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DR FARHAN ALI
        MBBS,MCPS,FCPS
     MEDICAL SPECIALIST
CDA 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 Tumors

a-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
 Cushing's syndrome , or 5-HT, causing 'carcinoid
 syndrome'.
CLINICAL FEATURES OF THE
CARCINOID 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                                               Delayed




Imaging studies property of James Yao, MD.   CT: computed tomography.
MRI = magnetic resonance imaging
Imaging 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
GASTROENTEROPANCREATIC NEUROENDOCRINE TUMORS
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
GASTROENTEROPANCREATIC NEUROENDOCRINE TUMORS

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GASTROENTEROPANCREATIC NEUROENDOCRINE TUMORS

  • 1. DR FARHAN ALI MBBS,MCPS,FCPS MEDICAL SPECIALIST CDA HOSPITAL ISLAMABAD PAKISTAN
  • 2. 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
  • 3. Do not have axons/synapses Are part of the diffuse endocrine system (DES) Endocrine tumors of the gut and pancreas originate from DES cells
  • 4. 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
  • 5. 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)
  • 6. 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
  • 7. 1-Endocrine Pancreatic Tumors a-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
  • 8. 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
  • 9. c-GLUCAGONOMAS Presents with mild DM and severe dermatitis (necrolytic migratory erythema), stomatitis, diarrhea ~70% are malignant Metastasis in >60% patients
  • 10. 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%
  • 11. 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%
  • 12. 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 Cushing's syndrome , or 5-HT, causing 'carcinoid syndrome'.
  • 13. CLINICAL FEATURES OF THE CARCINOID SYNDROME Flushing Wheezing Diarrhoea Facial telangiectasia Cardiac involvement (tricuspid regurgitation, pulmonary stenosis, right ventricular endocardial plaques leading to heart failure
  • 14.  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
  • 15. DIAGNOSTIC PROCEDURE Biopsy  Immunohistochemistry Antibodies to chromogranin A Neuron specific enolase Stain for serotonin if suspect carcinoid Stain for gastrin if suspect Zollinger – Ellison
  • 16. 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
  • 17. Glucagonoma: raised serum pancreatic glucagon and enteroglucagon VIPoma: raised fasting vasoactive intestinal peptide Somatostatinoma: elevated fasting somatostatin All NETs: elevated chromogranin A
  • 18. 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
  • 19. Anatomic Imaging: CT Std Arterial Venous Delayed Imaging studies property of James Yao, MD. CT: computed tomography.
  • 20. MRI = magnetic resonance imaging Imaging studies property of James Yao, MD.
  • 21. Imaging studies property of James Yao, MD.
  • 22. 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
  • 23. 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
  • 25. 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
  • 26. 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