Joseph R' Pisegna, M'D'

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Endocrine Tumors, the Other Kind of Pancreatic
Cancer
VA Greater Los Angeles Healthcare System
Joseph R. Pisegna, M.D. Associate
Professor Chief, Gastroenterology and
Hepatology VA Greater Los Angeles and UCLA School
of Medicine
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NE Tumors are being diagnosed with increasing
frequency

• Greater awareness of NE tumors
• Peptide Markers
• Improved Imaging Studies
• Octreoscan
• Endoscopic Ultrasonography

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Classification of NETs

• Foregut lungs and bronchi, stomach
• Midgut small intestine, appendix, proximal
  large bowel
• Hindgut distal large bowel, rectum

Pancreatic Endocrine Tumors
Carcinoid Tumors

• Insulinoma
• Gastrinoma
• Ppoma
• Glucagonoma
• VIPoma
• Somatostatin
• Etc.

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Initial Workup of NETs (NCCN)

• Grade of differentiation
• Specialized stains
• Laboratory studies depending on tumor suspected

• Localizing studies
• OctreoScan
• CT
• Ultrasound
• MRI
• Bone scan if symptoms

FNA or Biopsy
OctreoScan is a registered trademark of
Mallinckrodt, Inc.
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Hormone-Related Markers Indicated in Workup of
Neuroendocrine Tumors

• Carcinoid
• 24 hour urine 5HIAA
• Chromogranin A
• Gastrinoma (Gastrin)
• Insulinoma (Proinsulin, Insulin, C-peptide)
• Glucagonoma (Glucagon)
• VIPoma (VIP)
• Other Pancreas
• Chromogranin A
• Somatostatin
• Pancreatic Polypeptide

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Elevated Serum Ghrelin Levels in Patients with
Metastatic Neuroendocrine Tumors Maintains Body
Weight

• Hank S Wang, David S Oh, and Joseph R Pisegna
• Division of Gastroenterology, VA Greater Los
  Angeles Healthcare System and CURE VA/UCLA
  Digestive Disease Research Center, Department of
  Medicine, David Geffen School of Medicine at
  UCLA, Los Angeles, CA 90073

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Islet Cell Tumors Metastatic Disease (NCCN)
Hepatic resection if resectable
Asymptomatic
Observe with markers and scans every 3 to 6
months or clinical trial
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Islet Cell Tumors Metastatic Disease (NCCN)

• Octreotide 150 mcg SQ TID
• Octreotide LAR 20 mg IM monthly, then may
  gradually increase dose and frequencyor add
  Interferon Alpha
• Manage clinical syndromes as appropriate

Symptomatic or Progression
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Pancreatic Endocrine Tumors Medical Management
(NCCN)
Diet modifications, diazoxide, /- octreotide
Insulinoma
Octreotide, zinc, consider TPN Perioperative
anticoagulant, consider IVC Filter
Glucagonoma
VIPoma
Octreotide, consider TPN
Gastrinoma
H2-blockers or PPIs
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Octreotide

• A majority of NE tumors express somatostatin
  receptors
• A reduction in tumor growth has been demonstrated
  in vitro and in animal studies
• In two phase II trials, octreotide inhibited
  islet cell tumor growth in 37 of patients with
  metastatic disease, but this effect was short
  lasting.

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Role of ?-IFN Alone for Pancreatic NETs
No. Pts
Author
Dose
Response
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Interferon Sandostatin LAR UCLA Study

• 22 patients with NE Tumors (4 gastrinoma)
• Octreotide (20mg IU TID) and alpha interferon (5
  million IU TIW)
• CT scan at 3-month intervals
• Overall 64 response rate 12 patients had stable
  disease and 1 patient had a reduction in tumor
  size of at least 25

Oh, D., Ohning, G., and Pisegna, J.R. Am. J.
Gastro 2006
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Islet Cell Tumors Progressive Disease (NCCN)

• Hepatic regional therapy (arterial embolization,
  chemoembolization or other)
• Ablative therapy (RFA, cryotherapy)
• Systemic chemotherapy with streptozocin/doxorubici
  n
• Clinical trial
• Interferon Octreotide
• Radiation for bone metastases

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Current NCCN Guidelines for Neuroendocrine
Tumors Summary

• Surgery is mainstay of local management
• No indication for adjuvant therapy with possible
  exception of atypical tumors
• Octreotide used for control of hormonal symptoms
• No single standard treatment for progressive,
  metastatic disease

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Multiple Endocrine Neoplasias

• Classification
• MEN-I (Werners syndrome) Parathyroid
  hyperplasia, pancreatic NE tumor, pituitary
  tumors.
• MEN-2A(Sipples syndrome) bilateral meduallry
  thyroid carcinoma, pheochromocyoma,
  hyperparathyroidism.
• MEN-2B Medullary thyroid carcinoma,
  pheochromocytomas, hyperparathyroidism, marfanoid
  habitus, puffy lips, prognathis, and medullated
  corneal nerves.

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MEN I

• Genetic
• Inherited in autosomal dominant fashion
• Men I gene-Long arm chromosome 11 (near 11q13)
• Gen
• Epidemiology
• Prevelance of MEN I is 200-200/million
• Males-Females

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