Pancreatic Islet Cell Tumors.ppt Islet_Cell_Tumor… · • Pancreatic Islet cell tumorsPancreatic...

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Management of Pancreatic Islet Cell Tumors

Ravi Dhanisetty, MDNovember 5, 2009

Morbidity and Mortality Conference

www.downstatesurgery.org

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

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.

MRIMRIwww.downstatesurgery.org

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.

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

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.

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.

Management of Pancreatic Islet Cell Tumors

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.

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.

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

General ManagementGeneral Management

• Diagnosis of particular functioning tumorDiagnosis of particular functioning tumor.• Localization of the neoplasm.

• Surgical resection if feasible. • Palliation of symptoms.

Efficacy of Localization of Tumors f th Pof the Pancreas

MODALITY TRUE POSITIVES (%)Noninvasive

Ultrasonography 23

Octreotide radioimaging (SRS)[*] 86Octreotide radioimaging (SRS) 86

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

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

InsulinomaInsulinoma• Localization – CT or

EUSEUS• Octreotide scanning

not useful.• Transhepatic

selective venous sampling

• Exploration with intra-operative ultrasound.

GlucagonomaGlucagonoma

• Fifth decade of lifeFifth decade of life• Rare – 4D syndrome, necrolytic migratory

erythemaerythemaGlucagon level > 500 pg/ml.

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.

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.

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.

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.

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.

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

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.)

Metastatic Disease

Surgical resection confers survival advantage and relief from symptoms.

• Mansour & Chen, 2004.

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

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.

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.

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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