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소규섭,임영국,홍용택,김훈남,김대용,윤화영,우계형 대한성형외과학회 2011 Archives of Plastic Surgery Vol.38 No.6
Purpose: Malignant peripheral nerve sheath tumor without neurofibromatosis type 1 is very rare neoplasm. Development in the superficial soft tissue is exremely rare. Authors experienced one rare case of primary malignant peripheral nerve sheath tumor developed on abdomen. The clinical and histologic findings were described. Methods: An 83-year-old man visited hospital with an 11 × 6.5 × 4.5 cm sized ulcerated and hemorrhagic mass on abdomen. The tumor was localized in abdominal skin and started growing 3 years ago. Results: Wide excision with safety margin of 2 cm and limberg flap was done. The postoperative biopsy revealed a malignant peripheral nerve sheath tumor. There was no evidence of recurrence of tumor for 16 months. Conclusion: Malignant peripheral nerve sheath tumor is an aggressive malignant tumor. An abrupt enlargement of size, ulceration and bleeding are suggestive of malignant chnages of the tumor. We recommand early wide excision with enough safety margin as treatment of malignant peripheral nerve sheath tumor.
신경섬유종증 환자에 발생한 거대 악성말초신경초종의 치험례
최동일,서동국,조우성,정철훈,조성진 대한성형외과학회 2006 Archives of Plastic Surgery Vol.33 No.6
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 1cm 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.
제 1형 신경섬유종증 환자의 총상신경초종에서 발생한 악성 말초신경초종
윤여훈,노종렬 충남대학교 암공동연구소 2006 암공동연구소 업적집 Vol.5 No.
Malignant peripheral nerve sheath tumor (MPNST) is defined as a malignant tumor arising from cells of the peripheral nerve sheath. The patients with neurofibromatosis type 1 (NF1) have the highest probability of developing MPNST. Even with wide surgical excision and adjuvant chemoradiation, the prognosis of MPNST is generally poor. We experienced a case of MPNST in a 38-year-old man with a long-standing NF1. He had a painless, infraauricular mass of 1 month duration. The patient underwent excision of the mass followed by total parotidectomy and modified neck dissection. Permanent pathologic sections confirmed MPNST. We report this case with a review of the literature.
제 1형 신경섬유종증 환자의 총상신경초종에서 발생한 악성 말초신경초종
윤여훈,노종렬 충남대학교 암연구소 2006 암연구소 업적집 Vol.5 No.-
Malignant peripheral nerve sheath tumor (MPNST) is defined as a malignant tumor arising from cells of the peripheral nerve sheath. The patients with neurofibromatosis type 1 (NF1) have the highest probability of developing MPNST. Even with wide surgical excision and adjuvant chemoradiation, the prognosis of MPNST is generally poor. We experienced a case of MPNST in a 38-year-old man with a long-standing NF1. He had a painless, infraauricular mass of 1 month duration. The patient underwent excision of the mass followed by total parotidectomy and modified neck dissection. Permanent pathologic sections confirmed MPNST. We report this case with a review of the literature.
이기영,배준성,전영준,이희정,김영진 대한성형외과학회 2007 Archives of Plastic Surgery Vol.34 No.4
Purpose: Malignant peripheral nerve sheath tumors (MPNSTs) are rare neoplasms, usually arising from somatic soft tissues or peripheral nerves. Primary MPNST of the scalp is extremely rare. The case is being reported for its rarity.Methods: A 53-year-old female was presented with a scalp mass on vertex area. The tumor was localized in scalp skin and did not invade underlying periosteum or skull and treated with complete surgical excision followed by adjuvant chemotherapy and radiotherapy.Results: Histologically, the tumor showed malignant spindle cells with focal S-100 positivity on immunohistochemistry and a diagnosis of MPNST was made.Conclusion: Authors experienced a rare case of primary scalp MPNST and report the case.
구개골에 발생한 악성 Peripheral Nerve Sheath Tumor의 증례보고
권민수(Min-Su Kwon),이현상(Hyun-Sang Lee),김현창(Hyun-Chang Kim),고승오(Seung-O Ko),신효근(Hyo-Keun Shin) 대한구강악안면외과학회 2004 대한구강악안면외과학회지 Vol.30 No.3
Summary: The malignant peripheral nerve sheath tumor(MPNST) is an aggressive neoplasm and can either arise independently or result from malignant change in preexisting neurofibromatosis (von Recklinghausen’s disease). Its histologic characteristics remain controversial, but currently it is believed that the schwann cell is the origin of the peripheral nerve sheath tumors. MPNST is an uncommon neoplasm of the head and neck region, and its presentation in the oral cavity is quite rare. In this study, we report a patient with a rare case of a MPNST involving the maxilla. A case report: A 29-year-old female presented with a chief complaint of painless swelling with bleeding tendency on the left maxillary tuberosity area 2 months ago. Clinical examination showed a 5.0×3.0 cm2 sized, indurative swelling on the site. Conventional radiographs showed a relatively well-defined soft tissue mass involving the left maxillary sinus, and destruction of the anterior, posterolateral walls of the left maxillary sinus. Subtotal maxillectomy and split-thickness skin graft from thigh were undertaken. In histochemical and immunohistochemical studies, the specimen revealed positive reactivities to Vimentin and S-100 protein. Final diagnosis was made as MPNST.
증례 : 호흡기 ; 제1형 신경섬유종증 환자에서 발생한 흉강내 악성말초신경초종 1예
최진이 ( Jin Yi Choi ),김정훈 ( Jeong Hun Kim ),최자성 ( Ja Sung Choi ),정재호 ( Jae Ho Chung ),최정은 ( Jeong Eun Choi ),오화은 ( Hwa Eun Oh ),박찬섭 ( Chan Sub Park ) 대한내과학회 2009 대한내과학회지 Vol.76 No.2
저자 등은 1형 신경섬유종증 환자에서 발생한 흉강 내 악성말초신경초종 1예를 경험하였고 신경섬유종증을 가진 환자에서 흉강 내종괴를 보일 때 악성말초신경초종도 감별진단에 포함해야 할 것으로 생각된다. A malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue tumor defined as any malignant tumor arising from or differentiating toward the cells of the peripheral nerve sheath. It is one of the malignant tumors associated with neurofibromatosis type 1 (von Recklinghausen`s disease). They occur most commonly on the lower and upper extremities, trunk, head, and neck, while intrathoracic tumors are very rare. We report an intrathoracic MPNST in a 66-year-old female with neurofibromatosis type 1. (Korean J Med 76:234-237, 2009)
김찬규,박성규,홍대식 순천향의학연구소 2004 Journal of Soonchunhyang Medical Science Vol.10 No.2
Malignant peripheral nerve sheath tumor (MPNST) tend to develop in deep-seated large nerves, with the sciatic the most commonly affected nerve. We present a case of MPNST that developed at abdominal wall, without definite evidence of von Recklinghausen's disease. The patient was a 41-tr-old man who suffered from left flank and back pain. On physical examinations, 10 x 6 cm-sized mass palpated at left flank of abdomen. Excision and biopsy performed and he was diagnosed with MPNST.
Park, Man-Kyu,Sung, Joo-Kyung,Nam, Kyung-Hun,Kim, Kyoung-Tae The Korean Neurosurgical Society 2013 Journal of Korean neurosurgical society Vol.53 No.3
A malignant peripheral nerve sheath tumor (MPNST) is a type of sarcoma that arises from peripheral nerves or cells of the associated nerve sheath. This tumor most commonly metastasizes to the lung and metastases to the spinal cord and brain are very rare. We describe a case of young patient with spinal cord and brain metastases resulting from MPNST. An 18-year-old man presented with a 6-month history of low back pain and radiating pain to his anterior thigh. Magnetic resonance imaging showed a paraspinal mass that extended from the central space of L2 to right psoas muscle through the right L2-3 foraminal space. The patient underwent surgery and the result of the histopathologic study was diagnostic for MPNST. Six months after surgery, follow-up images revealed multiple spinal cord and brain metastases. The patient was managed with chemotherapy, but died several months later. Despite complete surgical excision, the MPNST progressed rapidly and aggressively. Thus, patients with MPNST should be followed carefully to identify local recurrence or metastasis as early as possible.
Primary Paraspinal Malignant Peripheral Nerve Sheath Tumor
Kim, Kyoung-Tae,Park, Yong-Suk,Kwon, Jeong-Taik,Kim, Young-Baeg The Korean Neurosurgical Society 2008 Journal of Korean neurosurgical society Vol.44 No.2
Malignant peripheral nerve sheath tumors (MPNSTs) are very rare tumors. We experienced a case of MPNST in the cervical paraspinal space which was not associated with neurofibromatosis. The tumor located in left C6-7 foramen and compressed C7 root. The tumor was removed through the occipital triangle. We report a case of the primary cerivcal MPNST in a patient who did not have neurofibromatosis-1.