Abstract
Pancreatic carcinosarcoma is a rare and aggressive malignancy that can mimic pancreatic adenocarcinomas in presentation but often has different disease biology and different responses to conventional treatment for pancreatic adenocarcinoma. Case reports have documented a 5-year overall survival of approximately 13% only if the disease is caught at an earlier stage and is amenable to multi-modality treatment, including surgery, chemotherapy, and radiation. In the advanced stage, treatments do not often provide benefit, and patients may decline rapidly. There are currently no studies demonstrating survival benefits with chemotherapy in patients with metastatic carcinosarcoma, owing to both the rarity and the often late diagnosis of this aggressive entity. We present a case of a 71-year-old male patient diagnosed with metastatic pancreatic carcinosarcoma who received four lines of palliative-intent treatment: gemcitabine and nab-paclitaxel, modified FOLFIRINOX, GTX, and doxorubicin. With careful selection of chemotherapeutic regimen as well as his ability to tolerate four lines of treatment, this resulted in an unprecedented 26-month survival. We also reviewed the literature on the histopathology, diagnosis, and treatment of this rare entity.