• Title/Summary/Keyword: neurofibroma

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Compression of the Superficial Radial Nerve by Schwannoma: A Case Report (신경초종에 의한 표재요골신경의 압박)

  • Kim, Hyun-Sung;Kim, Chul-Han;Kang, Sang-Gue;Tark, Min-Seong
    • Archives of Plastic Surgery
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    • v.38 no.4
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    • pp.494-497
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    • 2011
  • Purpose: Schwannoma, a benign peripheral nerve tumor, is slow-growing, encapsulated neoplasm that originates from the Schwann cell of the nerve sheath. Schwannoma most frequently involves the major nerve. Schwannoma occurring in the superficial radial nerve rare. This is a report of our experience with schwannoma arising from the superficial radial nerve with neurologic symptom. Methods: A 55-year-old woman presented with eight-month history of progressive numbness and paresthesia in dorsum of the thumb and index finger. Physical examination revealed a localized mass on the midforearm. Sonographic examination showed an ovoid, heterogenous, hypoechoic lesion, located eccentrically in related to the superficial radial nerve. The lesion was mobile in the transverse but not in the longitudinal axis of the nerve, which was thought to favour schwannoma rather than neurofibroma. At operation, a $20{\times}15mm$ ovoid, yellowish grey mass was seen arising from the superficial radial nerve. The tumor present as eccentric masses over which the nerve fibers are splayed. Using operating microscope, the tumor was removed, preserving the surrounding nerve. Results: Histology confirmed that the mass was a benign schwannoma. There were no postoperative complications. After two months the patient had no clinically demonstrable sensory deficit. Conclusion: An unsusual case of a schwannoma of the superficial radial nerve is presented. In case with neurologic symptom, prompt surgical decompression must be made to prevent further nerve damage and to restore nerve function early.

Cervical Ganglioneuroma Associated with Neurofibromatosis Type 1 (제 1형 신경섬유종증에 동반된 경부 신경절신경종)

  • Choi, Eui-Chul;Kim, Jun-Hyuk;Shin, Ho-Seong;Lee, Ji-Hye;Lee, Young-Man
    • Archives of Plastic Surgery
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    • v.37 no.4
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    • pp.477-480
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    • 2010
  • Purpose: Ganglioneuromas are well-differentiated tumors derived from neuroectodermal neural crest cells. Although these tumors can occur anywhere along the sympathetic chain from the base of the skull to the pelvic cavity, they usually develop in the posterior mediastinum and retroperitoneum these tumors are rarely found in the cervical region. Method: We report the case of a 16-year-old male patient with neurofibromatosis type 1 who was admitted because of a palpable mass centrally located on the left side of the neck. A preoperative contrast-enhanced neck computed tomography image showed a low-density homogeneous mass on the parapharyngeal space along with marked displacement of the trachea and carotid vessels. Round and soft masses were also detected on both axillae. Results: The patient subsequently underwent complete excision of the neck mass via the transcervical approach. The mass was smooth and well encapsulated between the sternocleidomastoid muscle and the trachea. Further, the mass appeared to arise from the cervical sympathetic chain, which was preserved during surgery. Both the axillary masses were also excised. The histopathological findings were ganglioneuroma for the neck mass and neurofibroma for both the axillary masses. Conclusion: Cervical ganglioneuromas are rare tumors that present as enlarging parapharyngeal cervical masses in the oropharynx or neck. To our knowledge, a case of cervical ganglioneuroma associated with neurofibromatosis type 1 has never been reported. In patients with neurofibromatosis, multiple tumors may develop, and therefore periodic clinical and radiological follow-up is recommended. Further, repeated imaging analysis should be performed if the presence of another tumor is suspected.

Is Video-assisted Thoracoscopic Resection for Treating Apical Neurogenic Tumors Always Safe? (흉강 첨부 양성 신경종의 흉강경을 이용한 절제술: 언제나 안전하게 시행할 수 있나?)

  • Cho, Deog Gon;Jo, Min Seop;Kang, Chul Ung;Cho, Kyu Do;Choi, Si Young;Park, Jae Kil;Jo, Keon Hyeon
    • Journal of Chest Surgery
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    • v.42 no.1
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    • pp.72-78
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    • 2009
  • Background: Mediastinal neurogenic tumors are generally benign lesions and they are ideal candidates for performing resection via video-assisted thoracoscopic surgery (VATS). However, benign neurogenic tumors at the thoracic apex present technical problems for the surgeon because of the limited exposure of the neurovascular structures, and the optimal way to surgically access these tumors is still a matter of debate. This study aims to clarify the feasibility and safety of the VATS approach for performing surgical resection of benign apical neurogenic tumors (ANT). Material and Method: From January 1996 to September 2008, 31 patients with benign ANT (15 males/16 females, mean age: 45 years, range: 8~73), were operated on by various surgical methods: 14 VATS, 10 lateral thoracotomies, 6 cervical or cervicothoracic incisions and 1 median sternotomy. 3 patients had associated von Recklinhausen's disease. The perioperative variables and complications were retrospectively reviewed according to the surgical approaches, and the surgical results of VATS were compared with those of the other invasive surgeries. Result: In the VATS group, the histologic diagnosis was schwannoma in 9 cases, neurofibroma in 4 cases and ganglioneuroma in 1 case, and the median tumor size was 4.3 cm (range: 1.2~7.0 cm). The operation time, amount of chest tube drainage and the postoperative stay in the VATS group were significantly less than that in the other invasive surgical group (p<0.05). No conversion thoracotomy was required. There were 2 cases of Hornor's syndrome and 2 brachial plexus neuropathies in the VATS group; there was 1 case of Honor's syndrome, 1 brachial plexus neuropathy, 1 vocal cord palsy and 2 non-neurologic complications in the invasive surgical group, and all the complications developed postoperatively. The operative method was an independent predictor for postoperative neuropathies in the VATS group (that is, non-enucleation of the tumor) (p=0.029). Conclusion: The VATS approach for treating benign ANT is a less invasive, safe and feasible method. Enucleation of the tumor during the VATS procedure may be an important technique to decrease the postoperative neurological complications.

Primary neurofibroma of the Diaphragm (횡격막에 발생한 신경섬유종 1례)

  • 유회성
    • Journal of Chest Surgery
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    • v.8 no.2
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    • pp.149-152
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    • 1975
  • In spite of great advances in surgical treatment during past several decades, surgery of the trachea failed to develop correspondingly, partly because of relative rarity of the tracheal lesions and partly because of difficulties in surgical technique and anesthesia. Surgical diseases of the trachea are largely obstructions due to neoplasm or cicatrical stenosis and tracheal malacia. The present treatment of respiratory failure, using cuffed endotracheal and tracheostomy tubes, has produced, apparently with increasing frequency, tracheal stenosis, tracheomalized tracheal erosion. Surgery is presently the only reasonable way to treat stenotic lesions of the tracheobronchial tree. In the case of tumors, the current trend has been that of radical excision. Primary end-to--end reconstruction of the trachea has been generally recognized as the ideal method of repair following resection. However, for decades it was believed that a maximum of four tracheal rings only might be excised and primary healing achieved with safety. A great variety of procedures, developed by numerous investigations and directed at tracheal substitution, have almost invariably met with discouraging results. A meticulous study done by Grillo and associates on autopsy specimens has shown that an average 6.4cm of mediastinal trachea can be safely resected by full mobilization of the right lung and transplantation of the left main bronchus into the bronchus intermedius. Recently, we experienced a case of successful resection of a tumor of the tracheal carina and primary tracheo-left main bronchial anastomosis at the Department of Thoracic & Cardiovascular Surgery, the National Medical Center in Seoul. The patient, a 29-year-old man, was admitted to the hospital with complaints of dyspnea and cough. On admission, chest film showed hydropneumothorax on the right. After closed thoracostomy, hydropneumothorax disappeared, but hazy densities, developed in the right middle and lower lung fields, resisted to treatment. Bronchoscopy uncovered irregular tumor covering the carina and the right main bronchus, and biopsy indicated well differentiated squamous Cell carcinoma. Operation was performed on July 2, 1975. A right postero-lateral thoracotomy was used. Excision involved the lower trachea, the carina, the left main bronchus and the right lung. This was followed by direct anastomosis between the trachea and the left main bronchus. Bronchography was done on 17th postoperative day revealed good result of operation without stricture at the site ofanastomosis. About one month after the operation symptoms and signs of bronchial irritation with dyspnea developed, and these responded to respiratory care. On 82nd postoperative day, sudden dyspnea developed at night and the patient expired several hours later. Autopsy was not done and the cause of death was uncertain.

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Multiple External Carotid Artery Aneurysms with Neurofibromatosis - Case Report - (신경섬유종을 동반한 다발성 외경동맥 동맥류 - 증 례 보 고 -)

  • Pyo, Sae Yeong;Kim, Moo Seong;Sim, Hong Bo;Lee, Sun Il;Jung, Yong Tae;Kim, Soo Chun;Sim, Jae Hong
    • Journal of Korean Neurosurgical Society
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    • v.29 no.9
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    • pp.1248-1254
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    • 2000
  • Multiple external carotid artery aneurysms with neurofibromatosis are rare. Trauma is the primary cause in the development of aneurysms of the external carotid artery. A 39-year-old female patient was referred to the emergency room because of a headache and a huge lump over the left temporo-parieto-occipital region. The physical examination revealed a huge round mass, $5{\times}15{\times}18cm$, in the left temporo-parieto-occipital region and low set left ear and multiple caf au lait spots in trunk and extremities. The external carotid artery angiography demonstrated multiple aneurysms arising from the superficial temporal artery and occipital artery. A MRI showed a huge hematoma on temporo-parieto-occipital region and postauricular mass suggested of subcutaneous neurofibroma. Embolization followed by surgical resections of those aneurysms and neighboring mass were performed and good result was obtained. We report our case with review of literature.

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A Study in Bridging Sciatic Nerve Defects with Combined Skeletal Muscle and Vein Conduit in Rats (백서의 좌골신경에서 정맥 및 골격근을 이용한 결손신경 봉합술에 대한 연구)

  • Lee, Jun-Mo
    • Archives of Reconstructive Microsurgery
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    • v.6 no.1
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    • pp.29-38
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    • 1997
  • A peripheral nerve when approximation of the ends imparts tension at the anastomosis and with a relatively long segment defect after excision of neuroma and neurofibroma cannnot be repaired by early primary suture. The one of the optimistic reconstruction method of severed peripheral nerves is to restore tension-free continuity at the repair site putting an autogenous nerve graft into the neural gap despite of ancipating motor or sensory deficit of the donor nerve area. To overcome the deficit of the autogenous nerve graft, several other conduits supplying a metabolically active environment which is able to support axon regeneration and progression, providing protection against scar invasion, and guiding the regrowing axons to the distal stump of the nerve have been studied. An author have used ipsilateral femoral vein, ipsilateral femoral vein filled with fresh thigh muscle, and autogenous sciatic nerve for the sciatic nerve defect of around 10 mm in length to observe the regeneration pattern in rat by light and electron microscopy. The results were as follows. 1. Light microscopically regeneration pattern of nerve fibers in the autogenous graft group was more abundant than vein graft and vein filled with muscle group. 2. On ultrastructural findings, the proxial end of the graft in various groups showed similar regenerating features of the axons, myelin sheaths, and Schwann cells. The fascicular arrangement of the myelinated and unmyelinated fibers was same regardless of the type of conduits. There were more or less increasing tendency in the number and the diameter of myelinated fibers correlated with the regeneration time. 3. In the middle of the graft, myelinated nerve fibers of vein filled with muscle group were more in number and myelin sheath was thinner than in the venous graft, but the number of regenerating axons in autogenous nerve graft was superior to that in both groups of the graft. The amount of collagen fibrils and amorphous materials in the endoneurial space was increased to elapsed time. 4. There was no difference in regenerating patterns of the nerve fibers of distal end of the graft. The size and shape of the myelinated nerve fbers were more different than that of proximal and middle portion of the graft. From the above results, the degree of myelination and regenerating activity in autogenous nerve is more effective and active in other types of the graft and there were no morphological differences in either ends of the graft regardless of regeneration time.

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External Fixation of Retaining Ligament in Correction of Facial Disfigurement in Type-1 Neurofibromatosis Patients (유지 인대의 외부 고정을 통한 제1형 신경섬유종증 환자의 안면부 변형 교정)

  • Myung, Yu-Jin;Lee, Yoon-Ho
    • Archives of Plastic Surgery
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    • v.38 no.3
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    • pp.257-262
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    • 2011
  • Purpose: In neurofibromatosis patients, complete surgical excision of the mass is almost impossible and surgical treatment usually consists of multiple serial excisions that only result in a debulking effect. Remnant tumor mass has a gravitational effect on facial soft tissues that leads to sagging of skin and soft tissue, and eventually, facial disfigurement and asymmetry. The purpose of our surgical method is to perform soft tissue lifting with longer lasting effect with less surgical risk of damaging facial nerve and vessels. With external fixation using K-wire or surgical screw, the procedure only called for a short incision length and had additional adhesive properties that enabled anchoring of soft tissue in a lifted position for a longer postoperative period. Methods: A total of 5 neurofibromatosis patients (NF-1) visited our clinic for mass reduction and face lifting. The age of patients ranged from 13 to 42 (mean 28.8 years), and most patients had a long history of multiple excisions in the past. Face lifting was performed in 2 different areas, the periorbital area in 3 patients, and the midface in 2 patients. The materials used in fixation of retaining ligament were K-wire (n=3) and titanium screw (n=2). Results: Follow up period was from 5 month to 3 years and 1 month (mean=2 years and 1 month). All patients conveyed satisfaction with the results and no major complications were reported. The lifting effect lasted for as long as 3 years, and there were no complaints of relapse of soft tissue depression or sagging within the operated area. 1 patient (M/13) needed secondary k-wire insertion and additional mass excision in 1 year and 10 months postoperatively due to tumor growth. In two patients with K-wire fixation, mild dimpling and tenderness were observed in the follow up period, but in about 2 months postoperatively, dimpling was relieved and there was no need for removal of fixating material. Conclusion: Surgical lifting in neurofibromatosis patients can be challenging, for mass excision cannot be done completely and gravitational effect by residual mass can be persistent. External fixation of the retaining ligament in patients with neurofibromatosis can give satisfactory results-for incision length is relatively shorter, and the lifting effect can last longer compared to other various face lifting techniques.

A MODIFIED ENDAURAL AND NECK APPROACH TO THE PAROTID GLAND TUMOR (이하선 종양에 대한 변형된 이내 및 경부접근법)

  • Ryu, Sun-Youl;Kook, Min-Suk;Kim, Sun-Kook;Han, Chang-Hun;Gu, Hong;Kwon, Jun-Kyung;An, Jin-Suk
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.31 no.6
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    • pp.501-508
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    • 2005
  • Generally we use the preauricular incision to access and remove the parotid gland tumor. But the preauricular approach has some complications such as damage of facial nerve and sensory nerve, Frey's syndrome, and postoperative scar. Especially, the postoperative scar can often cause an unesthetic result and mental stress in young patients. Therefore, if we avoid preauricular incision to be performed outside of tragus, the postoperative scar would be hardly remarkable, and patients would be satisfied cosmetically. We performed surgical excision using a modified endaural and neck approach in a 21-year-old female with a pleomorphic adenoma and 15-year-old male with a neurofibroma occured in the parotid gland. A new, modified endaural and neck approach is a combined method of the modified endaural incision by Starck et al and Gutierrez's neck extension. We obtained an adequate access and the cosmetically acceptable postsurgical scar. The postoperative scars were hidden in the external ear and the hairline. Moreover, except the neck dissection can this approach be applied to the surgery of temporomandibular joint as well as the parotid gland tumor.

A Case of Spontaneous Hemothorax Due to Rupture of Pseudoaneurysm in Type 1 Neurofibromatosis (신경섬유종증에 동반된 가성동맥류 파열로 발생한 자연 혈흉 1예)

  • Kim, Sun-Jong;Jeong, Hoon;Lee, Sung-Soon;Lim, Chae-Man;Lee, Sang-Do;Koh, Youn-Suck;Kim, Woo-Sung;Kim, Dong-Soon;Kim, Won-Dong;Shim, Tae-Sun
    • Tuberculosis and Respiratory Diseases
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    • v.50 no.1
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    • pp.122-126
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    • 2001
  • A non-traumatic, spontaneous hemothorax is rare. The most common causes are coagulopathy, due to anticoagulation treatment, and cancers with a metastasis to the pleural surface. Other unusual causes include thoracic endometriosis, ruptured aortic aneurysm, pulmonary arterio-venous malformation, coagulopathy, Osler-Rendeu-Weber syndrome, Ehlers-Danlos syndrome et cetera. A type 1 neurofibromatosis(Von Recklinghausen's disease) is an autosomal dominant disease that is characterized by multiple skin tumors(neurofibroma) and abnormal skin pigmentation(caf$\acute{e}$-au-lait spots). Some are accompanied by vasculopathy, and are present with a spontaneous hemothorax. Such cases are unusual but fatal. We have recently experienced a case where a young male patient with neurofibromatosis initially presented with hypovolemic shock due to a spontaneous hemothorax. Later, aortography revealed that the cause of the hemothorax was a rupture of a pseudoaneurysm of the right internal mammary artery and as a result, an embolization was performed. Here we report this case with a review of the appropriate literature.

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