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Z Gastroenterol 2013; 51(11): 1240-1250
DOI: 10.1055/s-0033-1335461
Originalarbeit
© Georg Thieme Verlag KG Stuttgart · New York

Nicht-funktionelle duodenale neuroendokrine Neoplasien im proximalen Duodenum – Fallserie und Vorschlag für ein „High-Risk-/Low-Risk-Konzept“ zur Entscheidung für eine lokale endoskopische Therapie

Non-Functional Duodenal Neuroendocrine Neoplasia in the Proximal Duodenum – Case Reports and Proposal for a “High-Risk-/Low-Risk-Concept” in the Decision for Local Endoscopic Therapy
F. Scheerer
,
W. Schmitt
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Publikationsverlauf

19. Oktober 2012

15. April 2013

Publikationsdatum:
15. November 2013 (online)

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Zusammenfassung

Frühe duodenale neuroendokrine Neoplasien (dNEN) werden vermehrt diagnostiziert. Im Bulbus lokalisierte nicht-funktionelle dNEN, welche Gastrin exprimieren, sind die mit Abstand häufigste Entität. Im Zeitraum zwischen 2004 und 2012 wurden 17 Fälle von 16 Patienten mit NEN im Bulbus duodeni ausgewertet. Ampullär bzw. periampullär gelegene und funktionelle dNEN/Gastrinome wurden aufgrund eines möglichen unterschiedlichen malignen Potenzials nicht berücksichtigt. Das Durchschnittsalter der Patienten lag bei 65,7 Jahren, die mittlere Tumorgröße bei 10,2 mm, der Proliferationsindex Ki67 maximal bei 5 % (NET G2). Zumeist zeigte sich eine maximale Tiefeninvasion bis in die Submucosa. Bei dNEN ohne Risikofaktoren (Größe bis max. 10 mm, G1-Situation, keine Invasion der Muscularis propria, keine Angioinvasion) war in 10 von 11 Fällen (90,9 %) die endoskopische Therapie ausreichend. Bei bestehenden Riskofaktoren war lediglich in einem von 5 Fällen eine alleinige endoskopische Therapie möglich. Bei Fehlen von Risikofaktoren konnte im bisherigen Nachbeobachtungszeitraum (im Mittel 36,7 Monate) keine lymphonoduläre Metastasierung festgestellt werden. Allerdings zeigte sich bei Vorhandensein von Risikofaktoren bzw. vorliegender Operationsindikation ein deutlicher Anstieg der Rate von Lymphknotenmetastasen. Die eigenen Daten weisen darauf hin, dass bei frühen nichtfunktionellen dNEN im Bulbus mit Vorhandensein einer G2-Situation oder einer Tumorgröße > 10 mm oder Infiltration der Muscularis propria, die Notwendigkeit einer operativen Versorgung deutlich ansteigt. Ein High-Risk-/Low-Risk-Konzept für die endoskopische Therapie früher nicht-funktioneller dNEN wird etabliert.

Abstract

Early duodenal neuroendocrine neoplasms (dNENs) are being increasingly diagnosed. Non-functional dNENs in the bulb expressing gastrin are by far the most frequent entity. In the period from 2004 to 2012, 17 cases of 16 patients with NET in the duodenal bulb were evaluated. dNENs of the ampulla of Vater and functional dNEN/gastrinoma were not included due to possibly different malignant potentials. The average age of the patients was 65.7 years, the mean tumour size was 10.2 mm, the maximum proliferation index Ki 67 was 5 % (NET G2). In most cases the maximum depth of invasion was down to the submucosa. In cases of dNEN without risk factors (size up to 10 mm, G1 situation, no invasion of the muscularis propria, no angioinvasion) in 10 out of 11 cases (90.9 %), endoscopic therapy was sufficient. In cases of existing risk factors, sole endoscopic treatment was only possible in 1 out of 5 cases (20 %). In the absence of risk factors in the current follow-up period (mean: 36.7 months) no lymph node metastases were detected. In the presence of risk factors or indications for surgery we found an increase in the rate of lymph node metastases. Our own data indicate that in case of a G2 situation, a tumour size >10 mm or infiltration of the muscularis propria the need for surgical treatment increases significantly for early non-functional dNENs in the duodenal bulb. A high-risk-/low-risk-concept for the endoscopic therapy for early non-functional dNEN has been established.

Schlüsselwörter

neuroendokriner Tumor - NEN - Duodenum - gastro-entero-pankreatische Tumoren - Gastrinom - MEN

Key words

neuroendocrine tumours - NEN - duodenum - gastroenteropancreatic tumours - gastrinoma - MEN
 

  • Literatur

  • 1 Delle F, Kwekkeboom DJ, Van C et al. ENETS Consensus Guidelines for the management of patients with gastroduodenal neoplasms. Neuroendocrinology 2012; 95: 74-87
    CrossrefPubMedGoogle Scholar
  • 2 Begum N, Maasberg S, Plockinger U et al. Neuroendocrine Tumours of the GI Tract – Data from the German NET Registry. Zentralbl Chir 2012; 137: e1
    Artikel in Thieme ConnectPubMedGoogle Scholar
  • 3 Bilimoria KY, Bentrem DJ, Wayne JD et al. Small bowel cancer in the United States: changes in epidemiology, treatment, and survival over the last 20 years. Annals of Surgery 2009; 249: 63-71
    CrossrefPubMedGoogle Scholar
  • 4 Hoffmann KM, Furukawa M, Jensen RT. Duodenal neuroendocrine tumors: Classification, functional syndromes, diagnosis and medical treatment. Best Practice & Research in Clinical Gastroenterology 2005; 19: 675-697
    PubMedGoogle Scholar
  • 5 Hatzitheoklitos E, Buchler MW, Friess H et al. Carcinoid of the ampulla of Vater. Clinical characteristics and morphologic features. Cancer 1994; 73: 1580-1588
    CrossrefPubMedGoogle Scholar
  • 6 Jensen RT, Niederle B, Mitry E et al. Gastrinoma (duodenal and pancreatic). Neuroendocrinology 2006; 84: 173-182
    CrossrefPubMedGoogle Scholar
  • 7 Klöppel G. Classification and pathology of gastroenteropancreatic neuroendocrine neoplasms. Endocrine Related Cancer 2011; 18: S1-S16
    CrossrefPubMedGoogle Scholar
  • 8 Garbrecht N, Anlauf M, Schmitt A et al. Somatostatin-producing neuroendocrine tumors of the duodenum and pancreas: incidence, types, biological behavior, association with inherited syndromes, and functional activity. Endocrine Related Cancer 2008; 15: 229-241
    CrossrefPubMedGoogle Scholar
  • 9 Hartel M, Wente MN, Sido B et al. Carcinoid of the ampulla of Vater. Journal of Gastroenterology & Hepatology 2005; 20: 676-681
    CrossrefPubMedGoogle Scholar
  • 10 Burke AP, Helwig EB. Gangliocytic paraganglioma. American Journal of Clinical Pathology 1989; 92: 1-9
    PubMedGoogle Scholar
  • 11 Williams ED, Sandler M. The classification of carcinoid tumours. Lancet 1963; 1: 238-239
    PubMedGoogle Scholar
  • 12 Schott M, Klöppel G, Raffel A et al. Neuroendocrine neoplasms of the gastrointestinal tract. Deutsches Ärzteblatt International 2011; 108: 305-312
    PubMedGoogle Scholar
  • 13 Ito T, Tanaka M, Sasano H et al. Preliminary results of a Japanese nationwide survey of neuroendocrine gastrointestinal tumors. Journal of Gastroenterology 2007; 42: 497-500
    CrossrefPubMedGoogle Scholar
  • 14 Witzigmann H, Loracher C, Geissler F et al. Neuroendocrine tumours of the duodenum. Clinical aspects, pathomorphology and therapy. Langenbecks Archives of Surgery 2002; 386: 525-533
    CrossrefPubMedGoogle Scholar
  • 15 Eriksson B, Klöppel G, Krenning E et al. Consensus guidelines for the management of patients with digestive neuroendocrine tumors – well-differentiated jejunal-ileal tumor/carcinoma. Neuroendocrinology 2008; 87: 8-19
    CrossrefPubMedGoogle Scholar
  • 16 Scherubl H. Options for gastroenteropancreatic neuroendocrine tumours. Lancet Oncology 2008; 9: 203
    CrossrefPubMedGoogle Scholar
  • 17 Zimmer T, Scherubl H, Faiss S et al. Endoscopic ultrasonography of neuroendocrine tumours. Digestion 2000; 62: 45-50
    CrossrefPubMedGoogle Scholar
  • 18 Scherubl H, Faiss S, Zeitz M. Neuroendocrine tumors of the gastrointestinal tract--diagnosis and therapy. Deutsche Medizinische Wochenschrift 2003; 128: S81-S83
    PubMedGoogle Scholar
  • 19 Dietrich CF, Schreiber DietrichD, Schuessler G et al. Contrast enhanced ultrasound of the liver – state of the art. Deutsche Medizinische Wochenschrift 2007; 132: 1225-1231
    Artikel in Thieme ConnectPubMedGoogle Scholar
  • 20 Mork H, Ignee A, Schuessler G et al. Analysis of neuroendocrine tumour metastases in the liver using contrast enhanced ultrasonography. Scandinavian Journal of Gastroenterology 2007; 42: 652-662
    CrossrefPubMedGoogle Scholar
  • 21 Paulson EK, McDermott VG, Keogan MT et al. Carcinoid metastases to the liver: role of triple-phase helical CT. Radiology 1998; 206: 143-150
    PubMedGoogle Scholar
  • 22 Dromain C, de Baere T, Baudin E et al. MR imaging of hepatic metastases caused by neuroendocrine tumors: comparing four techniques. American Journal of Roentgenology 2003; 180: 121-128
    CrossrefPubMedGoogle Scholar
  • 23 Modlin IM, Champaneria MC, Chan AK et al. A three-decade analysis of 3,911 small intestinal neuroendocrine tumors: the rapid pace of no progress. American Journal of Gastroenterology 2007; 102: 1464-1473
    CrossrefPubMedGoogle Scholar
  • 24 Maasberg S, Denecke T, Pascher A et al. Neuroendokrine Neoplasien des gastroenteropankreatischen Systems. Gastroenterologe 2012; 7: 139-155
    CrossrefPubMedGoogle Scholar
  • 25 Oberg K, Knigge U, Kwekkeboom D et al. Neuroendocrine gastro-entero-pancreatic tumors: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up +. Annals of Oncology 2012; 23: vii124-vii130
    CrossrefPubMedGoogle Scholar
  • 26 Soga J. Early-stage carcinoids of the gastrointestinal tract: an analysis of 1914 reported cases. Cancer 2005; 103: 1587-1595
    CrossrefPubMedGoogle Scholar
  • 27 Merchant SH, VanderJagt T, Lathrop S et al. Sporadic duodenal bulb gastrin-cell tumors: association with Helicobacter pylori gastritis and long-term use of proton pump inhibitors. American Journal of Surgical Pathology 2006; 30: 1581-1587
    CrossrefPubMedGoogle Scholar
  • 28 Mullen JT, Wang H, Yao JC et al. Carcinoid tumors of the duodenum. Surgery 2005; 138: 971-977
    CrossrefPubMedGoogle Scholar
  • 29 Capella C. Endocrine tumors of the small intestine. World Health Organisation Classification of Tumors. Pathology and Genetics of. Tumours of the Digestive System 2000; 77-82
    PubMedGoogle Scholar
  • 30 Schmiegel W, Reinacher SchickA, Arnold D et al. Update S3-guideline “colorectal cancer” 2008. Zeitschrift für Gastroenterologie 2008; 46: 799-840
    Artikel in Thieme ConnectPubMedGoogle Scholar
  • 31 Solcia E, Capella C, Fiocca R, Sessa L, LaRosa S, Rindi G. Disorders of the endocrine system. In: Ming SC Goldman H, (ed). Pathology of the gastrointestinal tract, 2nd edn. Baltimore: Williams and Wilkins; 1998: 295-322
    Google Scholar
  • 32 Scherubl H, Schwertner C, Steinberg J et al. Neuroendocrine tumors of the small bowels are on the rise: early tumors and their management. Zeitschrift für Gastroenterologie 2010; 48: 406-413
    Artikel in Thieme ConnectPubMedGoogle Scholar
  • 33 Soga J. Endocrinocarcinomas (carcinoids and their variants) of the duodenum. An evaluation of 927 cases. Journal of Experimental & Clinical Cancer Research 2003; 22: 349-363
    PubMedGoogle Scholar
  • 34 Miederer M, Weber MM, Fottner C. Molecular imaging of gastroenteropancreatic neuroendocrine tumors. Gastroenterology Clinics of North America 2010; 39: 923-935
    CrossrefPubMedGoogle Scholar
  • 35 Rindi G, Klöppel G, Alhman H et al. TNM staging of foregut (neuro)endocrine tumors: a consensus proposal including a grading system. Virchows Archiv 2006; 449: 395-401
    CrossrefPubMedGoogle Scholar
  • 36 Nilsson O, Van C, Delle F et al. Poorly differentiated carcinomas of the foregut (gastric, duodenal and pancreatic). Neuroendocrinology 2006; 84: 212-215
    CrossrefPubMedGoogle Scholar
  • 37 Nassar HMD, Albores SaavedraJMD, Klimstra DSM. High-Grade Neuroendocrine Carcinoma of the Ampulla of Vater: A Clinicopathologic and Immunohistochemical Analysis of 14 Cases. American Journal of Surgical Pathology 2005; 29: 588-594
    CrossrefPubMedGoogle Scholar