Endocrine tumors of the gastrointestinal tract

1. Endocrine tumors of the gastrointestinal tract Dr n med. JacekPytel

2. Endocrine tumors of the gastrointestinal tract • In 1902 secretin was discovered by Bayliss and Starling • Among the cells covering the gastrointestinaltract are single cells with characteristic typical endocrine cellsThey create a kind of "diffuse"endocrine organ - scattered intraepithelial endocrine glands

3. Endocrine tumors of the gastrointestinal tract • Thanks to cytochemical- imunohistochemistry- and fluorescent reactions presence of these glands in the epithelium were found ingastrointestinal tract and in the islands of Langerhans of pancreas, adrenal medulla,bronchial epithelium, fallopian tubes, vagina and uterus

4. Endocrine tumors of the gastrointestinal tract • These cells are characterized by the ability to capture and metabolize biogenic amines and their precursors, and synthesizing small molecule and peptide hormone secretion

5. Endocrine tumors of the gastrointestinal tract • Properties:Amine precursor uptake and decarboxylation    APUD

6. Endocrine tumors of the gastrointestinal tract • APUD system cells origin fromneuroectoderm and migrate to the mucosaof primitive gut and the buds of future organ in embryonic development

7. Endocrine tumors of the gastrointestinal tract • This same origin as the cells of the nervous system, chemoreceptors cells, adrenal medulla cells

8. Endocrine tumors of the gastrointestinal tract • Digestive tract there are a lot of active peptidesgastrin, secretin, cholecystokinin-pancreozymin (CCK-PZ) - classified as hormones - regulate physiological functions of the gastrointestinal tract

9. Endocrine tumors of the gastrointestinal tract • The epithelial cells of gastrointestinal tract and pancreatic endocrine function displaying can develop cancer of a APUDomaThey can be „silent” or produce characteristic symptoms of hormone secretion

10. Endocrine tumors of the gastrointestinal tract • The incidence 5-20 cases per million population per year • In about 70% of patients have the syndrome of multiple endocrine tumors type I(Multiple Endocrine Neoplasia - MEN) • Hormonally inactive tumors - significantlymore

11. Endocrine tumors of the gastrointestinal tract • 3% of pancreatic cancers - neuroendocrine tumors (ca. 76% - hormonally inactive) • Resective in approximately 76% (pancreatic cancer 19%) • 30-50% located outside the pancreas • About 50% - malignant

12. Endocrine tumors of the gastrointestinal tract • Treatment aimed at inhibiting grow tumor and reduce the excessive production of hormones • Somatostatin analogs effectively control the secretion of hormones • The therapeutic target for tumor treatment

13. Endocrine tumors of the gastrointestinal tract • 80-90% of neuroendocrine tumors hassomatostatin receptorSomatostatin analogues (octreotide, lanreotide, RC-160)-for treatment,Diagnostics - marked octreotide - toscyntygraphy

14. Endocrine tumors of the gastrointestinal tract • Endocrine tumors serotonin capture(precursor 5-hydroxytryptophan)PET (positron emission tomography - PET)for locating small changesundetectable by scyntygraphy use octreotide

15. Endocrine tumors of the gastrointestinal tract Diagnostics • Angiography of the celiac trunk • USG • Computed tomography CT • MRI Magnetic resonance tomography • Scyntygraphy (isotope indium-labeled octreotide (In111) • Endoscopic Ultrasonography - EUS

16. Endocrine tumors of the gastrointestinal tract Diagnostics: • Arterial stimulation venossus sampling ASVS • Transhepatic portal venosus sampling THPVS • Intraoperative usltrasonography • Intraoperative hormone measure

17. Endocrine tumors of the gastrointestinal tract • 20-60% insulinomas fails to specify the position before the operation10-20% intraoperatively (experienced surgeon - 90% of the palpable sense)

18. Endocrine tumors of the gastrointestinal tract • Treatment:chemotherapy: streptozotocin and 5 fluorouracil, doxorubicin, cispaltyna (partial remission for about two years 40-60%)Interferon alpha - 50% biochemical response, tumor reduction 20%Somatostatin analogues (octreotide), symptomatic and biochemical response 35-75%

19. Endocrine tumors of the gastrointestinal tract • Treatment: There is no uniform pattern of diagnostic and therapeutic- Removal of the tumor or tumors- Liver metastases - arterial embolization,liver transplant!

20. Endocrine tumors of the gastrointestinal tract • treatment:  - Radiation therapy - tumors generally resistant to radiotherapy  - Isotope-labeled somatostatin analogue itrium (Y90)

21. Insulinoma • 1869-Langerhans - described the pancreatic insulas

22. Insulinoma • 1902 - Nicholls described a pancreatic endocrine tumor cells • The incidence of approx. 0.5-5 / 1 million persons / year • The most common tumor causing hypoglycemia • Other tumors - beyond the pancreas - insulin-secreting growth factors (IGF-insulin-like growth factors)

23. Insulinoma • 60% of all pancreatic insulomasTumor diameter 5-60 mm aprx. 20 mm 90% of cases does not exceed 20 mm10% of multiple endocrine syndrome (MEN 1)10-15% of malignant

24. Insulinoma Clinical symptoms: Whipple’ triad • occurrence of spontaneous hypoglycemia • low blood glucose levels below 50 mg% • regression of hypoglycemia after oral or intravenous administration of glucose

25. Insulinoma • Treatment of choice - surgery

26. Insulinoma • Approximately 70% of patients after surgery recover • Perioperative mortality rate is 10% • In the case of malignant changes in the average survival time is about 2 years

27. Insulinoma Ultrasonografia przedoperacyjna

28. Insulinoma Ultrasonografia przedoperacyjna Tomografia komputerowa

29. Insulinoma Tomografia komputerowa – guz w głowie trzustki

30. Insulinoma Ultrasonografia śródoperacyjna Guz o średnicy 13 mm na pograniczu głowy i trzonu trzustki

31. Insulinoma Ultrasonografia śródoperacyjna Dobrze odgraniczony guz o średnicy 10 mm w okolicy wyrostka haczykowatego

32. Insulinoma Złośliwy guz naciekający śledzionę, liczne przerzuty w wątrobie

33. Insulinoma Mały guz ostro odgraniczony od miąższu trzustki

34. Insulinoma Mały guz trzustki – 21 mm

35. Insulinoma Ultrasonografia endoskopowa

36. Insulinoma Ultrasonografia endoskopowa

37. Insulinoma Laparoskopia

38. Gastrinoma • Gastrinoma - a tumor, which causes gastrin releasing hypergastrinaemia and increased gastric acid secretionTumors: ovarian cancer, bronchial carcinoid may secrete gastrin, but in such quantity that do not cause hypergastrinaemia

39. Gastrinoma • In1955 Zollinger and Ellison described a syndrome: • - Persistent, recurrent ulcers stomach or duodenum, even after partial gastric resection and vagotomy • - Excessive basal secretion of gastric acid    • - The presence of pancreatic cancer island       cells derived from D

40. Gastrinoma • Frequency of gastrinoma 0.5-10 / 1mln/per year • About 0.1% of patients with pepticulcerdisease

41. Gastrinoma • Excessive production of gastrin by tumors derived from not beta-cell (G cells) pancreatic islet • 70% malignat tumors • In 20% of single tumor • Often develop slowly and multifocuses • Metastasis in to lymph nodes and liver but rarely to the lung. • In about 20% occur in MEN I syndrome

42. Gastrinoma Zolliner Ellison Syndrom: -The presence of multiple peptic ulcers in the esophagus, stomach or duodenum -Hipersecretion gastrc acid -The presence of pancreatic tumor secreting gastrin

43. Gastrinoma Symptoms: • Abdominal pain associated with active peptic ulceration or perforation in the course of morbus ulcerosus • Diarrhea caused by excessive production of gastric juice • Tarry stools due to bleeding from the upper gastrointestinal tract

44. Gastrinoma The symptoms and laboratory results gives rise to a suspicion of ZES: -Recurrent peptic ulcer disease resistant to treatment -Basal secretion of gastric juice above 15 mmol HCl /h -Ratio of primary to a maximum of gastric acid secretion (BAO: MAO) does not exceed 0.6 -Gastrin concentration in serum above 500 pg / ml (normal up to 200 pg / ml) - In tests with intravenous administration calcium, glucagon, or secretin in patients with ZES showed an increase in gastrin

45. Inhibition of gastric acid secretion with high acidity by surgery Tumor resection Total gastric resection Not always possible Multifocus changes Ectopic placement metastases Gastrinoma Treatment The basic goal

46. Malignant neoplasms in many cases - may grow slowly Survival can be long Gastrinoma Prognosis In the case of ZES good provided hypersecretion and eradicate gastric hyperacidity

47. Gastrinoma

48. Vipoma Vernera-Morrisona Syndrom (1958), WDHA – watery diarrhea hypokalemia achlorhydria Tumor derived from pancreatic islet beta produced vasoactive intestinal hormon, often malignant, usually single, 80% of pancreatic APUDoma Clinical symptoms • persistent watery diarrhea (more than 3l / d) • symptoms of hypokalaemia • asthenie • achlorhydria • Tetany (as a result ofhypomagnesaemia) WDHA syndrom diagnosed very rare o Pure prognosis

49. Vipoma Liczne przerzuty w wątrobie

50. Vipoma Tomografia komputerowa