Korean Journal of Otorhinolaryngology-Head and Neck Surgery

Korean Society of Otolaryngology-Head and Neck Surgery (대한이비인후과학회)
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A Case of Malignant Peripheral Nerve Sheath Tumor in Parapharyngeal Space

부인두 공간에서 발생한 악성 말초 신경초 종양 1예

  • Lee, Hyun Sub (Department of Otorhinolaryngology-Head and Neck Surgery, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine) ;
  • Lee, Chang Hee (Department of Otorhinolaryngology-Head and Neck Surgery, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine) ;
  • Jin, Sung Min (Department of Otorhinolaryngology-Head and Neck Surgery, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine) ;
  • Lee, Sang Hyuk (Department of Otorhinolaryngology-Head and Neck Surgery, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine)
  • 이현섭 (성균관대학교 의과대학 강북삼성병원 이비인후과학교실) ;
  • 이창희 (성균관대학교 의과대학 강북삼성병원 이비인후과학교실) ;
  • 진성민 (성균관대학교 의과대학 강북삼성병원 이비인후과학교실) ;
  • 이상혁 (성균관대학교 의과대학 강북삼성병원 이비인후과학교실)
  • Received : 2011.12.13
  • Accepted : 2012.01.27
  • Published : 2012.03.25
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Abstract

The malignant peripheral nerve sheath tumor is an extremely rare soft tissue sarcoma. It is a highly malignant sarcoma, which is locally invasive, frequently leading to multiple recurrences and eventual metastatic spread. The peak incidence of disease is known to occur sporadically between the age of 20s and 50s, and is usually associated with the neurofibromatosis type I. In human body, the trunk and extremities are the most commonly involved sites, with only 8-14% of all lesions appearing in the head and neck region. We present a case of malignant peripheral nerve sheath tumor involving the right parapharynx in a 48-year-old patient who complained of headaches in the right parietal area and of dysphagia that aggravated over a month. After surgery, tumor was finally diagnosed as malignant peripheral nerve sheath tumor by histopathologic examinations. The authors report a case of malignant peripheral nerve sheath tumor in the right parapharynx with a review of the literature.

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