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  • • No overt clinical syndrome

    • Pancreatic mass on CT scan

    • Elevated serum levels of chromogranin A, pancreatic polypeptide, and hCG

    • Overall worse prognosis than other pancreatic islet cell tumors

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Epidemiology

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  • • Account for 30-50% of pancreatic endocrine tumors

    • Elevated serum levels of chromogranin A, pancreatic polypeptide, and hCG are common, without any accompanying clinical syndrome

    • Most nonfunctioning tumors are large, malignant, and located in the head of the pancreas

    • Metastases are present at the time of diagnosis in 80% of patients

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Symptoms and Signs

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  • • Abdominal and back pain

    • Weight loss

    • Jaundice

    • Nausea and vomiting

    • Palpable abdominal mass

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Laboratory Findings

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  • • Elevated serum bilirubin

    • Elevated serum chromogranin A

    • Elevated serum pancreatic polypeptide

    • Elevated serum hCG

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Imaging Findings

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  • CT scan

    • -Shows a pancreatic mass, which is typically hypervascular and/or partially cystic with areas of calcification

      -Also useful for detection of metastases

    Octreotide scintigraphy may be useful

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  • • The histologic pattern on biopsy specimens is diagnostic of islet cell tumor, but whether or not the lesion is malignant rests on evidence of invasiveness or metastases, not the histology

    • Immunohistochemical staining of the tissue is positive for chromogranin and neuron-specific enolase (markers of APUD tumors)

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Rule Out

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  • • Functional islet cell tumors

    • Adenocarcinoma of the head of the pancreas

    • Chronic pancreatitis

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  • • CT scan or somatostatin receptor scintigraphy

    • Serum levels of chromogranin A, pancreatic polypeptide, and hCG

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When to Admit

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  • • Severe symptoms

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Surgery

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  • • Resection of primary tumor (head, pancreaticoduodenectomy; body and tail, distal pancreatectomy) and debulking of metastases

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Indications

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  • • All cases of completely respectable disease

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Medications

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  • • Streptozocin and doxorubicin for unresectable tumor

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Complications

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  • • Biliary obstruction

    • Duodenal obstruction

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Prognosis

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  • • 5-year disease-free survival rate is 15%

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References

Bartsch DK et al. Management of nonfunctioning islet cell carcinomas. World J Surg. 2000;24:1418.  [PubMed: 11038216]
Somogyi L, Mishra G. Diagnosis and staging of islet cell tumors of the pancreas. Curr Gastroenterol Rep. 2000;2:159.  [PubMed: 10981018]

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