Pancreatic endocrine tumors: a report on a patient treated with sorafenib

Abstract

A 31-yr-old man with abdominal pain was diagnosed with a pancreatic endocrine tumor and multiple hepatic metastases. Despite optimal treatment with interferon alpha, a somatostatin analog, local therapy with high-intensity focused ultrasound ablation for multiple hepatic metastases, and multiple lines of chemotherapy with etoposide/cisplatin combination chemotherapy and gemcitabine monotherapy, the tumor progressed. As few chemotherapeutic options were available for him, sorafenib (800 mg/day, daily) was administered as a salvage regimen. Sorafenib was continued despite two episodes of grade 3 skin toxicity; it delayed tumor progression compared to the previous immunotherapy and chemotherapy. Serial computed tomography scans showed that the primary and metastatic tumors were stable. Thirteen months after beginning targeted therapy, and up to the time of this report, the patient is well without disease progression. We suggest that sorafenib is effective against pancreatic endocrine tumors.

Keywords: Chemotherapy; Neuroendocrine Tumors; Sorafenib.

Fig. 1

Radiologic images of pancreatic, non-functioning, well-differentiated, endocrine carcinoma and multiple liver metastases. (A) Contrast enhanced CT scan (02/19/2009) shows a 6.8 cm lobulated mass in the uncinated process of the pancreas (arrow). The tumor shows focal cystic and necrotic areas, and heterogeneous enhancement. Multiple hematogenous metastases are also noted (arrowheads). (B) Follow-up contrast enhanced CT scan (03/31/2009) shows the lobulated mass in the uncinated process measuring 5.4 cm in maximal diameter. A comparison with the previous CT scan (A) reveals interval partial regression of the primary tumor mass in the uncinate process (arrow) and hematogenous hepatic metastases (arrowheads).

Fig. 2

Pathologic findings of the pancreatic, non-functioning, well-differentiated, endocrine carcinoma. (A) Histologic examination shows that the tumor cells were arranged in trabeculae and solid nests separated by a fibrous or loose fibrovascular stroma (H&E, × 100). (B) All tumor cells were large and polygonal in shape and had abundant, eosinophilic, and finely granular cytoplasm containing round to oval nuclei with finely stippled chromatin (H&E, × 400). (C) Tumor cells were strongly positive for chromogranin (IHC, × 400).