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Current Management of Gastroduodenal N euroendocrine T umors

Current Management of Gastroduodenal N euroendocrine T umors. Dr Lo Hau Ching Michelle Tuen Mun Hospital Joint Hospital Surgical Grand Round 14 Jan 2017. Presentation Outline. Epidemiology Clinical presentation Classification Endoscopic features Other investigations Treatment options

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Current Management of Gastroduodenal N euroendocrine T umors

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  1. Current Management of Gastroduodenal Neuroendocrine Tumors Dr Lo Hau Ching Michelle Tuen Mun Hospital Joint Hospital Surgical Grand Round 14 Jan 2017

  2. Presentation Outline • Epidemiology • Clinical presentation • Classification • Endoscopic features • Other investigations • Treatment options • Follow up strategy

  3. Presentation Outline • Epidemiology • Clinical presentation • Classification • Endoscopic features • Other investigations • Treatment options • Follow up strategy

  4. What is a neuroendocrine tumor (NET)? • First labelled as “carcinoid” tumor by German pathologist Siegfried Oberndorfer in 1907 • A heterogenous group of neoplasms arising from the neuroendocrine system • Generally characterized by their ability to produce functional hormones or polypeptides • Leading to a wide variety of clinical presentation Hallet, et al. "Exploring the rising incidence of neuroendocrine tumors: A population‐based analysis of epidemiology, metastatic presentation, and outcomes." Cancer 121.4 (2015): 589-597. Delle Fave, et al. "ENETS consensus guidelines for the management of patients with gastroduodenal neoplasms." Neuroendocrinology 95.2 (2011): 74-87. Sato, Yuichi, et al. "Management of gastric and duodenal neuroendocrine tumors." World Journal of Gastroenterology 22.30 (2016): 6817.

  5. What is a neuroendocrine tumor (NET)? • More than 50% of neuroendocrine tumor arises from gastrointestinal tract • Overall incidence • Gastric NET: • 0.33/100000 (US data) • 1.67/100000 (Japan data) • 6% of all neuroendocrine tumors • Duodenal NET: • 0.19/100000 (US data) • 0.17/100000 (Japan data) • 3.8% of all neuroendocrine tumors Hallet, et al. "Exploring the rising incidence of neuroendocrine tumors: A population‐based analysis of epidemiology, metastatic presentation, and outcomes." Cancer 121.4 (2015): 589-597. Sato, Yuichi, et al. "Management of gastric and duodenal neuroendocrine tumors." World Journal of Gastroenterology 22.30 (2016): 6817. Yao, James C., et al. "One hundred years after “carcinoid”: epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States." Journal of Clinical Oncology 26.18 (2008): 3063-3072.

  6. What is a neuroendocrine tumor (NET)? • Increasing incidence worldwide resulting from widespread use of endoscopy and increasing awareness among clinicians and pathologists Tsai, Hui-Jen, et al. "The epidemiology of neuroendocrine tumors in Taiwan: a nation-wide cancer registry-based study." PLoS One 8.4 (2013): e62487.

  7. Presentation Outline • Epidemiology • Clinical presentation • Classification • Endoscopic features • Other investigations • Treatment options • Follow up strategy

  8. Clinical presentation • Anemia (72%) • GI bleeding (32%) • Abdominal pain (69%) • Carcinoid syndrome (11%): diarrhea, flushing, wheeze • Serotonin released by NET into portal circulation is metabolized by the liver • Carcinoid syndrome seldom occurs in primary GI-NET (unless in extensive liver metastasis) and it is more common in extra-GI NET (such as bronchial NET) • Associated autoimmune abnormalities • Atrophic gastritis (67%) • Pernicious anemia (58%) • Not uncommonly, as incidental finding on endoscopic examination or upon histological assessment Gough, David B., et al. "Diverse clinical and pathologic features of gastric carcinoid and the relevance of hypergastrinemia." World journal of surgery 18.4 (1994): 473-479. Hoffmann, K. Martin, Masayuki Furukawa, and Robert T. Jensen. "Duodenal neuroendocrine tumors: classification, functional syndromes, diagnosis and medical treatment." Best Practice & Research Clinical Gastroenterology 19.5 (2005): 675-697. Jayasena, Channa N., and Waljit S. Dhillo. "Carcinoid syndrome and neuroendocrine tumours." Medicine 41.10 (2013): 566-569.

  9. Presentation Outline • Epidemiology • Clinical presentation • Classification • Endoscopic features • Other investigations • Treatment options • Follow up strategy

  10. WHO classification of NET (2010) • Chan, David L., et al. ”Neuroendocrine tumors: models for rare tumour management.” Cancer Forum March 2015 Vol 39 Issue No 1.

  11. classification of gastric NET • DelleFave, G., et al. "ENETS consensus guidelines update for gastroduodenalneuroendocrineneoplasms." Neuroendocrinology 103.2 (2016): 119-124.

  12. Types of duodenal NET • Important to identify possible association with hormonal or hereditary syndromes (e.g. MEN) as this would guide further investigation or treatment • Gastrinoma (60%) • 15-30% clinical Zollinger Ellison Syndrome • Rest of the gastrinoma are clinically silent • Most gastrinoma are found in “gastrinoma triangle” • Somatostatin rich NET (15-20%) • Gangliocytic paraganglioma • Duodenal neuroendocrine carcinoma

  13. Presentation Outline • Epidemiology • Clinical presentation • Classification • Endoscopic features • Other investigations • Treatment options • Follow up strategy

  14. Endoscopic features – gastric NET • Conventional endoscopy with white light imaging • Smooth hemispherical submucosal lesions • Polypoid lesions • Look for presence of background atrophic gastritis • Magnifying endoscopy with narrow band imaging (NBI) • Gastric mucosal pit structures preserved • Endoscopic ultrasonography (EUS) • Hypoechoic structure arising from submucosal layer Sato, Yuichi, et al. "Management of gastric and duodenal neuroendocrine tumors." World Journal of Gastroenterology 22.30 (2016): 6817.

  15. Endoscopic features – duodenal NET • Conventional endoscopy with white light imaging • Smooth hemispherical submucosal lesions • Polypoid lesions • Central depression or crater as tumor grows • Magnifying endoscopy with narrow band imaging (NBI) • Duodenal mucosal pit structures preserved • Pit structures may be absent and replaced by cyan coloured, corkscrew shaped capillaries over the central depression • Endoscopic ultrasonography (EUS) • Hypoechoic structure arising from submucosal layer Sato, Yuichi, et al. "Management of gastric and duodenal neuroendocrine tumors." World Journal of Gastroenterology 22.30 (2016): 6817.

  16. EUS features of gastroduodenal NET 1. Chak, Amitabh. "EUS in submucosal tumors." Gastrointestinal endoscopy 56.4 (2002): S43-S48.

  17. Presentation Outline • Epidemiology • Clinical presentation • Classification • Endoscopic features • Other investigations • Treatment options • Follow up strategy

  18. Radiological assessment • Conventional imaging • CT / MRI • Limited value for small type 1 and 2 gastric NET and duodenal NET in terms of cost-benefit ratio • PET with 18-FDG (fluorodeoxyglucose) • May identify high grade neuroendocrine tumors as they are more metabolically active • Somatostatin receptor imaging • Octretide scan (OctreoScan) • PET with 68-Gallium labelled tracers (-DOTATOC, -DOTANOC, DOTATATE) Bushnell, et al. "Standard imaging techniques for neuroendocrine tumors." Endocrinology and metabolism clinics of North America 40.1 (2011): 153-162. Johnbeck, et al. "PET tracers for somatostatin receptor imaging of neuroendocrine tumors: current status and review of the literature." Future Oncology 10.14 (2014): 2259-2277. Maxwell JE, Howe JR. Imaging in neuroendocrine tumors: an update for the clinician. International journal of endocrine oncology. 2015;2(2):159-168. doi:10.2217/ije.14.40.

  19. Biochemical assessment • Chromogranin A (CgA) • Elevated in 60-100% of NET • Produced by chromaffin cells of the adrenal medulla, paraganglia, enterochromaffin-like cells and beta cells of pancreas • Serum level reflects tumor load • Serial measurement as the most important parameter for monitoring of disease status • 24hr urine 5-hydroxyindolacetic acid (5-HIAA) • Serotonin metabolite excreted in urine • Elevated levels generally imply the presence of liver metastasis Vinik AI, Woltering EA, Warner RR et al. NANETS consensus guidelines for the diagnosis of neuroendocrine tumor. Pancreas. 2010;39:713-734.

  20. Biochemical assessment • Measurement of gut hormones • Gastrin – gastrinoma • Insulin – insulinoma • Other biochemical screening tests for MEN type 1 • Serum calcium • Parathyroid hormone • Prolactin • Insulin-like growth factor-1 (IGF-1) Vinik AI, Woltering EA, Warner RR et al. NANETS consensus guidelines for the diagnosis of neuroendocrine tumor. Pancreas. 2010;39:713-734.

  21. Presentation Outline • Epidemiology • Clinical presentation • Classification • Endoscopic features • Other investigations • Treatment options • Follow up strategy

  22. How to treat Gastroduodenal NET?

  23. Treatment of type 1 gastric NET • Endoscopic resection by means of EMR/ESD • Tumor size ≥ 10mm • Wedge excision or partial gastrectomy • Positive resection margin • T2 lesions (invades muscularis propria) or above • Somatostatin analogues (SSA) • No study comparing the use of SSA vs surveillance • Maybe useful in treatment multiple small lesions that are hard to eradicate on OGD • Netazepide • Gastrin receptor anatgonist • Antiproliferative properties in non-controlled studies

  24. Treatment of type 2 gastric NET • Treatment is usually dictated by possible presence of duodenal or pancreatic NET as part of MEN-1 • Local or limited excision is recommended • Use of Netazepide for type 2 NET is under trial

  25. Treatment of type 3 gastric NET • Follow the same management strategy as in gastric adenocarcinoma • Surgical treatment is recommended if feasible • Partial or total gastrectomy with LN dissection • Systemic therapies for inoperable or stage 4 disease

  26. Treatment of duodenal NET D-NETs: Duodenal neuroendocrine tumors; EUS: Endoscopic ultrasound; CT: Computed tomography; N+: Lymph node metastasis; M+: Distant metastasis; SRS: Somatostatin receptor scintigraphy; PRRT: Peptide receptor radionuclide therapy; SSA: Somatostatin analogues; CHT: Chemotherapy.

  27. Presentation Outline • Epidemiology • Classification • Clinical presentation • Endoscopic features • Other investigations • Treatment options • Follow up strategy

  28. Surveillance for gastroduodenal NET • Regular follow up is recommended but the exact time interval for surveillance endoscopy has not been well defined • In general, patients are recommended to repeat OGD at least every 2 years

  29. Bring home messages • Gastroduodenal neuroendocrine tumors is a rare disease entity • Endoscopic and relevant hormonal workup are essential • Endoscopic and surgical resection remains the cornerstone of therapy • Importance of regular follow up • Surveillance OGD • Monitoring of biochemical marker: chromogranin A

  30. Thank you

  31. Diagnosis algorithm of gastric NET 1. Tan, Huangying. "Advances in the diagnosis and treatment of gastric neuroendocrine neoplasms." Translational Gastroenterology and Hepatology 1.9 (2016).

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