of solitary plexiform neurofibroma arising from the laryngeal surface of epiglottis in a 55-year old female who found the lesion incidentally. We removed the tumor completely by transoral laser surgery and no recurrence was found after 7 months. The case of solitary neurofibroma arising from laryngeal surface of epiglottis has not been reported in Korea. We report this case regarding the diagnosis and treatment with review of literatures.

• 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.

• Archives of Reconstructive Microsurgery
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• 제22권2호
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• pp.78-81
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• 2013

Neurofibroma may present as a solitary lesion or as multiple lesions. Although there is no site of predilection for solitary lesions, occurrence on the hand is rare. Plexiform neurofibroma can develop in isolation or more commonly as a part of neurofibromatosis type 1. In those that apper in isolation, trauma has been suggested as a precipitating factor. A 68-year-old male farmer had experienced repetitive prior episodes of trauma in the involved finger. He presented with a painless mass on the dorsal aspect of the fifth finger. Physical examination showed a protruding mass measuring approximately $15{\times}20mm$ which was not tenderness to palpation and any skin changes or pigmentation. Ultrasonography showed a cystic mass on the dorsal aspect of the middle phalanx. Microsurgical dissection was applied in order to seperated the lesion from the ulnar side of the dorsal branch of the digital nerve. Pathologic examination of the specimens revealed neurofibroma. At three-month follow-up, motor and sensory function were intact, and range of motion was fully recovered. Traumatic solitary neurofibroma is a rare tumor of the hand, especially in the finger. Hand surgeons should be aware of the diagnostic possibilities of this tumor based on examination, history taking and imaging studies.

• Investigative Magnetic Resonance Imaging
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• 제15권2호
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• pp.170-175
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• 2011

고대신경초종(ancient schwannoma)은 신경초종의 변이(variant)로서 천천히 자라나고 퇴행성 변화를 동반한 드문 양성종양으로 알려져 있으며 자기공명영상상 큰 크기와 불균질한 신호강도 때문에 악성 종양으로 오인될 수 있다. 연부조직 종양이 얼기모양(plexiform)을 보일때, 총상신경섬유종, 또는 악성말초신경초종양 등을 감별해야 한다. 이에 저자들은 63세 여자 환자의 왼쪽 넓적다리에 발생한 고대신경초종 1예를 경험하였기에 자기공명영상 소견을 보고하고자 한다. 자기공명영상에서 불균질한 신호강도와 다결절 소견을 보여 총상(plexiform) 악성 말초신경초종양으로 오인하였다.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제11권2호
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• pp.81-86
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• 1989

말초 신경 조직에서 발생되는 종양은 신경섬유종, 신경초종, 파립세포종, 악성신경초종 등이 있다. 신경섬유종은 2가지 형태로 분류될 수 있다. 첫째 형태는 단독형 신경섬유종이고, 둘째는 신경섬유종 혹은 Von Recklinghausen's병으로 불리우는데 이는 주로 피부, 신경조직, 뼈, 연조직에 기형을 유발할 수 있고, 선천적이며, 가족력을 나타낸다. 단독형 신경섬유종은 양성이며, 경계는 명확하나 capsule에 의해 싸여 있지 않으며, 피부나 피하조직에 sessile 혹은 pedunculated 형태로 나타난다. 두경부의 어느 부위에나 나타날 수 있으며, 구강내의 호발 부위는 혀, 협점막, 구개 순이다. 단독형 신경섬유종은 방사선에 의한 치료에 효과가 적으며, 재발율이 낮고, 치료는 주로 외과적 절제술로 제거를 하고, 결손부를 삼각흉피판(D-P flap)과 설피판(tongue flap)으로 수복하여 현재까지 합병증 없이 양호한 결과를 얻었다.

• 대한두경부종양학회지
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• 제37권2호
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• pp.77-80
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• 2021

Plexiform neurofibromas (PNFs) represent an uncommon variant (30%) of neurofibromatosis type 1 (NF-1), in which neurofibromas arise from multiple nerves as bulging and deforming masses involving connective tissue and skin folds. We report the case of a 17-year-old man with known NF-1 presenting with bilateral occipital neuralgia that began in his late adolescence. His chief complaint was radiating pain in the occiput induced by protective helmet wear when riding alpine skiing. Craniofacial magnetic resonance imaging (MRI) confirmed the presence of fusiform masses arising from the bilateral greater occipital nerves. Histopathological examination of the biopsy samples showed PNFs. After surgical treatment, the patient's symptoms completely improved. Unlike cutaneous neurofibromas, PNFs have different clinical characteristics and have the risk of malignant mutations. Correct diagnosis and adequate surgical treatment are necessary for PNFs.