• Title/Summary/Keyword: Neurofibromas

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The Comparison of the Benign Tumors Originating from the Peripheral Nerves (말초 신경에 발생한 양성 종양의 비교)

  • Kim, Joon-Buhm;Cha, Jin-Han;Kim, Sang-Yoon
    • Archives of Reconstructive Microsurgery
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    • v.1 no.1
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    • pp.39-44
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    • 1992
  • Benign peripheral nerve tumors, although infrequent, must be considered as a possible cause of pain and disability in the extremities. There are three varieties of these tumors that are of clinical importance: neurilemmomas, neurofibromas, and post-traumatic neuroma. Neurilemmomas are the most common primary solitary tumor of the peripheral nerve trunks, and are almost always benign, Neurofibromas may occur as a solitary nerve tumor, but can present as multiple lesions as in von Recklinghausen's disease. Clinically, this tumor may presents as a solitary mass in the subcutaneous tissue which is centrally located with the nerve fibers travelling through the tumor mass. Traumatic neuroma is the proliferation of nerve elements with connective tissue during the process of regeneration from severed nerves undergoing Wallerian degeration, and is therefore not a true neoplasm. A neuroma-in-countinuity is the result of partial severance of a nerve, or of a crushing or traction injury in which all or part of the epineurium and perineurium is intact. We experienced each of the three varieties. With magnification, the neurilemmoma was removed by meticulous dissection from the parent nerve preserving the normal fascicles to which it was attached. The neurofibroma was excised and the nerve was reconstructed with interposed vein graft and the neuroma-in-continuity was excised and reconstructed with sural nerve graft. We report histologic characteristics of each tumors and the methods to repair the nerve defects after tumor excision with brief discussion.

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Early Gastric Cancer with Neurofibroma Mimicking a Metastatic Node: A Case Report

  • Kim, Sungsoo;Kim, Yoo Seok;Kim, Ji Hoon;Min, Yong Don;Hong, Ran
    • Journal of Gastric Cancer
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    • v.13 no.3
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    • pp.185-187
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    • 2013
  • Neurofibromas are benign tumors that originate from the peripheral nerves, including neurites and fibroblasts. Generally, a solitary neurofibroma is located in the skin and rarely in other places. A 72-year-old female suffered from epigastric discomfort for 2 months. Endoscopic findings showed an early gastric cancer type IIc at the antrum. Abdominal computed tomography revealed early gastric cancer with a 1.6 cm-sized metastatic node posterior to the duodenum. Laparoscopic assisted distal gastrectomy and retro-pancreatic dissection were performed uneventfully. Histological examination revealed gastric adenocarcinoma, invading the mucosa without nodal metastasis, and a neurofibroma. Herein, we present a case of a gastric cancer patient with a solitary retroperitoneal neurofibroma which mimicked a distant metastatic node.

A Case of Gliosarcoma Associated with Neurofibromatosis Type 1 (제 1 형 신경섬유종증과 동반된 교육종)

  • Kim, Dae Won;Park, Jong Tae;Kim, Jong Moon;Kim, Tae Young
    • Journal of Korean Neurosurgical Society
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    • v.30 no.2
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    • pp.221-226
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    • 2001
  • A rare case of gliosarcoma with neurofibromatosis type I is presented. The patient was a 33-year-old woman who had headache and vomiting for one week. Multiple neurofibromas over her whole body with many cafeau- lait spots were present since childhood. At admission, she had no focal neurological deficit and ophthalmologic examination revealed bilateral Lisch nodules. Brain CT and MRI revealed a heterogeneously enhancing mass in the left fronto-parietal region with marked peritumoral edema and mass effect. The tumor was removed gross totally and a gliosarcoma was diagnosed histopathologically. Post operative course was uneventful with resolution of symptom, followed by radiotherapy with 60 Gy. A brief overview is given of this rare case together with the pertinent literature.

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Neurofibromatosis type I: points to be considered by general pediatricians

  • Kang, Eungu;Yoon, Hee Mang;Lee, Beom Hee
    • Clinical and Experimental Pediatrics
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    • v.64 no.4
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    • pp.149-156
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    • 2021
  • Neurofibromatosis type 1 (NF1), a prevalent genetic disease that is transmitted in an autosomal dominant manner, is characterized by multiple cutaneous cafe-au-lait spots and neurofibromas as well as various degrees of neurological, skeletal, and neoplastic manifestations. The clinical features of NF1 increase in frequency with age, while the clinical diagnosis can remain undetermined in some pediatric patients. Importantly, affected patients are at risk for developing tumors of the central and peripheral nervous system. Therefore, adequate counseling for genetic testing, age-appropriate surveillance, and management are important. This review suggests several issues that should be considered to help general pediatricians provide adequate clinical care and genetic counseling to patients with NF1 and their families.

Two Cases of Buccal Mass:Plemorphic Adenoma of an Accessory Parotid Gland and Angiomyoma (협부 종물 2례:부이하선의 다형선종과 혈관 평활근종)

  • Lee, Sang-Hyuk;Lee, No-Hee;Park, Il-Seok;Kim, Jin-Hwan
    • Korean Journal of Head & Neck Oncology
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    • v.23 no.1
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    • pp.63-66
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    • 2007
  • The differential diagnosis of lesions in buccal area include lipoma, neurofibromas, epidermoid cyst, salivary ducts calculus, hemangioma, lymphadenopathy. Accessory parotid glands is defined as salivary gland tissue adjacent to the parotid duct, but separated from the body of parotid and it may be found in approximately 20% of human parotid glands. The appearance of an accessory parotid tumor is rare, with a reported frequency of 7.7% of all parotid neoplasm. Angiomyoma, which is also termed angioleiomyoma, is a rare solitary subcutaneous tumors arising from the vascular smooth muscle. It often occur in the extremities and is rarely found in buccal area. We present 2 cases of rare tumor in buccal mass and resected surgically without facial nerve palsy.

A Case of Huge Neurofibroma of the Parapharyngeal Space in the Neurofibromatosis Type I Patients (Neurofibromatosis Type I 환자에서 부인두강에 발생한 거대 신경섬유종증 1례)

  • Lee Hyung-Seok;Lee Seung-Hwan;Huh Young-Don;Hong Dong-Kyun;Lee Yoon-Seo
    • Korean Journal of Head & Neck Oncology
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    • v.16 no.1
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    • pp.87-91
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    • 2000
  • Neurofibromatosis type I or Von Recklinghausen's disease can occur at any site in the body. It is characterized by multiple $c\'{a}fe\;\'{a}u\;l\'{a}it$ spots on the skin-more than six spots greater than 1.5cm-, neurofibromas of the peripheral and centarl nervous system, and variety of other dysplastic abnormalities of the skin, bones, endocrine organs, nervous systems, and blood vessels. It is an autosomal dominant trait disease with a frequency of 1 of 3000. Neurofibromatosis is known to be complicated by malignancies. Neurofibromatosis is progressive disease and shows a marked variations in expression in affected individuals. In this report we describe a male patient with neurofibromatosis type I developed in the parapharyngeal space. The patient had huge mass at left parapharyngeal space and inguinal area. We successfully treated the patient with surgery without complication.

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A Case of Lung Cancer associated with von Recklinghausen's Disease (Von Recklinghausen 병에 동반된 폐암 1예)

  • Han, Yo-Seb;Kang, Hong-Mo;Han, Min-Soo;Yoo, Jee-Hong
    • Tuberculosis and Respiratory Diseases
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    • v.45 no.3
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    • pp.604-608
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    • 1998
  • Von Recklinghausen's disease is an autosomal dominant hereditary disease associated with characteristic cafe-au-lait spots of skin and multiple neurofibromatosis. It is complicated by malignancies, which in most cases is neurofibrosarcoma. The development of lung cancer in von Recklinghausen's disease is rare. A 61-year-old male was admitted for cough and sputum for 20 days. He had multiple cafe-au-lait spots and subcutaneous neurofibromas in whole body area and Lisch nodules in both iris and he had been diagnosed von Recklinghausen's disease 35 years ago. Chest radiography showed emphysematous bullae in both upper lung field and mass in right upper lung field. Chest CT scan revealed subcarinal lymph node enlargement. Bronchoscopic biopsy was done in mass in superior segment of right lower lobe and the results showed squamous cell carcinoma. The presence of von Recklinghausen's disease and lung cancer are noteworthy.

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A Large Malignant Peripheral Nerve Sheath Tumor in the Neurofibromatosis Patient: A Case Report (신경섬유종증 환자에 발생한 거대 악성말초신경초종의 치험례)

  • Choi, Dong Il;Seo, Dong Kuk;Jo, Woo Sung;Chung, Chul Hoon;Cho, Seong Jin
    • 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.

A Neurofibroma arising from nasal septum (비중격에 발생한 신경섬유종의 내시경적 비내 수술 치험 1예)

  • Hong, Ji-Won;Lee, Jun-Ho;Park, Dong-Jin;Kim, Myung-Gu
    • Korean Journal of Bronchoesophagology
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    • v.14 no.1
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    • pp.46-49
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    • 2008
  • Neurofibroma is a neurogenic tumor arising from schwann cells or peripheral tissues of nerve sheaths. Neurofibroma rarely occurs as a solitary lesion but mostly occurs as part of neurofibromatosis and reports of neurofibromas developed in the nasal cavity or paranasal sinus are very rare. We report on a case of neurofibroma of the nasal cavity treated by endoscopic surgery. Preoperative computed tomography(CT), and punch biopsy suggested that the tumor was benign neurogenic tumor cofined to right nasal cavity. The tumor was removed with endoscopic surgery completely, and confirmed as neurofibroma by histological and immunohistochemical examination. We discuss the clinical and pathological characters of neurofibroma arising in the nasal septum.

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A Case of the Solitary Neurofibroma at the Median Nerve -Ultrastructural Study- (정중신경에 발생한 신경섬유종 치험례 -초미형태학적 관찰-)

  • Ihn, Joo-Chul;Ahn, Jong-Chul;Kim, Sae-Dong;Ahn, Myun-Whan
    • Journal of Yeungnam Medical Science
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    • v.1 no.1
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    • pp.199-206
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    • 1984
  • With respect to the histogenesis of the neurofibroma, there are many controversies, since von Recklinghausen postulated that neurofibromas are mainly fibrous tumors arising from the connective tissue of the nerve sheaths. According to the recent studies through the electron microscope, the neoplastic perineurial cells are regarded as the main components of the tumor. So, the neurofibroma enucleated from the median nerve at the level of proximal arm without resulting neurologic sequale was examined through the light- and electron microscope. Ultrastructurally, this tumor is composed of the perineurial cells, fibroblasts, and collagen fibers. The predominant perineurial cells show distinct basal lamina, and contain abundant microfilaments.

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