DOI QR코드

DOI QR Code

작은 크기에서 현저한 주췌관 확장과 췌장실질 위축을 동반한 신경내분비종

A Small Pancreatic Neuroendocrine Tumor with Marked Pancreatic Duct Dilatation and Parenchymal Atrophy

  • 원성현 (울산대학교 의과대학 서울아산병원 내과) ;
  • 홍승모 (울산대학교 의과대학 서울아산병원 병리과) ;
  • 김명환 (울산대학교 의과대학 서울아산병원 내과) ;
  • 박상우 (울산대학교 의과대학 서울아산병원 내과) ;
  • 최윤식 (울산대학교 의과대학 서울아산병원 내과) ;
  • 장일영 (울산대학교 의과대학 서울아산병원 내과) ;
  • 김형중 (울산대학교 의과대학 서울아산병원 영상의학과)
  • Won, Sunghyun (Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Hong, Seung-Mo (Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Kim, Myung-Hwan (Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Park, Sangwoo (Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Choi, Yunsik (Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Jang, Il-Young (Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Kim, Hyoung Jung (Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine)
  • 투고 : 2013.08.14
  • 심사 : 2013.09.13
  • 발행 : 2014.05.01

초록

췌장 신경내분비종양은 작은 크기에서도 췌관의 협착에 의한 상류쪽 주췌관의 확장을 동반할 수 있다. 췌관을 직접 침범하지 않고도 췌관의 협착이 발생할 수 있으며 특징적으로 세로토닌 면역염색에서 양성을 보이는 경우가 많다. CT에서 조영증강되는 종괴가 있으면서 주췌관의 확장과 췌장 실질의 위축이 동반될 경우, 일단 췌장선암의 존재 여부를 확인하는 것이 가장 중요하지만 췌장 신경내분비종양도 감별진단에 포함해야 한다. 그리고 세로토닌 분비 췌장 신경내분비종양 가능성을 고려하여 추가 면역화학염색이 필요함을 생각할 수 있다.

Pancreatic ductal adenocarcinomas often cause marked pancreatic duct dilatation and associated parenchymal atrophy. We present the case of a small pancreatic neuroendocrine tumor with upstream pancreatic duct dilatation and severe parenchymal atrophy. A small enhancing tumor was observed at the head of the pancreas on computed tomography (CT). Endoscopic ultrasonography-guided fine-needle aspiration was negative for malignancy. We performed a pylorus-preserving pancreatoduodenectomy since we could not exclude the presence of pancreatic ductal adenocarcinoma. The pathological and immunohistochemical examination revealed a serotonin-positive neuroendocrine tumor, measured $1.0{\times}0.5cm$. The pathological specimen was remarkable for the marked stromal fibrosis in the area of the tumor, which resulted in narrowing of the main pancreatic duct. Here, we report a rare small pancreatic neuroendocrine tumor, the CT image of which resembled that of pancreatic ductal adenocarcinoma, in which the expression of serotonin and associated fibrosis might be a possible mechanism for the marked main pancreatic duct dilatation.

키워드

참고문헌

  1. Yao JC, Eisner MP, Leary C, et al. Population-based study of islet cell carcinoma. Ann Surg Oncol 2007;14:3492-3500. https://doi.org/10.1245/s10434-007-9566-6
  2. Bilimoria KY, Tomlinson JS, Merkow RP, et al. Clinicopathologic features and treatment trends of pancreatic neuroendocrine tumors: analysis of 9,821 patients. J Gastrointest Surg 2007;11:1460-1467. https://doi.org/10.1007/s11605-007-0263-3
  3. Hamada Y, Nakayama Y, Maeshiro K, Ikeda T, Hayashi H, Iwasaki H. Two cases of primary carcinoid tumor of the pancreas associated with marked stenosis of the main pancreatic duct. Pancreas 2009;38:834-835. https://doi.org/10.1097/MPA.0b013e3181b3afec
  4. Kawamoto S, Shi C, Hruban RH, et al. Small serotonin-producing neuroendocrine tumor of the pancreas associated with pancreatic duct obstruction. AJR Am J Roentgenol 2011;197:W482-488. https://doi.org/10.2214/AJR.10.5428
  5. Shi C, Siegelman SS, Kawamoto S, et al. Pancreatic duct stenosis secondary to small endocrine neoplasms: a manifestation of serotonin production? Radiology 2010;257:107-114. https://doi.org/10.1148/radiol.10100046
  6. Halfdanarson TR, Rabe KG, Rubin J, Petersen GM. Pancreatic neuroendocrine tumors (PNETs): incidence, prognosis and recent trend toward improved survival. Ann Oncol 2008;19:1727-1733. https://doi.org/10.1093/annonc/mdn351
  7. Kent RB 3rd, van Heerden JA, Weiland LH. Nonfunctioning islet cell tumors. Ann Surg 1981;193:185-190. https://doi.org/10.1097/00000658-198102000-00010
  8. Franko J, Feng W, Yip L, Genovese E, Moser AJ. Nonfunctional neuroendocrine carcinoma of the pancreas: incidence, tumor biology, and outcomes in 2,158 patients. J Gastrointest Surg 2009;14:541-548.
  9. Kunz PL, Reidy-Lagunes D, Anthony LB, et al. Consensus guidelines for the management and treatment of neuroendocrine tumors. Pancreas 2013;42:557-577. https://doi.org/10.1097/MPA.0b013e31828e34a4
  10. Mao C, el Attar A, Domenico DR, Kim K, Howard JM. Carcinoid tumors of the pancreas: status report based on two cases and review of the world's literature. Int J Pancreatol 1998;23:153-164. https://doi.org/10.1385/IJGC:23:2:153