Archives of Craniofacial Surgery (대한두개안면성형외과학회지)

Korean Cleft Palate-Craniofacial Association (대한두개안면성형외과학회)
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Epithelioid sarcoma associated with neurofibromatosis type I

  • Hwang, Sung Oh (Department of Plastic and Reconstructive Surgery, Inje University Ilsan Paik Hospital, Inje University College of Medicine) ;
  • Lee, Soo Hyang (Department of Plastic and Reconstructive Surgery, Inje University Ilsan Paik Hospital, Inje University College of Medicine) ;
  • Lee, Han Byul (Department of Plastic and Reconstructive Surgery, Inje University Ilsan Paik Hospital, Inje University College of Medicine)
  • Received : 2019.11.04
  • Accepted : 2019.12.16
  • Published : 2020.02.20
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Abstract

In general, patients with neurofibromatosis type I have a higher risk than those with other types of neurofibromatosis of developing soft-tissue sarcomas related to the nervous system. We here present a 42-year-old man with neurofibromatosis type I who developed a protruding mass over only 2 weeks. The histopathological diagnosis was epithelioid sarcoma. Epithelioid sarcomas are rare and, to the best of our knowledge, no epithelioid sarcomas have been reported in patients with neurofibromatosis type I. Radical excision of the primary lesion was performed and postoperative radiotherapy and chemotherapy administered, as is recommended for epithelioid sarcoma. Our case emphasizes that patients with neurofibromatosis type I may develop malignant tumors.

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References

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