• 제목/요약/키워드: Neurofibromatosis type 2 (NF2)

검색결과 23건 처리시간 0.018초

Staged Management of a Ruptured Internal Mammary Artery Aneurysm

  • Kwon, O Young;Kim, Gun Jik;Oh, Tak Hyuk;Lee, Young Ok;Lee, Sang Cjeol;Cho, Jun Yong
    • Journal of Chest Surgery
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    • 제49권2호
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    • pp.130-133
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    • 2016
  • The rupture of an internal mammary artery (IMA) aneurysm in a patient with type 1 neurofibromatosis (NF-1) is a rare but life-threatening complication requiring emergency management. A 50-year-old man with NF-1 was transferred to the emergency department of Kyungpook National University Hospital, where an IMA aneurysmal rupture and hemothorax were diagnosed and drained. The IMA aneurysmal rupture and hemothorax were successfully repaired by staged management combining endovascular treatment and subsequent video-assisted thoracoscopic surgery (VATS). The patient required cardiopulmonary cerebral resuscitation, the staged management of coil embolization, and a subsequent VATS procedure. This staged approach may be an effective therapeutic strategy in cases of IMA aneurysmal rupture.

제 I 형 신경섬유종증에 병발하였던 종격동내 횡문근육종에 의한 척수압박 1례 - 증례보고 - (A Case of Spinal Cord Compression Caused by Rhabdomyosarcoma of the Mediastinum Associated with Type I Neurofibromatosis(NF Type I) - Case Report -)

  • 김세윤;황금;홍순기;변진수;허철;김헌주;한용표;이명섭;이종국;조미연
    • Journal of Korean Neurosurgical Society
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    • 제30권5호
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    • pp.642-646
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    • 2001
  • A 12-years-old female admitted to the hospital with the complaint of pain on the right upper chest area which persisted about 1 month prior to admission. $Caf{\acute{e}}$-au-lait spots of various size laying on a whole body and freckling on the axilla were found on physical examination. A huge mass was found on the plain chest X-ray and on chest MRI. The mass encroached thoracic spine, posterior rib, back muscles, and then into the neural canal and compressed thoracic spinal cord. On the 5th day of hospitalization, the patient complained tingling on the both legs and 2 days later, monoparesis on the right leg. Open thoracotomy and decompressive laminectomy was done to remove mass. Pathologic reports confirmed rhabdomyosarcoma, embryonal type.

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쇄골 상부에 발생한 악성 Triton 종양 1예 (A Case of Malignant Triton Tumor on Supraclavicular Area)

  • 임상호;박희택;홍기환
    • 대한두경부종양학회지
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    • 제29권2호
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    • pp.54-57
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    • 2013
  • Malignant triton tumor(MTT) is a rare type of malignant peripheral nerve sheath tumor(MPNST) with focal rhabdomyoblastic differentiation. MTT constitutes about 5% of all MPNSTs and described the first case of a MTT in a patient with Von Recklinghausen disease by Masson in 1932. MTT is commonly seen in the head, neck, extremities and trunk. It can occur in sporadic form or over a setting of neurofibromatosis-1(NF-1). The diagnosis can be confirmed based on morphologic grounds supported by an immunostain such as S-100 protein. Desmin, myo-D1 and myogenin are immunostains positive for rhabdomyoblasts. MTT has an aggressive biological behavior so prognosis of this rare and highly malignant tumor is poor and optimal treatment remains unclear. But modern treatment consisted of radical excision and postoperative radiotherapy has improved the prognosis of such cases.