Authors: Venu Julapalli, MD; Mehnaz Shafi, MD; Jim Schwartz, MDSeries Editor: Richard Goodgame


April 19, 2004

Further Evaluation

The fasting serum gastrin level was 550 pg/mL. In response to secretin administration, the following increases were recorded: at 10 minutes, 785 pg/mL; at 20 minutes, 841 pg/mL; at 25 minutes, 794, pg/mL; at 35 minutes, 757 pg/mL; and at 40 minutes, 705 pg/mL. Adrenal hormone levels were also elevated: androstenedione, 7.2 mcmol/L; and dehydroepiandrosterone, 11.8 mcmol/L. All other hormone levels were within the normal range: insulin, 11 mcU/mL; glucagons, 120 pg/mL; pancreatic polypeptide, 159 pg/mL; vasoactive intestinal peptide (VIP), 10 pg/mL; intact parathyroid hormone, 31 pg/mL; calcitonin, < 1 ng/L; renin, 1.8 ng/mL; aldosterone, 3.2 ng/dL; prolactin, 12 ng/mL; growth hormone, 0.47 ng/mL; and thyroid-stimulating hormone, 0.52 mcU/mL.

Results of all brain imaging studies were normal. An octreotide scan (somatostatin receptor scintigraphy) was performed and tracer accumulated in the pancreas and also in the liver (Figure 7). The CT scan showed the pancreatic tumor (Figure 8). MRI showed both the primary pancreatic tumor (Figure 9) and small, very vascular hepatic metastases (Figure 10). An endoscopic ultrasound demonstrated the pancreatic tumor (Figure 11). Fine-needle aspiration of the tumor showed a neuroendocrine morphology (Figure 12) that stained positive for synaptophysin and chromogranin, 2 generic neuroendocrine immunohistochemical markers.

Based on the above evaluation, the diagnosis was evident: a malignant sporadic gastrin- and ACTH-producing neuroendocrine tumor of the pancreas with metastases to the liver and severe Cushing's syndrome and difficult-to-manage ulcer diathesis.

What is the role of each of the following strategies in the management of this patient's illness?

  • Surgical removal of the primary tumor

  • Surgical removal of the hepatic metastases

  • Surgical adrenalectomy

  • Octreotide

  • Ketoconazole and spironolactone

  • PPIs

  • Anticancer chemotherapy

  • Radiation therapy

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