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

Disclosures

April 19, 2004

Hospital Course

A multidisciplinary approach was necessary in deciding the management strategy for our patient, including the involvement of the endocrinologist, surgeon, radiologist, gastroenterologist, and the patient himself. Symptom control was the first goal of treatment. The patient received an intravenous PPI in high doses (pantoprazole, 80 mg intravenous, twice daily) to control gastric acid hypersecretion and aggressive potassium replacement, as well as ketoconazole and spironolactone for glucocorticoid and mineralocorticoid inhibition. He then underwent surgery with distal pancreatectomy, splenectomy, and bilateral adrenalectomy for control of his hypercortisolism and severe, refractory hypokalemia. At surgery, the patient was found to have extensive hepatic metastases, precluding hepatic resection and radiofrequency ablation. His postoperative course was uneventful. He was given octreotide for attempted tumor suppression and was referred to an oncology research center for chemotherapy on a protocol-directed basis.

The findings at surgery confirmed the diagnosis. The 5 x 3 cm mass in the pancreas was resected and is shown in Figure 13. A low-power view shows the typical small cell neuroendocrine tumor near normal-appearing pancreatic acinar cells (Figure 14).

High-power view shows the tumor cells invading neural tissue (Figure 15) and blood vessels (Figure 16). The tumor stained positive for both gastrin (Figure 17) and ACTH (Figure 18). One biopsy specimen from the liver shows the metastatic tumor (Figure 19).

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