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Management of Pancreatic Islet Cell Tumors
Ravi Dhanisetty, MDNovember 5, 2009
Morbidity and Mortality Conference
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Case PresentationCase Presentation
• 42 yr female with chronic abdominal pain42 yr female with chronic abdominal pain.• PMHx: Uterine fibroids
M di ti t f i• Medications: percocet prn for pain.
• Physical exam: non-contributory• Labs:Labs:
– Calcium – 9.0, amylase – 30, lipase – 70
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Case PresentationCase Presentation
• Evaluated by patient’s gynecologistEvaluated by patient s gynecologist.• CT Scan
Dil t d ti d t ith b t t ff– Dilated pancreatic duct with abrupt cutoff. – No discrete mass or evidence of metastatic
diseasedisease.
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Octerotide ScanOcterotide Scan
• Octreotide Scan - normal studyOctreotide Scan normal study
• EUS –EUS – Mass in the body of pancreas– Dialated duct distal to it– Biopsy: neuro-endocrine tumor of pancreas
PROCEDURE• Distal pancreatectomy and hysterectomy.
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ProcedureProcedure• Entered lesser sac by lifting greater omentum off transverse colon• Posterior wall of stomach lifted off the pancreas• Posterior wall of stomach lifted off the pancreas. • Kocherized duodenum.• Mass was visible in the body of pancreas lateral to the SMV
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PathologyPathology
• Low-grade pancreatic endocrine neoplasmLow grade pancreatic endocrine neoplasm• 2.5 x 2.2 x 1.6 cm• Multiple satellite nodules in the tail of pancreasMultiple satellite nodules in the tail of pancreas• 0/10 HPF mitotic figures• Lymph nodes and margins negative• Lymph nodes and margins negative.• Evidence of acute and chronic pancreatitis.• Immuno histochemistry positive for• Immuno-histochemistry – positive for
chromogranin and serotonin.
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Post-operative CoursePost operative Course
• Complicated by post operative ileusComplicated by post operative ileus• Tolerated diet on pod #8 and discharged
on POD #11on POD #11.
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Management of Pancreatic Islet Cell Tumors
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Pancreatic Islet Cell TumorsPancreatic Islet Cell Tumors• Rare neoplasms (1/100,000)Rare neoplasms (1/100,000)• 1000 x common in autopsy statistics than in
clinical practice.p• Most are benign / non-functional• Malignant ones – usually metastatic at diagnosisMalignant ones usually metastatic at diagnosis• All secrete Chromogranin A – detect in serum.
• Sporadic or can occur w/ inherited syndromes -MEN 1MEN 1
• Taupenot et al. 2003.
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CharacteristicsCharacteristics
% Malignant % AssociatedTumor % Malignant(% Resectable) % Multicentric 5-Year Survival % Associated
with MEN-1
Insulinoma 10 (>90%) 5–10 97 5
Gastrinoma 60–90 (79%) 60–70 60–70 25
Glucagonoma 50–80 (35%) <5 50–60 1–20Glucagonoma 50–80 (35%) <5 50–60 1–20
VIPoma 40–70 (70%) <5 50 5
Somatostatinoma 75 (60%) <5 40 45–50
Nonfunctioning and PPoma >60 <5 30–50 20–40and PPoma
Modified from Mansour JC, Chen H: J Surg Res 120:139, 2004.
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Pancreatic Islet Cell TumorsPancreatic Islet Cell Tumors
• Anatomic distribution:Anatomic distribution:– Gastrinomas, PPomas, and
somatostatinomas - 75% right of SMAsomatostatinomas 75% right of SMA– Insulinomas, glucagonomas – 75% body and
tail of pancreastail of pancreas
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General ManagementGeneral Management
• Diagnosis of particular functioning tumorDiagnosis of particular functioning tumor.• Localization of the neoplasm.
• Surgical resection if feasible. • Palliation of symptoms.
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Efficacy of Localization of Tumors f th Pof the Pancreas
MODALITY TRUE POSITIVES (%)Noninvasive
Ultrasonography 23
Octreotide radioimaging (SRS)[*] 86Octreotide radioimaging (SRS) 86
CT 43
MRI 26
Invasive
Endoscopic ultrasonography 82
S l i i h 56Selective angiography 56
Portal venous sampling 76
Provocative angiography[†] 65Provocative angiography – Calcium and glucagon stimulation test for insulinoma.
Norton JA: Neuroendocrine tumors of the pancreas and duodenum. Curr Probl Surg 31:97, 1994.
g g p y
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InsulinomaInsulinoma
Most common typeMost common type• 5th & 6th decade, females
S t i li ( i li ) Whi l ' t i d• Secrete insulin(proinsulin): Whipple's triad• Ratio serum Insulin : glucose > 0.3.• Provocative tests:
– Glucagon stimulation test – 72% sensitiveGlucagon stimulation test 72% sensitive– Calcium infusion test
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InsulinomaInsulinoma• Localization – CT or
EUSEUS• Octreotide scanning
not useful.• Transhepatic
selective venous sampling
• Exploration with intra-operative ultrasound.
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GlucagonomaGlucagonoma
• Fifth decade of lifeFifth decade of life• Rare – 4D syndrome, necrolytic migratory
erythemaerythemaGlucagon level > 500 pg/ml.
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GlucagonomaGlucagonoma
Localization:Localization:• Large (>5cm) and metastatic (75%)
L t d i b d / t il f• Located in body / tail of pancreas• CT/MRI. Octreotide scanning (75%)
• Aggressive nutritional support and DVTAggressive nutritional support and DVT prophylaxis prior to intervention.
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Vasoactive Intestinal Peptide –S i TSecreting Tumor
• 30 – 50 years, rare (1/10,000,000); WDHA syndrome.y ( ) y
S lit > 3 l t d i t il f• Solitary, > 3 cm, located in tail of pancreas• 60-80 % metastatic at diagnosis.• CT / MRI.CT / MRI. • Octreotide scanning is highly sensitive.
• Pre-operative octreotide, hydration with correction of metabolic abnormalities and hypokalemia.
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SomatostatinomaSomatostatinoma
• Rarest of islet cell tumorsRarest of islet cell tumors. • Fifth decade of life, diagnosed incidentally
Di b t (60%) h l lithi i (70%)• Diabetes (60%), cholelithiasis (70%), steatorrhea
• Somatostatin level > 10 ng/ml• Mostly solitary, large, located in head of y y, g ,
pancreas, metastatic (75%)• CT/MRI; Octreotide scanningCT/MRI; Octreotide scanning.
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Pancreatic Polypeptide – Secreting TTumors
Third most commonThird most commonSymptoms:
i ht l j di bd i l i– weight loss, jaundice, abdominal pain. Diagnosis: PP - > 300 pg /ml
Localizationoca at o• Large lesions – CT / MRI.
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Non-Functioning Islet Cell TumorsNon Functioning Islet Cell Tumors• Secrete low levels of hormones / chromogranin A• Common symptom: abdominal pain (86%)• Diagnosed with CT / MRI.g
• Should be considered as aggressive as• Should be considered as aggressive as functioning tumors and with similar prognosis
Similar 5 yr survival– Similar 5 yr survival
– Mansour and Chen, 2004.
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MEN IMEN I• Parathyroid, pituitary, pancreas and occasionally y p y p y
adrenal gland.• 25% of gastrinomas, 10% of insulinomas
associated with MEN Iassociated with MEN I• MEN I - > 50% have gastrinomas, 20% have
insulinomas.
• All patients with pancreatic endocrine tumors –check serum calcium levelscheck serum calcium levels– Elevated calcium levels: study parathyroid and
pituitary
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Surgical TreatmentSurgical Treatment
• Only curative therapyOnly curative therapy.• After appropriate serum marker testing
and localization studyand localization study. • Operative exploration and feasibility of
tiresection• Formal resection except for cases of
insulinoma (enucleation.)
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Metastatic Disease
Surgical resection confers survival advantage and relief from symptoms.
• Mansour & Chen, 2004.
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Metastatic DiseaseMetastatic Disease
• Hepatic Resection – for liver only metsHepatic Resection for liver only mets• RFA / Cryoablation
H ti t b li ti• Hepatic artery embolization
• Radionuclide therapy – octreotide, MIBG• ChemotherapyChemotherapy
– Limited benefit Enroll in clinical trials– Enroll in clinical trials
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Surgical Treatment AlgorithmSurgical Treatment Algorithm
• Chen H: SSAT/AGA/ASGE State of the Art Conference on Pancreatic Neuroendocrine Tumors: consensus statement, J Gastrointest Surg 10:321, 2006.
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ConclusionsConclusions
• Pancreatic Islet cell tumors - uncommonPancreatic Islet cell tumors uncommon class of neoplasms
• Can be easily diagnosed with careful• Can be easily diagnosed with careful history and few laboratory tests.L li ti d l t i l• Localization and complete surgical resection provides best symptom control
d i ll i land improves overall survival.
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ReferencesReferences• Chen H: SSAT/AGA/ASGE State of the Art Conference on
Pancreatic Neuroendocrine Tumors: consensus statement JPancreatic Neuroendocrine Tumors: consensus statement, J Gastrointest Surg 10:321, 2006.
• Chen H. Management of Pancreatic Islet Cell tumors excluding gastrinoma. Cameron: Current Surgical Therapy, 9th Ed. 2008.
• Mansour JC, Chen H: Pancreatic endocrine tumors. J Surg Res 120:139, 2004.
• Norton JA: Neuroendocrine tumors of the pancreas and duodenum. Curr Probl Surg 31:97 1994Curr Probl Surg 31:97, 1994.
• Thompson, JC and Townsend CM. Endocrine Pancreas. Townsend: Sabiston Textbook of Surgery, 18th Ed. 2007.
• Taupenot, L. et al. The Chromogranin-Secretogranin Family. NEJM 2003 348 1194 492003;348:1194-49.
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