The Korean Journal of Medicine

  • 제79권4호
  • /
  • Pages.443-447
  • /
  • 2010
  • /
  • 1738-9364(pISSN)
  • /
  • 2289-0769(eISSN)
대한내과학회 (The Korean Association of Internal Medicine)
권호 표지

신경섬유종증 환자에서 종격동과 복강 내 양측을 침범한 총상 신경섬유종 1예

A case of plexiform neurofibroma involving the mediastinum and abdomen in a patient with neurofibromatosis

  • 안건희 (가톨릭대학교 의과대학 내과학교실) ;
  • 전병연 (가톨릭대학교 의과대학 내과학교실) ;
  • 정미향 (가톨릭대학교 의과대학 내과학교실) ;
  • 정희경 (가톨릭대학교 의과대학 내과학교실) ;
  • 진종률 (가톨릭대학교 의과대학 내과학교실) ;
  • 이기현 (가톨릭대학교 의과대학 내과학교실)
  • An, Gun-Hee (Department of Internal Medicine, Bucheon St. Mary's Hospital, The Catholic University of Korea College of Medicine) ;
  • Jun, Byung-Yeon (Department of Internal Medicine, Bucheon St. Mary's Hospital, The Catholic University of Korea College of Medicine) ;
  • Jung, Mi-Hyang (Department of Internal Medicine, Bucheon St. Mary's Hospital, The Catholic University of Korea College of Medicine) ;
  • Jeong, Hee-Kyoung (Department of Internal Medicine, Bucheon St. Mary's Hospital, The Catholic University of Korea College of Medicine) ;
  • Jin, Jong-Yul (Department of Internal Medicine, Bucheon St. Mary's Hospital, The Catholic University of Korea College of Medicine) ;
  • Lee, Kee-Hyun (Department of Internal Medicine, Bucheon St. Mary's Hospital, The Catholic University of Korea College of Medicine)
  • 투고 : 2009.09.26
  • 심사 : 2009.11.04
  • 발행 : 2010.10.01
⟨ 이전 논문 다음 논문 ⟩

초록

총상 신경섬유종(Plexiform neurofibromatosis)은 슈반세포(Schwann's cell)에서 발생하며 조직학적으로 양성이고 비피막성이며 경계가 불명확한 신경초 종양이다. 국내에서 종격동과 복강 내에서 동시에 발견된 신경섬유종증 환자에 대해 보고된 바 없어, 이에 저자들은 문헌고찰과 함께 본 증례를 보고하는 바이다. 환자는 신경섬유종증을 확진시키는 피부 병변과 함께 흉부와 복부 CT에서 거대하고 균질한 융합성 종괴가 관찰되었고, 조직검사를 통해 종격동과 복강 내에 동시에 퍼져 있는 총상 신경섬유종임을 진단할 수 있었다. 본 증례에서는 특이증상이 없어 외래에서 추적관찰하기로 하였으나 향후 악성 종양의 가능성이 있으므로 주의깊은 추적관찰이 필요할 것이다.

Plexiform neurofibroma is a benign proliferation that arises from the peripheral nerves and represents one of the diagnostic features of neurofibromatosis type I (NF-1). They are commonly found in the gastrointestinal tract, and intrathoracic neurofibroma is relatively uncommon. Ultimately, this tumor grows along the length of any single nerve and may involve multiple fascicles or branches of major nerves. Here, we report a case of multiple-site plexiform neurofibromas in a 40-year-old man previously diagnosed with NF-1. Although he had no perceptible symptoms, contrast-enhanced CT revealed massive diffuse neurofibromas that involved the mediastinum, periportal region, retroperitoneal space, and the mesentery. Histopathological studies of the neck, skin, and intra-abdominal soft tissue showed loose spindle cells and collagen bundles. The microscopic appearance was typical of a plexiform-type neurofibroma. It was decided that we would observe the patient without surgical management, because the neurofibromas were asymptomatic.

키워드

참고문헌

  1. Aloi FG, Massobrio F. Solitary plexiform nerufibroma. Dermatologica 179:84-86, 1989
  2. Hwang SM, Ahn SK, Lee BJ, Lee WS, Choi EH. A case of Von Recklinghausen's disease with plexiform neurofibroma, giant pigmentation, and skeletal abnormalities. Korean J Dermatol 33:1179-1183, 1995
  3. Park JJ, Choi KJ, Lee SW, Sung SH. Plexiform neurofibroma involving the ascending colon and its mesentery in a child. J Korean Surg Soc 71:238-241, 2006
  4. Riccardi VM. Neurofibromatosis: clinical heterogenecity. Curr Probl Cancer 7:1-34, 1982
  5. Caldemeyer KS, Mirowski GW. Neurofibromatosis type 1: part I. clinical and central nervous system manifestations. J Am Acad Dermatol 44:1025-1026, 2001 https://doi.org/10.1067/mjd.2001.111615
  6. Rahman A, Sedera MA, Mourad IA, Aziz SA, Saber TK, Alsakary MA. Posterior mediastinal tumors: outcome of surgery. J Egypt Natl Canc Inst 17:1-8, 2005
  7. Lee JH, Kwon JB, Moon MH, Park K. Mediastinal neurofibroma in a patient with thpe 1 neurofibromatosis. Korean J Thorac Cardiovas Surg 40:317-320, 2007
  8. Tonsgard JH, Kwak SM, Short MP, Dachman AH. CT imaging in adults with neurofibromatosis-1: frequent asymptomatic plexiform lesions. Nerology 50:1755-1760, 1998 https://doi.org/10.1212/WNL.50.6.1755
  9. Zacharia TT, Jaramillo D, Poussaint TY, Korf G. MR imaging of abdominopelvic involvement in neurofibromatosis type 1: a review of 43 patients. Pedeatr Radiol 35:317-322, 2005 https://doi.org/10.1007/s00247-004-1352-z
  10. Hartley N, Rajesh A, Verma R, Sinha R, Sandrasegaran K. Abdominal manifestataions of neruofibromatosis. J Comput Assist Tomogr 32:4-8, 2008 https://doi.org/10.1097/rct.0b013e318054e1ca
  11. Bourgouin PM, Shepard JO, Moore EH, McLoud TL. Plexiform neurofibromatosis of the mediastinum: CT applearance. AJR Am J Roentgenol 151:461-463, 1988 https://doi.org/10.2214/ajr.151.3.461
  12. Ros RP, Eshaghi N. Plexiform neurofibroma of the pelvis: CT and MRI fingding. Magn Reson Imaging 9:463-465, 1991 https://doi.org/10.1016/0730-725X(91)90436-P
  13. Riccardi VM. The prenatal diagnosis of NF-1 and NF-2. J Dermatol 19:885-891, 1992 https://doi.org/10.1111/j.1346-8138.1992.tb03800.x
  14. Roa AG, Chinthagunta SR, Danturty I, Chigullapalli D. Plexiform neurofibroma encasing vital organs. Indian J Dermatol Venereol Leprol 75:315-317, 2009 https://doi.org/10.4103/0378-6323.51268
  15. Yohay K. Neurofibromatosis type 1 and 2. Neurologist 12:86-93, 2006 https://doi.org/10.1097/01.nrl.0000195830.22432.a5