GASTROENTEROPANCREATIC NEUROENDOCRINE TUMORS
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GASTROENTEROPANCREATIC NEUROENDOCRINE TUMORS

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GASTROENTEROPANCREATIC NEUROENDOCRINE TUMORS

GASTROENTEROPANCREATIC NEUROENDOCRINE TUMORS

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GASTROENTEROPANCREATIC NEUROENDOCRINE TUMORS GASTROENTEROPANCREATIC NEUROENDOCRINE TUMORS Presentation Transcript

  • DR FARHAN ALI MBBS,MCPS,FCPS MEDICAL SPECIALISTCDA HOSPITAL ISLAMABAD PAKISTAN
  • BACKGROUND1969 (Pearse) described APUD cells (amine precursor uptake and decarboxylation) cells that make polypeptides and biogenic aminesThese cells have dense core secretory granules which store and release hormones in response to external stimuli
  • Do not have axons/synapsesAre part of the diffuse endocrine system (DES)Endocrine tumors of the gut and pancreas originate from DES cells
  • IncidenceIncidence 1-2 in 100,000Account for <2% of GI malignanciesNeuroendocrine tumors of the lung, GI tract and mediastinum have a higher incidence in patients >50carcinoid of the appendix have a higher incidence in patients age <30
  • WHO CLASSIFICATION Well differentiated NET (non-invasive, benign behaving or uncertain malignant potential) Well-differentiated NE carcinomas (low grade malignant and has invasion or muscularis propria or metastasis) Poorly differentiated endocrine carcinomas (high grade, malignant)
  • GENERAL CLASSIFICATION1- Carcinoid Tumors  25% foregut (lung, thymus, gastric mucosa, duodenum)  40-60% midgut (distal ileum and jejunum) (includes carcinoid syndrome)  Hindgut (colon, rectum)2-Endocrine Pancreatic Tumors  60% Functioning (Zollinger Ellison, insulinoma, glucagonomas, VIPomas, etc)  Non-functioning (usually large and metastatic at the time of diagnosis
  • 1-Endocrine Pancreatic Tumorsa-INSULINOMASIslet cell tumorsSecrete excess of predominantly insulinUsually present at age 40-50More common in womenClinical symptoms include sweating, tremors, tachycardia, confusion, weakness10% of patients develop metastasisComplete resection cures most patients
  • b-GASTRINOMASOver secretion of gastrinZollinger-Ellison Syndrome: atypical peptic ulcer disease, gastric hyperacidity and hypersecretion, associated with islet cell pancreatic tumorsAge at diagnosis ~50More common in males (~60%)Metastasis in 60% of patientsComplete resection results in 10 year survival of 90%; less likely if large primary
  • c-GLUCAGONOMASPresents with mild DM and severe dermatitis (necrolytic migratory erythema), stomatitis, diarrhea~70% are malignantMetastasis in >60% patients
  • d-VIPOMASOver secretion of VIPCauses watery diarrhea, marked hypokalemia80% are associated with the pancreasMetastasis occurs in ~70% of patientsComplete resection results in 5 year survival of 95%
  • e-SOMATOSTATINOMASCholelithiasis, DM, diarrhea, weight loss, steatorrheaMetastasis in ~50% patientsComplete resection with 5 year survival of 95% and if has metastasis the 5 year survival decreases to 60%
  • 2-Carcinoid Tumoursarise in the thymus, bronchi and throughout the gastrointestinal tract,most commonly observed in the small bowel.present due to local mass effects, e.g. small bowel obstruction, appendicitis, pain from hepatic metastases, or because of symptoms related to hormone excess.This includes ectopic secretion of ACTH, causing Cushings syndrome , or 5-HT, causing carcinoid syndrome.
  • CLINICAL FEATURES OF THECARCINOID SYNDROMEFlushingWheezingDiarrhoeaFacial telangiectasiaCardiac involvement (tricuspid regurgitation, pulmonary stenosis, right ventricular endocardial plaques leading to heart failure
  •  Carcinoid syndrome only occurs when the vasoactive hormones reach the systemic circulation. In the case of gastrointestinal carcinoids, this invariably means that the tumour has metastasised to the liver, as hormones secreted by the primary tumour into the portal vein are metabolised by the liver
  • DIAGNOSTIC PROCEDUREBiopsy  Immunohistochemistry Antibodies to chromogranin A Neuron specific enolase Stain for serotonin if suspect carcinoid Stain for gastrin if suspect Zollinger – Ellison
  • LABORATORY EVALUATIONCarcinoid: 24 hour urinary 5-HIAA raised in carcinoid tumors of the foregut and midgut but not generally raised in tumors of the hindgutGastrinoma: raised basal serum gastrin, high gastric acid secretionInsulinoma: raised fasting insulin/glucose ratio, proinsulin or C-peptide
  • Glucagonoma: raised serum pancreatic glucagon and enteroglucagonVIPoma: raised fasting vasoactive intestinal peptideSomatostatinoma: elevated fasting somatostatinAll NETs: elevated chromogranin A
  • RADIOLOGIC DIAGNOSISCTMRIUSSomatostatin Receptor Scintigraphy (SRS) – based on presence of somatostatin receptors in 80-90% of NETPET to evaluate tumor metastasisEndoscopic ultrasound – sensitivity/specificity appx 80% for tumors in pancreas and duodenum and can allow for FNA
  • Anatomic Imaging: CT Std Arterial Venous DelayedImaging studies property of James Yao, MD. CT: computed tomography.
  • MRI = magnetic resonance imagingImaging studies property of James Yao, MD.
  • Imaging studies property of James Yao, MD.
  • THERAPYSurgery  For localized disease  Only way to cure  Can include debulking or laser procedures  however not applicable to all cases as many pts present with metastatic diseaseMedical therapy:  Somatostatin analogs  Interferon alpha  Diazoxide may reduce insulin secretion in insulinomas  Cytotoxic drugs
  • SOMATOSTATIN ANALOGSUsed since 1980’sHormone blocking agents that are synthetic somatostatin derivatives (ex: octreotide and lanreotide)First line of treatment for neuroendocrine gastroenteropancreatic tumors2nd -3rd line for insulinomas and gastrinomasSide effects: development of gallstones secondary to inhibition of cholecystokinin release, pain at site, hypo or hyperglycemia, rash, alopecia, fluid retention
  • Interferon AlphaFor mid-gut carcinoidsWork by direct effect on tumor cells by blocking cell cycle in G1/S phase and inhibiting protein/hormone synthesis and inhibition of angiogenic functionCan by used with or without somatostatin analogsSE: flu-like symptoms, fever, anemia, thrombocytopenia, leukopenia
  • CHEMOTHERAPYCytotoxic treatment is generally a palliative option for metastasizing neuroendocrine carcinomasStreptozotocin, + 5-FU and doxorubicin (response rate >50% in malignant NET)Cisplatin/paraplatin + etoposide (for poorly differentiated NET in fore-gut