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Allgemein- und Viszeralchirurgie up2date 2022; 16(01): 53-69
DOI: 10.1055/a-1324-8816
Leber, Galle, Pankreas, Milz

Seltene chirurgische Tumoren im biliopankreatoduodenalen Bereich

Gastrointestinale Stromatumoren, Choledochuszysten und pankreatische neuroendokrine Tumoren
Maximilian Weniger
,
Jens Werner
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Gastrointestinale Stromatumoren, Choledochuszysten und pankreatische neuroendokrine Tumoren sind seltene Tumoren des biliopankreatoduodenalen Bereichs. Eine frühzeitige Diagnose ist entscheidend für die Prognose und das Überleben der betroffenen Patienten. Dieser Beitrag gibt einen Überblick über das diagnostische Vorgehen und die therapeutischen Optionen.
Kernaussagen

  • Sowohl Patienten mit KIT- als auch mit PDGFRA-Mutation eines GIST sprechen auf Imatinib an. Dagegen gelten Patienten mit c-KIT/PDGFRA-Wildtyp-Status oder PDGFRA-D842V-Mutation als Imatinib-resistent.

  • Resektable GIST sollten reseziert werden, lokal fortgeschrittene Tumoren neoadjuvant therapiert und im Anschluss reseziert und metastasierte Tumoren nur selten chirurgisch behandelt werden.

  • Bei Patienten mit lokalisierter, nicht metastasierter GIST-Erkrankung, bei denen eine R0-Resektion unwahrscheinlich ist, sollte eine Resektion nach Vorbehandlung mit Imatinib erfolgen.

  • Potenzielle Folgen von Choledochuszysten sind Strikturen, Steinbildung, Cholangitiden, Pankreatitiden, Rupturen, Entstehung einer sekundären biliären Zirrhose sowie von Malignomen. Ihre Therapie ist deshalb chirurgisch.

  • Das Operationsziel bei Choledochuszysten ist heute, die Zyste in toto zu exzidieren und im Anschluss die Kontinuität durch eine biliodigestive Anastomose wiederherzustellen.

  • PNET werden in funktionell aktive und funktionell inaktive Tumoren unterteilt. Die meisten der Tumoren sind funktionell inaktiv.

  • Die chirurgische Resektion ist die einzige Möglichkeit auf kurative Entfernung des Tumors und um im Falle von funktionell aktiven PNET eine Symptomkontrolle zu erlangen.

  • Die medikamentöse Erstlinientherapie bei PNET besteht aus Somatostatinanaloga.

Schlüsselwörter

gastrointestinale Stromatumoren - Choledochuszysten - pankreatische neuroendokrine Tumoren - Therapie


Publication History

Article published online:
21 February 2022

© 2022. Thieme. All rights reserved.

Georg Thieme Verlag KG
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  • Literatur

  • 1 Mazur MT, Clark HB. Gastric stromal tumors. Reappraisal of histogenesis. Am J Surg Pathol 1983; 7: 507-519
    CrossrefPubMedGoogle Scholar
  • 2 Joensuu H, Hohenberger P, Corless CL. Gastrointestinal stromal tumour. Lancet 2013; 382: 973-983
    CrossrefPubMedGoogle Scholar
  • 3 Reichardt P, Bauer S, Dürr D. et al. Gastrointestinale Stromatumoren (GIST). Onkopedia-Leitlinie. Im Internet (Stand: 24.11.2021): http://www.onkopedia.com/de/onkopedia/guidelines/gastrointestinale-stromatumoren-gist/@@guideline/html/index.html%23ID0EKC%22%3ehttps://%3cDCCnv::EMAIL%3ewww.onkopedia.com/de/onkopedia/guidelines/gastrointestinale-stromatumoren-gist/@@guideline/html/index.html%23ID0EKC
    Google Scholar
  • 4 Demetri GD, Benjamin RS, Blanke CD. et al. NCCN Task Force report: management of patients with gastrointestinal stromal tumor (GIST) – update of the NCCN clinical practice guidelines. J Natl Compr Canc Netw 2007; 5 (Suppl. 02) S1-S29 quiz S30
    CrossrefPubMedGoogle Scholar
  • 5 Joensuu H, Vehtari A, Riihimäki J. et al. Risk of recurrence of gastrointestinal stromal tumour after surgery: an analysis of pooled population-based cohorts. Lancet Oncol 2012; 13: 265-274
    CrossrefPubMedGoogle Scholar
  • 6 Miettinen M, Lasota J. Gastrointestinal stromal tumors: pathology and prognosis at different sites. Semin Diagn Pathol 2006; 23: 70-83
    CrossrefPubMedGoogle Scholar
  • 7 Feng F, Liu Z, Zhang X. et al. Comparison of Endoscopic and Open Resection for Small Gastric Gastrointestinal Stromal Tumor. Transl Oncol 2015; 8: 504-508
    CrossrefPubMedGoogle Scholar
  • 8 Wang C, Gao Z, Shen K. et al. Safety and efficiency of endoscopic resection versus laparoscopic resection in gastric gastrointestinal stromal tumours: A systematic review and meta-analysis. Eur J Surg Oncol 2020; 46: 667-674
    CrossrefPubMedGoogle Scholar
  • 9 Onimaru M, Inoue H, Ikeda H. et al. Combination of laparoscopic and endoscopic approaches for neoplasia with non-exposure technique (CLEAN-NET) for gastric submucosal tumors: updated advantages and limitations. Ann Transl Med 2019; 7: 582
    CrossrefPubMedGoogle Scholar
  • 10 Demetri GD, von Mehren M, Blanke CD. et al. Efficacy and safety of imatinib mesylate in advanced gastrointestinal stromal tumors. N Engl J Med 2002; 347: 472-480
    CrossrefPubMedGoogle Scholar
  • 11 Machairas N, Prodromidou A, Molmenti E. et al. Management of liver metastases from gastrointestinal stromal tumors: where do we stand?. J Gastrointest Oncol 2017; 8: 1100-1108
    CrossrefPubMedGoogle Scholar
  • 12 Oppelt PJ, Hirbe AC, Van Tine BA. Gastrointestinal stromal tumors (GISTs): point mutations matter in management, a review. J Gastrointest Oncol 2017; 8: 466-473
    CrossrefPubMedGoogle Scholar
  • 13 Miettinen M, Makhlouf H, Sobin LH. et al. Gastrointestinal stromal tumors of the jejunum and ileum: a clinicopathologic, immunohistochemical, and molecular genetic study of 906 cases before imatinib with long-term follow-up. Am J Surg Pathol 2006; 30: 477-489
    CrossrefPubMedGoogle Scholar
  • 14 Ronnekleiv-Kelly SM, Soares KC, Ejaz A. et al. Management of choledochal cysts. Curr Opin Gastroenterol 2016; 32: 225-231
    PubMedGoogle Scholar
  • 15 Söreide K, Körner H, Havnen J. et al. Bile duct cysts in adults. Br J Surg 2004; 91: 1538-1548
    CrossrefPubMedGoogle Scholar
  • 16 Cheng SP, Yang TL, Jeng KS. et al. Choledochal cyst in adults: aetiological considerations to intrahepatic involvement. ANZ J Surg 2004; 74: 964-967
    CrossrefPubMedGoogle Scholar
  • 17 Alonso-Lej F, Rever jr. WB, Pessagno DJ. Congenital choledochal cyst, with a report of 2, and an analysis of 94, cases. Int Abstr Surg 1959; 108: 1-30
    PubMedGoogle Scholar
  • 18 Todani T, Watanabe Y, Tabuchi K. et al. Congenital bile duct cysts: Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg 1977; 134: 263-269
    CrossrefPubMedGoogle Scholar
  • 19 Lee SE, Jang JY, Lee YJ. et al. Choledochal cyst and associated malignant tumors in adults: a multicenter survey in South Korea. Arch Surg 2011; 146: 1178-1184
    CrossrefPubMedGoogle Scholar
  • 20 Soares KC, Arnaoutakis DJ, Kamel I. et al. Choledochal cysts: presentation, clinical differentiation, and management. J Am Coll Surg 2014; 219: 1167-1180
    CrossrefPubMedGoogle Scholar
  • 21 Soreide K, Soreide JA. Bile duct cyst as precursor to biliary tract cancer. Ann Surg Oncol 2007; 14: 1200-1211
    CrossrefPubMedGoogle Scholar
  • 22 Xia HT, Yang T, Liang B. et al. Treatment and outcomes of adults with remnant intrapancreatic choledochal cysts. Surgery 2016; 159: 418-425
    CrossrefPubMedGoogle Scholar
  • 23 Edil BH, Cameron JL, Reddy S. et al. Choledochal cyst disease in children and adults: a 30-year single-institution experience. J Am Coll Surg 2008; 206: 1000-1005 discussion 1005–1008
    CrossrefPubMedGoogle Scholar
  • 24 Soares KC, Kim Y, Spolverato G. et al. Presentation and Clinical Outcomes of Choledochal Cysts in Children and Adults: A Multi-institutional Analysis. JAMA Surg 2015; 150: 577-584
    CrossrefPubMedGoogle Scholar
  • 25 Patel N, Benipal B. Incidence of Cholangiocarcinoma in the USA from 2001 to 2015: A US Cancer Statistics Analysis of 50 States. Cureus 2019; 11: e3962
    PubMedGoogle Scholar
  • 26 Perri G, Prakash LR, Katz MHG. Pancreatic neuroendocrine tumors. Curr Opin Gastroenterol 2019; 35: 468-477
    CrossrefPubMedGoogle Scholar
  • 27 Ben Q, Zhong J, Fei J. et al. Risk Factors for Sporadic Pancreatic Neuroendocrine Tumors: A Case-Control Study. Sci Rep 2016; 6: 36073
    CrossrefPubMedGoogle Scholar
  • 28 Giraldi L, Vecchioni A, Carioli G. et al. Risk factors for pancreas and lung neuroendocrine neoplasms: a case-control study. Endocrine 2021; 71: 233-241
    CrossrefPubMedGoogle Scholar
  • 29 Falconi M, Eriksson B, Kaltsas G. et al. ENETS Consensus Guidelines Update for the Management of Patients with Functional Pancreatic Neuroendocrine Tumors and Non-Functional Pancreatic Neuroendocrine Tumors. Neuroendocrinology 2016; 103: 153-171
    CrossrefPubMedGoogle Scholar
  • 30 Worhunsky DJ, Krampitz GW, Poullos PD. et al. Pancreatic neuroendocrine tumours: hypoenhancement on arterial phase computed tomography predicts biological aggressiveness. HPB (Oxford) 2014; 16: 304-311
    CrossrefPubMedGoogle Scholar
  • 31 Hashim YM, Trinkaus KM, Linehan DC. et al. Regional lymphadenectomy is indicated in the surgical treatment of pancreatic neuroendocrine tumors (PNETs). Ann Surg 2014; 259: 197-203
    CrossrefPubMedGoogle Scholar
  • 32 Kuo EJ, Salem RR. Population-level analysis of pancreatic neuroendocrine tumors 2 cm or less in size. Ann Surg Oncol 2013; 20: 2815-2821
    CrossrefPubMedGoogle Scholar
  • 33 Galleberg RB, Knigge U, Janson ET. et al. Results after surgical treatment of liver metastases in patients with high-grade gastroenteropancreatic neuroendocrine carcinomas. Eur J Surg Oncol 2017; 43: 1682-1689
    CrossrefPubMedGoogle Scholar
  • 34 Pavel M, OʼToole D, Costa F. et al. ENETS Consensus Guidelines Update for the Management of Distant Metastatic Disease of Intestinal, Pancreatic, Bronchial Neuroendocrine Neoplasms (NEN) and NEN of Unknown Primary Site. Neuroendocrinology 2016; 103: 172-185
    CrossrefPubMedGoogle Scholar
  • 35 Kennedy AS. Hepatic-directed Therapies in Patients with Neuroendocrine Tumors. Hematol Oncol Clin North Am 2016; 30: 193-207
    CrossrefPubMedGoogle Scholar
  • 36 Partelli S, Bartsch DK, Capdevila J. et al. ENETS Consensus Guidelines for Standard of Care in Neuroendocrine Tumours: Surgery for Small Intestinal and Pancreatic Neuroendocrine Tumours. Neuroendocrinology 2017; 105: 255-265
    CrossrefPubMedGoogle Scholar
  • 37 Garcia-Carbonero R, Sorbye H, Baudin E. et al. ENETS Consensus Guidelines for High-Grade Gastroenteropancreatic Neuroendocrine Tumors and Neuroendocrine Carcinomas. Neuroendocrinology 2016; 103: 186-194
    CrossrefPubMedGoogle Scholar
  • 38 Dasari A, Shen C. Halperin. et al. Trends in the Incidence, Prevalence, and Survival Outcomes in Patients With Neuroendocrine Tumors in the United States. JAMA Oncol 2017; 3: 1335-1342
    CrossrefPubMedGoogle Scholar
  • 39 Strosberg JR, Cheema A, Weber J. et al. Prognostic validity of a novel American Joint Committee on Cancer Staging Classification for pancreatic neuroendocrine tumors. J Clin Oncol 2011; 29: 3044-3049
    CrossrefPubMedGoogle Scholar
  • 40 Landoni L, Marchegiani G, Pollini T. et al. The Evolution of Surgical Strategies for Pancreatic Neuroendocrine Tumors (Pan-NENs): Time-trend and Outcome Analysis From 587 Consecutive Resections at a High-volume Institution. Ann Surg 2019; 269: 725-732
    CrossrefPubMedGoogle Scholar
  • 41 Jensen RT, Cadio G, Brandi ML. et al. ENETS Consensus Guidelines for the management of patients with digestive neuroendocrine neoplasms: functional pancreatic endocrine tumor syndromes. Neuroendocrinology 2012; 95: 98-119
    CrossrefPubMedGoogle Scholar
  • 42 Norton JA, Foster DS, Ito T. et al. Gastrinomas: Medical or Surgical Treatment. Endocrinol Metab Clin North Am 2018; 47: 577-601
    CrossrefPubMedGoogle Scholar
  • 43 Norton JA, Fraker DL, Alexander HR. et al. Value of surgery in patients with negative imaging and sporadic Zollinger-Ellison syndrome. Ann Surg 2012; 256: 509-517
    CrossrefPubMedGoogle Scholar
  • 44 Norton JA, Foster DS, Blumgart LH. et al. Incidence and Prognosis of Primary Gastrinomas in the Hepatobiliary Tract. JAMA Surg 2018; 153: e175083
    CrossrefPubMedGoogle Scholar