• Title/Summary/Keyword: plexiform neurofibroma

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Surgical Correction of Disfiguring Plexiform Neurofibroma Using an Anterolateral Thigh Free Flap (거대 층상 신경 섬유종 절제 후 전외측 대퇴부 유리피판술을 이용한 재건)

  • Kim, Seong-Ki;Roh, Si-Gyun;Lee, Nae-Ho;Yang, Kyung-Moo
    • Archives of Plastic Surgery
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    • v.38 no.5
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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.

Plexiform Neurilemmoma Unassociated with Neurofibromatosis - 2 Cases Report - (신경섬유종증의 동반이 없는 총상 신경초종 - 2 례 보고 -)

  • Lee, Kee-Haeng;Kim, Youn-Soo;Jeong, Chang-Hoon;Sung, Mi-Sook;Kim, Jean-A;Choi, Seung-Woog;Kim, Hyoung-Min
    • The Journal of the Korean bone and joint tumor society
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    • v.11 no.1
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    • pp.82-87
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    • 2005
  • Both neurilemmoma and neurofibroma are originated from Schwann cell. Plexiform tumor was mostly neurofibroma but Neurilemmoma which has plexiform is extremely rare. So it is important to differentiate plexiform neurilemmoma from plexiform neurofibroma, because plexiform neurilemmoma appears to have neither a significant association with neurofibromatosis nor a propensity for malignant transformation. We report two cases of plexiform neurilemmoma involving the left arm and right foot.

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Plexiform Neurofibroma Treated with Pharmacopuncture

  • Lim, Chungsan;Kwon, Kirok;Lee, Kwangho
    • Journal of Pharmacopuncture
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    • v.17 no.3
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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.

Facial hematoma induced spontaneously or by minimal trauma in a facial plexiform neurofibroma: a case report and literature review

  • Sang Min Lee;Dae Ho Leem
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.49 no.3
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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.

A Case of Plexiform Neurofibroma Arising from Laryngeal Surface of Epiglottis (후두개 후두면에 발생한 망상형 신경섬유종 1예)

  • Kim, So Yeon;Kim, Tae Hwan;Lee, Sang Hyuk;Jin, Sung Min
    • Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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    • v.26 no.2
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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.

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Gastric neurofibroma in von Recklinghausen disease : a cause of upper gastrointestinal bleeding (Von Recklinghausen disease병 환아에서 상부위장관 출혈로 발현한 위의 신경섬유종 1례)

  • Kwon, Bo Sang;Shim, Jeong Ok;Seo, Jeong Kee;Yang, Hye Ran;Ko, Jae Sung;Jung, Seong Eun;Kim, Woo Sun;Kang, Gyeong Hoon
    • 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.

A Solitary Neurofibroma of the Small Finger Associated with Trauma

  • Choi, Hwan Jun;Jung, Kyu Hwa;Nam, Doo Hyun
    • Archives of Reconstructive Microsurgery
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    • v.22 no.2
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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.

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Ancient Schwannoma of the Thigh mimicking a Plexiform Malignant Peripheral Nerve Sheath Tumor: A Case Report (총상악성말초신경초종양으로 오인한 넓적다리에 생긴 고대 신경초종: 1예 보고)

  • Lee, Yeon-Soo;Park, Sang-Eun;Lee, Jung-Uee
    • Investigative Magnetic Resonance Imaging
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    • v.15 no.2
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    • pp.170-175
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    • 2011
  • Ancient schwannoma is a rare variant of schwannoma and a slow growing benign tumor associated with degeneration that may be diagnosed as a malignant tumor, because it presents with a large size and an inhomogeneous signal intensity. The main differential diagnosis of plexiform soft tissue tumor includes plexiform neurofibroma, malignant peripheral nerve sheath tumor (MPNST). In this case, we describe the MRI findings in a case of ancient schwannoma involving left thigh of a 63-year-old woman mimicking a plexiform MPNST. The tumor appeared as an inhomogeneous signal intensity and multinodular appearance, causing misdiagnosis as a plexiform MPNST.

SOLITARY NEUROFIBROMA OF THE CHIN (이부에 발생한 신경섬유종)

  • Chang, Se-Hong;Ann, Jae-Jynn;Jeong, Min-Won
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.11 no.2
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    • pp.81-86
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    • 1989
  • Tumors originated from peripheral nerve tissues are neurofibroma, neurilemoma, plexiform neurofibroma, malignant schwannoma, and granular cell tumor. Neurofibromas seem to occur in two forms : The first one is circumscribed solitary neurofibroma and the second group is neurofibromatosis or Von Recklinghausen's disease, which is a congenital and familial disease, presenting abnormalities of the skin, nerve system, bones and soft tissue. A solitary neurofibroma is a benign, relatively circumscribed, noncapsulated tumor which often presents in the skin and subcutaneous tissue as a soft sessile or pedunculated mass. It may occur anywhere in the head and neck, but the common site of the occurrence is the tongue, buccal mucosa, palate in frequency. Since solitary neurofibroma is a relatively radioresistant and its recurrence rate seem to be low, the treatment of choice is surgical excision. The author would like to present a case of unusually large solitary neurofibroma occured in the chin, which was successfully treated with surgical excision and reconstructed using deltopectoral flap and tongue flap.

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Bilateral Occipital Neuralgia in a Patient with Neurofibromatosis Type 1: A Case Report (신경 섬유종증 1형 환자의 양측 후두 신경통: 증례보고)

  • Kim, Ji-Young;Jeon, Sungmi;Kim, Sang Wha
    • Korean Journal of Head & Neck Oncology
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    • v.37 no.2
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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.