Endocrine Tumors Of The Pancreas
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Endocrine Tumors Of The Pancreas Presentation Transcript

  • 1. Endocrine Tumors of the Pancreas Dr. Methal Isam Al-Bayat General Surgery – KFHU Khobar – Saudi Arabia
  • 2. Anatomy
  • 3. Pancreatic Ducts
  • 4. Blood Supply
  • 5. Lymphatic Drainage
  • 6. Nerve Supply
  • 7. Physiology
    • The pancreas is divided functionally into
      • Exocrine Pancreas
        • 85% of pancreatic mass
      • Endocrine Pancreas
        • 2% of pancreatic mass
  • 8. Exocrine Pancreas
    • secretes a clear, alkaline (pH 7-8.3) solution of 1-2 L/day, containing digestive enzymes
    • secretion is stimulated by
      • secretin
      • CCK
      • parasympathetic vagal discharge
  • 9. Endocrine Pancreas
    • Nearly one million islets of Langerhans in the normal adult pancreas
    • vary greatly in size
    • larger islets are located closer to the major arterioles
    • smaller islets are embedded more deeply in the pancreatic parenchyma
    • islet cells originate from neural crest cells, aka APUD cells
  • 10. cont...
  • 11. Hormone Functions
  • 12. Endocrine Tumors
    • Pancreatic endocrine tumors are rare
    • approximately 5 per 1,000,000 per year
    • about 20% are non-functional
    • functional tumors may be benign or malignant
    • the histologic characteristics do not predict their clinical behavior
    • for all types of endocrine tumors, malignancy is defined by the presence of metastases
  • 13. cont...
    • no environmental risk factors have been associated with the development of these tumors
    • some syndromes have been associated with pancreatic endocrine tumors
      • MEN-1
      • von Hippel-Lindau
      • Neurofibramatosis
  • 14. Multiple Endocrine Neoplasia Type 1
    • characterized by
      • parathyroid hyperplasia
      • pituitary tumors
      • pancreatic endocrine tumors
    • autosomal dominant
    • equal incidence in men & women
  • 15. cont...
    • features of PET
      • occur at an younger age
      • multifocal disease
      • aggressive and malignant
      • found in uncommon sites
      • different incidence
        • PPomas (>80%) ‏
        • gastrinomas (54%) ‏
        • insulinomas (21%) ‏
        • glucagonomas (3%) ‏
        • VIPomas (1%) ‏
  • 16. Insulinomas
    • 60% of all PET
    • the average age at diagnosis 45 years
    • men and women are equally affected
    • equally distributed in the head, body, and tail of the pancreas
    • rarely, they may be located in the duodenum, splenic hilum, or gastrocolic ligament
    • 90% < 2 cm in size
  • 17. cont...
    • they are encapsulated, firm, yellow-brown nodules that are typically hypervascular
    • malignancy occurs in 10% of the cases
    • most are solitary lesions
    • 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
  • 18. cont...
    • Clinical presentation
      • headache
      • lethargy
      • dizziness
      • diplopia
      • amnesia
      • trembling
      • sweating
      • palpitations
      • nervousness
      • hunger
    neuroglycopenic symptoms catecholamine release
  • 19. cont...
      • weight gain
    • Whipple's Triad
      • low glucose level (<50 mg/dL) ‏
      • symptoms of hypoglycemia
      • symptoms resolve with administration of glucose
  • 20. cont...
    • Investigations
      • Laboratory Studies
        • low glucose levels (< 50 mg/dL) ‏
        • insulin levels > 7 U/mL
        • insulin/glucose ratio > 0.3
        • C-peptide to confirm endogenous source of insulin
      • Localization
        • CT and MRI for larger tumors
        • EUS can detect small tumors (<2 cm in size) ‏
        • angiography showing a “blush”
  • 21. cont...
    • management
      • pre-operatively
        • administration of diazoxide to prevent hypoglycemic attacks
        • other agents as verapamil, glucocorticoids, and growth hormone
      • peri-operatively
        • glucose infusions
  • 22. cont...
      • operative
        • tumor enucleation
        • pancreaticoduodenectomy
        • tumor debulking
      • post-operatively
        • octreotide
        • systemic chemotherapy
    • Outcomes
      • normal life expectancy for benign insulinoma
      • 5 years for malignant insulinomas
  • 23. Gastrinoma
    • 2 nd most common
    • mean age of patients is 50 years
    • slight male predominance (60%) ‏
    • gastrinomas produce ZES by overproduction of gastrin
    • over 60% are malignant
    • metastases may also involve the lungs or bone
  • 24. cont...
    • 90% of gastrinomas are located within Passaro's triangle
  • 25. cont...
    • Clinical presentation
      • severe form of peptic ulcer disease
        • refractory to standard treatment
        • atypical location
      • upper abdominal pain
      • GI bleeding
      • weight loss, nausea, vomiting
      • GERD
      • diarrhea relieved by NG suction
  • 26. cont...
    • Investigations
      • 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
  • 27. cont...
      • Localization
        • CT (not for small tumors) ‏
        • MRI (liver metastases) ‏
        • SRS with radiolabeled octreotide
        • EUS
  • 28. cont...
    • Management
      • indications for surgery
        • curable resection appears possible
        • symptom control
      • operative
        • enucleation of small lesions
        • distal pancreatectomy
        • pancreaticoduodenectomy
      • focus on symptomatic control for patients with metastatic disease
  • 29. cont...
    • Outcome
      • 5-yr survival 90% for patients without metastasis
      • 5-yr survival < 50% for patients with bulky metastasis
  • 30. 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
    • 2/3 are malignant
  • 31. cont...
    • Clinical presentation
      • 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
  • 32. cont...
      • 75% hypochlorhydia or achlorhydria
      • 20% flushing
    • W atery
    • D iarrhea
    • H ypokalemia
    • A chlorhydria
  • 33. cont...
    • Investigations
      • Laboratory findings
        • serum levels of VIP > 150pg/mL after an overnight fast
      • Localization
        • CT or SRS
        • intraoperative U/S will localize most tumors if pre-operative studies are inconclusive
  • 34. cont...
    • Management
      • pre-operative
        • aggressive hydration
        • correction of electrolyte abnormalities and acid-base disturbances
        • octreotide to reduce diarrhea volume, if diarrhea persists glucocorticoid added
      • operative
        • partial pancreatic resection
        • simple enucleation is inadequate as VIPomas tend to be invasive
  • 35. cont...
    • Outcome
      • only 1/3 are cured after resection
  • 36. 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% of patients
    • 5-17% are associated with MEN 1
  • 37. cont...
    • Clinical presentation
      • weight loss
      • hyperglycemia, with 76-94% having diabetes
      • normochromic normocytic anemia
      • fat-soluble vitamin deficiency
      • hypoaminoacidemia
      • thromboembolism
      • diarrhea
      • vulvovaginitis
  • 38. cont...
      • 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
  • 39. cont...
    • Investigations
      • Laboratory findings
        • fasting glucagon level > 50 pmol/L
      • Localization
        • CT easily detects them
        • angiography is also successful because of vascularity
    • Management
      • pre-operative
        • supplemental enteral nutrition
        • high dose of octreotide to reverse catabolic state
  • 40. cont...
        • IV infusion of amino acids to reverse symptoms and improve dermatitis
        • prophylaxis against thromboembolism
      • operative
        • enucleation is rarely sufficient
        • distal pancreatectomy
        • pancreaticoduodenectomy and rarely total pancreatectomy may be required
    • Outcome
      • 5-yr survival 85% if no metastases present
      • 5-yr survival 60% if metastases present
  • 41. Somatostatinoma
    • Exceedingly rare tumors
    • solitary, large tumors > 2 cm
    • patients are usually in their 5 th or 6 th decade of life
    • most are in the head
    • majority are malignant
    • may be associated with neurofibromatosis
  • 42. cont...
    • Clinical presentation
      • steatorrhea
      • cholelithiasis
      • diabetes
      • hypochlorhydria
    • Investigations
      • Laboratory
        • elevated somatostatin levels
      • Localization
        • CT
  • 43. cont...
    • Dx of somatostatinomas is rarely made pre-operatively
    • Management
      • pancreaticoduodenectomy
    • Outcome
      • cure rarely achieved
  • 44. Nonfunctional Tumors & PPomas
    • 20% of PET are nonfunctional
    • PPomas are classified as nonfunctional
    • 2/3 are malignant
    • 60-80% of malignant tumors have distant metastases at the time of Dx
    • typically larger (4-5 cm) ‏
    • Clinical presentation
      • abdominal pain
      • jaundice
  • 45. cont...
    • Investigations
      • localization with CT or MRI
    • Management
      • pancreaticoduodenectomy
      • distal pancreatectomy
    • Outcome
      • 5-yr survival approximately 50%
  • 46. Other Pancreatic Endocrine Tumors
    • Tumors which secrete
      • gastrin-releasing factor
      • adrenocorticotropic hormone
      • neurotensin
      • calcitonin
      • enteroglucagon
      • CCK
      • gastric inhibitory peptide
      • leutenizing hormone
  • 47. References
    • Schwartz's Principles of Surgery, by F. Charles Brunicardi, 8 th edition
    • Maingot's Abdominal Operations, by Michael J. Zinner and Stanely W. Ashley, 11 th edition
    • Current Surgical Therapy, by John L. Cameron, 8 th edition
    • 126 Current Surgical Diagnosis and Treatment, 12 th edition
  • 48. Thank You