Pancreatic tumors.pptx

1. PANCREATIC TUMORS BY Dr.NILESH SINHA {Associate Professor} DEPT. OF GENERAL SURGERY

2. EMBRYOLOGY The pancreas arises as a larger dorsal bud and a smaller ventral bud that fuse to form the pancreas.

3. ANATOMY

4. DUCTS The main pancreatic duct of Wirsung, 3 mm in diameter lies near the posterior surface of the pancreas. It is related to the bile duct which join to form the ampulla of Vater which opens on the major duodenal papilla, 8 to 10 cm distal to the pylorus. The accessory pancreatic duct of Santorini opens into the duodenum at the minor duodenal papilla.

5. BLOOD SUPPLY

6. NERVE SUPPLY The vagus or parasympathetic and splanchnic sympathetic nerves supply the pancreas and control pancreatic secretion.

7. LYMPHATIC DRAINAGE Lymphatics follow the arteries and drain into the pancreatico-splenic, coeliac and superior mesenteric groups of lymph nodes.

8. FUNCTIONS 1. Digestive: Trypsin breaks down proteins to lower peptides. Amylase hydrolyses starch and glycogen to disaccharides. Lipase breaks down fat into fatty acids and glycerol. • Normal serum amylase – 25 to 115 U/L • Normal serum lipase – 73 to 393 U/L 2. Endocrine: Insulin helps in utilizations of sugar in the cells. Deficiency of insulin results in hyperglycaemia and is called diabetes mellitus. 3. Pancreatic juice: It provides appropriate alkaline medium (pH 8) for the activity of the pancreatic enzymes.

9. BENIGN EXOCRINE PANCREATIC NEOPLASMS

10. WHO CLASSIFICATION • Serous Cystic Neoplasm • Serous cystadenoma • Serous microcystic adenoma • Serous oligocystic adenoma • Serous cystadenocarcinoma • Mucinous Cystic Neoplasm • Mucinous cystadenoma • Mucinous cystic neoplasm with moderate dysplasia • Mucinous cystadenocarcinoma(invasive/non-invasive) • Intraductal Papillary Mucinous Neoplasm • Intraductal papillary mucinous adenoma • Intraductal papillary mucinous neoplasm with moderate dysplasia • Intraductal papillary mucinous carcinoma (invasive/non-invasive) • Solid pseudopapillary neoplasm

11. FOUR MORPHOLOGICAL TYPES

12. SEROUS CYSTIC NEOPLASM • 30% of cystic neoplasm • Women, 50 - 70 years old • Low risk of malignancy • Presentation • Incidental • Epigastric pain • Abdominal fullness • Weight loss.

13. INVESTIGATIONS EUS with FNA o Cytology: Scant cellularity/Bloody o Biochemistry  Low CEA.  Low amylase  Low CA 19-9 CT o Polycystic/Microcystic o Stellate scar or sunburst calcifications o Internal septate o Honey comb appearance

14. MANAGEMENT • Observation for 6-12 months if no symptoms • Consider resection if: o> 4 cm and symptomatic oNo definite diagnosis. oRapid growth.

15. MUCINOUS CYSTIC NEOPLASMS • Female • Middle age 30 - 50 years • There is association with KRAS mutation • Body or tail of the pancreas 90% • Presentation: • Abdominal discomfort. • Recurrent Pancreatitis • Gastric outlet obstruction

16. INVESTIGATIONS • CT/MRI: • Thick cyst wall • Smooth sharp boundaries. • DO NOT communicate with pancreatic ductal system. • Pancreatic duct dilation • Eggshell calcification • EUS with FNA: • Viscous fluid • CEA • Adenoma > 200 ng/mL • Mucinous cystadenocarcinoma > 6000ng/mL

17. MANAGEMENT • Observation for small tumors. • Surgical resection if any of the following: • >3 cm • Main duct dilation • Mural nodule. • Follow up • Non invasive: Annually the first years. • Invasive: • Every 4 month the first 2 years. • Biannually until year 5

18. INTRADUCTAL PAPILLARY MUCINOUS NEOPLASM • Male = Female 50 - 70 years. • Head > Body • Presentation: • Abdominal pain. • Pancreatitis. • Weight loss. • Jaundice. • New onset diabetes.

19. • Types: • According to the affected duct • Main duct type. • Branch duct type. • According to the dysplasia • Adenoma • Borderline • Carcinoma in situ. • Frankly invasive. • CT • Main pancreatic or duct dilation. • Involvement of any part of the pancreas or the whole pancreas • Continuity of cyst with ductal system • Irregular and poorly demarcated

20. MALIGNANT EXOCRINE PANCREATIC NEOPLASMS

21. CLINICAL PRESENTATION

22. PHYSICAL EXAMINATION • Icterus • Palpable distended gall bladder (1/3rd) • Courvoisier’s Law – Obstructive jaundice with palpable gall bladder is seldom due to gall stones • Exceptions – double stone impaction, primary cbd stones, oriental chalangiohepatitis • Widespread disease • Virchow’s /left supraclavicular nodes • Sister Mary Joseph /Periumbilical nodes • Blumer shelf/ perirectal nodes • Trosseau syndrome/ Migratory thrombophlebitis

23. INVESTIGATIONS • Hepatic function evaluation • Coagulation profile • Nutritional assessment (Albumin) • Tumour markers • CEA • CA 19-9

24. IMAGING MODALITIES 1. Ultrasound - first imaging investigation in a patient presenting with obstructive jaundice 2. CT - PANCREATIC PROTOCOL • Neutral oral contrast like water is used. Positive contrast may compromise the three dimensional image • Thinnest possible sections <3mm preferably 0.5-1mm

25. • CT tells us the Tumour location, size, vessel involvement, local resectability, Nodal involvement, Metastatic status • Scan acquisition time – • Pancreatic parenchymal phase at 40-50s • Portal venous phase at 65-70s (relation of tumour to the vessels) • MRCP/ERCP – Double duct sign • Xray – Widening of C loop • Duodenography – Frosberg inverted 3 sign • PET CT – IOC for staging

26. SURGERY • For tumors involving the head of the pancreas, pancreaticoduodenectomy is the procedure of choice. • Walter Kausch was the first to successfully perform pancreaticoduodenectomy in Berlin 1912. • Allen Whipple popularized the operation in US in 1935. • Operative mortality of >25% and morbidity of >50%

31. • Tumors arising in the body and tail of the pancreas are rarely resectable at the time of presentation. The survival is significantly shorter because of the more advanced nature of resected tumors. • Involvement of the celiac axis is a contraindication to resection. • For resectable tumors, distal pancreatectomy and en bloc splenectomy should be performed. • The distal pancreas and spleen are devascularized and the neck of the pancreas is divided. • Laparoscopic distal pancreatectomy (LDP) is increasingly used for benign conditions and its use for the treatment of PDAC remains to be fully validated although it has shown significantly increased mortality.

32. • Delayed gastric emptying is characterized by the need for prolonged nasogastric decompression or inability to tolerate oral intake. • An underlying structural abnormality is ruled out with imaging and endoscopy. • Enteral feeding with a feeding tube placed during surgery is used to maintain nutrition while waiting for stomach function to return. POST OP COMPLICATIONS

33. A pancreatic fistula or a leak is defined as an output via an intraoperatively placed drain of any measurable volume on or after postoperative day 3, with amylase >3 times normal serum value. Most fistulas require no additional intervention.

34. CHEMOTHERAPY • More than 80% of patients with pancreatic cancer present with locally advanced or metastatic disease and are primarily managed with chemotherapy. • Gemcitabine has been the standard of care for the treatment of metastatic pancreatic. • A regimen that has shown greater efficacy than gemcitabine is FOLFIRINOX (5-FU, oxaliplatin, irinotecan, and leucovorin) • FOLFIRINOX is being used as the neoadjuvant regimen of choice in patients with borderline resectable pancreatic cancer and has good performance status who can tolerate this aggressive regimen.

35. PALLIATIVE THERAPY • Biliary obstruction ERCP with metal stent placement Roux-en-Y hepaticojejunostomy. • Delayed gastric emptying Endoscopic stenting Preventive gastrojejunostomy • Pain relief Antiinflammatories/ long-acting opioids Celiac nerve block CT-guided percutaneous neurolysis

36. ENDOCRINE PANCREATIC NEOPLASMS

37. INTRODUCTION • The pancreatic islets or islets of Langerhans are the regions of the pancreas that contain its endocrine cells. • Islet cells originate from neural crest cells, aka APUD cells - Amine precursor uptake and decarboxylation cell. Alpha cells (A) Beta cells (B) Delta cells (D) F or polypeptide cells (PP)

38. CELL TYPES AND DISTRIBUTION CELL TYPE HORMONE PRODUCE ENDOCRINE TUMOUR/ SYNDROME DISTRUBUTION THROUGHOUT THE PANCREAS ALPHA (A) GLUCACON GLUCAGONOMA UNIFORM THROUGHOUT THE BODY/TAIL BETA (B) INSULIN INSULINOMA BODY/TAIL DELTA (D) SOMATOSTATIN SOMATOSTATINOMA UNIFORM THROUGHOUT F PP PPOMA UNCINATE PROCESS D 2 VIP VIPoma/WDHA UNIFORM THROUGHOUT G GASTRIN GASTRINOMA/ZES NOT PRESENT/SECRETED IN NORMAL STATE

39. RISK FACTORS • SYNDROMIC • MEN 1 (Wermer syndrome)- parathyroid hyperplasia, pituitary tumours, pancreatic endocrine tumours (30-80 % of patient with MEN 1) • Von Hippel-Lindau • Neurofibromatosis • Tuberous sclerosis complex (TSC) • GENETICS • Homozygous deletion or silencing of 5′ CpG island methylation; > 90% of gastrinomas and non-functioning pancreatic tumours. • LOH (loss of heterozygosity) at chromosome 11q – functional tumours • LOH at chromosome 6q – nonfunctional tumours

40. INSULINOMA • 60% of all pancreatic endocrine tumours • Average age at diagnosis 45 years • Men and women are equally affected • Equally distributed in the head, body, and tail of the pancreas • 90% < 2 cm in size • Typically hypervascular • Malignancy occurs in 10% of the cases • Multicentricity occurs in about 10% of cases and should raise the suspicion of MEN-1 • Release large amounts of proinsulin (C- peptide and insulin) which cause hypoglycemia

41. • CATECHOLAMINE RELEASE causes trembling, sweating, palpitations, nervousness, hunger, weight gain • NEUROGLYCOPENIC SYMPTOMS like headache lethargy dizziness diplopia amnesia • WHIPPLE'S TRIAD - low glucose level (<50 mg/dL) - symptoms of hypoglycemia - symptoms resolve with administration of glucose • LABORATORY STUDIES • low glucose l levels (< 50 mg/dL) • insulin levels > 7 U/mL • C-peptide to confirm endogenous source of insulin (marker of insulin secretion) • DIAGNOSIS • CT and MRI for larger tumors • EUS can detect small tumors (<2 cm in size) • Angiography showing a “blush” CLINICAL FEATURES INVESTIGATIONS

42. GASTRINOMA • 2nd most common • Mean age of patients is 50 years • Slight male predominance (60%) • Gastrinomas produce ZES (Zollinger Ellison syndrome) by overproduction of gastrin • Over 60% are malignant (Most common malignant endocrine pancreatic tumour is gastrinoma) • 90% of gastrinomas are located within PASSARO'S TRIANGLE 1. Junction between the head and neck of the pancreas 2. Junction of cystic duct with CBD 3. Junction between the 2nd and 3rd parts of the duodenum

43. LABORATORY FINDINGS • fasting serum gastrin level 200-1000 pg/mL • H2 blockers should be stopped 1 week prior to testing, and PPI 3 weeks • basal acid output > 15 mEq/L ENDOSCOPY • multiple ulcers • large gastric rugal folds • mucosal edema • jejunal hypermotility - Severe form of peptic ulcer disease • refractory to standard treatment • atypical location – jejunal ulcers - upper abdominal pain - GI bleeding - weight loss, nausea, vomiting - GERD - Diarrhea relieved by NG suction CLINICAL FEATURES

44. VIPOMA • VIPomas originate from neoplastic D2 cells aka WDHA or Verner- Morrison syndrome • Exceedingly rare tumors • Bimodal age distribution • most patients are middle aged • 10% < 10 years • Usually solitary located in body or tail • 2/3rd are malignant

45. CLINICAL FEATURES Profuse, watery, iso-osmotic secretory diarrhea • May exceed 3 L/day • Independent of food intake • Doesn't resolve with NG suction • Devoid of blood, fat, or inflammatory cells • Weight loss • Crampy abdominal pain • Dehydration • Electrolyte abnormalities • Metabolic acidosis (due loss of large amount of bicarbonate from pancreatic secretion ) WDHA • Watery • Diarrhea • Hypokalemia • Achlorhydria LABORATORY FINDINGS Serum levels of VIP > 150 pg/mL after an overnight fast LOCALIZATION CT or SRS Intraoperative U/S will localize most tumors

46. GLUCAGONOMA • Exceedingly rare tumors • 2-3 times more common in women • Averaging 5-10 cm • Highly vascular • 65-75% are found in the body or tail • Malignancy occurs in 50-80% • 5-17% are associated with MEN 1 • Glucagon is a catabolic hormone, and most patients present with malnutrition.

47. CLINICAL PRESENTATION • Weight loss • Hyperglycemia, with 76-94% having diabetes • Normochromic normocytic anemia • Fat-soluble vitamin deficiency • Hypoaminoacidemia • Thromboembolism • Diarrhea • Vulvovaginitis LABORATORY FINDINGS Fasting glucagon level > 50 pmol/L LOCALIZATION CT easily detects them Angiography is also successful because of vascularity

48. MIGRATORY NECROLYTIC DERMATITIS • Found in 2/3 of patients due to severe amino acid deficiency • Begins as erythematous patches that spread radially • Bullae develop then slough with bacterial or fungal superinfection • Healing begins in center, takes 2-3 weeks, leaving hyperpigmented skin

49. SOMATOSTATINOMA • Exceedingly rare tumors • Solitary, large tumors > 2 cm • Patients are usually in their 5th or 6th decade of life • Most are in the head of pancreas • Majority are malignant • May be associated with neurofibromatosis • Somatostatin inhibits pancreatic and biliary secretions

50. LABORATORY Elevated somatostatin levels • DIAGNOSIS CT CLINICAL FEATURES • Steatorrhea • Cholelithiasis • Diabetes • Hypochlorhydria • They present gallstones due to bile stasis, diabetes due to inhibition of insulin secretion, and steatorrhea due to inhibition of pancreatic exocrine secretion and bile secretion

51. SRS – SOMATOSTATIN-RECEPTOR SCINTIGRAPHY • Many pancreatic endocrine tumours overexpress somatostatin receptor subtype 2 • Used for metastatic disease • SRS is useful if tumours are not evident in CT/MRI. • Sensitivity is 80% excluding insulinoma. • In insulinomas somatostatin receptors are present only at low levels or absent entirely.

52. ROLE OF TUMOUR MARKERS • A variety of tumor markers have been proposed for functional and non-functional pNETs. • The most common of these is chromogranin A (CgA), an acid soluble protein that is found in secretory granules of neuroendocrine cells, CgA is sensitive with elevated levels present in 72-100% of patients. • Others such as neuron-specific enolase (NSE), pancreatic polypeptide, pancreastatin, and human chorionic gonadotropin have been proposed but less sensitive.

53. CYTOREDUCTIVE SURGERY Most pNETs have a relatively indolent course compared to other pancreatic neoplasms, and that tumor debulking, while not curative, provides the theoretical advantages of symptom control in functional tumors. TARGETTED THERAPY • Everolimus an oral mTOR signaling inhibitor, has shown some effect in treating pNETs. • Sunitinib is an oral tyrosine kinase inhibitor that is known to target VEGF receptors.

54. THANK YOU

Pancreatic tumors.pptx

Pancreatic tumors.pptx

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