Pancreatic Carcinosarcoma with a Rare Presentation

Introduction/Objective Carcino-sarcoma of the pancreas is a rare tumor and very limited clinical and pathologic data have been reported in the literature. As per World Health Organization (WHO) classification of tumors of the digestive system, the carcinosarcoma of the pancreas is classified together with sarcomatoid carcinoma and anaplastic giant cell carcinomas in undifferentiated carcinoma of pancreas. Carcinosarcoma is a rare entity with very poor prognosis. Methods/Case Report Here we report a rare case of pancreatic carcinosarcoma in a 68 year old male patient who underwent a pancreatoduodenectomy for unilocular cystic mass in the head of the pancreas. The mass grossly replaces the whole head of the pancreas. Histologically, the lesion showed a high-grade spindle cell component and adjacent moderately differentiated adenocarcinoma. On immunohistochemical examination, the carcinomatous component was positive for epithelial markers, and the sarcomatous component was positive for DOG1 and had a focal cytoplasmic staining for S-100. The diagnosis of pancreatic carcinosarcoma was rendered. Treatment options are same as of pancreatic carcinoma. Surgical resection is the best option available for patients. Systemic chemotherapy is indicated for patients with distant metastasis or patients with other contraindications. Despite surgery and adjuvant chemotherapy, recurrence rates are high, and prognosis is poor. However, there are no relevant standard chemotherapies available. Based on the limited number of reported cases, the prognosis of carcinosarcoma of the pancreas appears to be poor. But some cases with long term survival have been reported. There are very few primary pancreatic neoplasms with carcinomatous and sarcomatous components reported in the current literature. Results (if a Case Study enter NA) NA Conclusion This case highlights the importance of familiarity with histopathology of this rare entity, and to order proper immunohistochemical and molecular work-up when there is a suspicious abnormal spindle cell component.