A Long-term Survivor after Pancreaticoduodenectomy for Metastatic Undifferentiated Carcinoma of an Unknown Primary

Undifferentiated carcinoma of unknown primary (CUP) is a relatively rare disease and carries a dismal prognosis. With this context, there is no report of a long-term (>5-year) disease-free survivor after a pancreaticoduodenectomy (PD). A 53-year-old female had complaints of intermittent high fever and a large palpable epigastric tumor. A CT scan revealed that a very large well-circumscribed solid mass occupied the posterior portion of the head of the pancreas and extended to the hepatic hilum. The serum carcinoembryonic antigen (CEA), CA-125 and carbohydrate antigen 19-9 (CA19-9) levels were within the normal ranges. A PD with hepatic arterial reconstruction was performed. Histologically, the tumor showed solid or diffuse proliferation of pleomorphic cells, and the immunohistochemistry suggested a metastatic tumor originating from the epithelium, the primary site was not determined despite of full review of all metastatic lesions to pancreas handled surgically and non-surgically. Adjuvant oral chemotherapy of UFT and cimetidine continued for 3 years and the patient alive and healthy 6 years after surgery. Even for dismal prognostic undifferentiated CUP, radical surgery would be an effective component of multidisciplinary treatment, provided that the tumor is respectable without elevation of serum tumor markers and adjuvant chemotherapy is able to appropriately supplement.