A Rare Cause of Abdominal Pain by Pancreatic Mass in a Young Female Patient

Abstract

Question: A 24-year-old woman presented with epigastric pain which began about 2 years ago and progressively worsened over the past 6 months. The pain occurred intermittently but usually began after a meal, lasted for about 1 hour, and radiated to the back. The patient said that she also had lost 4 kg during the past 3 months. Her past medical history was unremarkable and physical examination was positive only for mild epigastric tenderness. Laboratory test results at admission were as follows: amylase, 208 U/L; lipase, 261 U/L; total serum bilirubin, 0.48 mg/dL; direct serum bilirubin, 0.1 mg/dL; aspartate transaminase, 14 IU/L; alanine transaminase, 6 IU/L; alkaline phosphatase, 49IU/L; γ-glutamyl transferase, 12 IU/L; carbohydrate antigen 19-9, 7.7 IU/mL; α-fetoprotein, 1.0 ng/mL; carcinoembryonic antigen, 1.0 ng/mL; and cancer antigen 125, 7.1 U/mL. To evaluate the cause of epigastric pain and weight loss, an abdominal CT was taken and revealed a 4.8-cm mass in the tail of the pancreas. On pre–contrast-enhanced image, the hypoattenuated central portion had a focal high attenuated lesion (white arrow), suggesting the presence of both hemorrhage and necrosis (Figure A). This pancreatic mass showed peripheral enhancement as it progressed from the arterial phase (Figure B) to the portal phase (Figure C). In addition, there was infiltration around the pancreatic mass (the splenic vein was also obstructed by the mass, evidenced by the presence of gastric fundal varix formation (black arrow; Figure D). Laparoscopic distal pancreatectomy and splenectomy was performed on this pancreatic mass. What is the diagnosis in this young female patient? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. Initial impression of the lesion was a solid and pseudopapillary tumor considering the patient’s age and gender. However, the histopathologic result was compatible with carcinosarcoma of the pancreas. The resected mass measured 4.7 × 3.5 cm in size, had necrotic portion in the center, and extended to the peripancreatic soft tissue. Along with neural and vascular invasion, metastasis was also present in 6 out of 8 lymph nodes. The tumor was poorly demarcated and was composed of oval to spindle-shaped cells with large pleomorphic nuclei (Figure E [stain: hematoxylin and eosin; original magnification, ×20]; Figure F [stain: hematoxylin and eosin; original magnification, ×400). Immunohistochemical staining was positive for cytokeratin (Figure G; stain: cytokeratin; original magnification, ×400) and vimentin (Figure H; stain: vimentin; original magnification, ×400). Based on these histopathologic examination and immunohistochemical staining pattern, the patient was diagnosed with carcinosarcoma of the pancreas. Carcinosarcoma of the pancreas is an extremely rare neoplasm of the pancreas and only several cases have been reported in the English literature with most cases occurring in middle-aged (48 years) or elderly patients (66–90 years).1Kane J.R. Laskin W.B. Matkowskyj K.A. et al.Sarcomatoid (spindle cell) carcinoma of the pancreas: A case report and review of the literature.Oncol Lett. 2014; 7: 245-249PubMed Google Scholar, 2Gelos M. Behringer D. Philippou S. et al.Pancreatic carcinosarcoma. Case report of multimodal therapy and review of the literature.JOP. 2008; 9: 50-55PubMed Google Scholar In the present case, however, carcinosarcoma occurred in a young 24-year-old woman, which to the best of our knowledge is the first reported case in this age group. Although histogenesis of carcinosarcoma of the pancreas is not clearly understood, there are 3 proposed mechanisms that explain the presence of both the carcinomatous and sarcomatous components in the same tumor: transformation, combination, and collision.3Yao J. Qian J.J. Zhu C.R. et al.Laparoscopic left pancreatectomy for pancreatic sarcomatoid carcinoma: A case report and review of the literature.Oncol Lett. 2013; 6: 568-570PubMed Google Scholar Based on the limited number of case reports, the prognosis of pancreatic carcinosarcoma is very poor. Although no randomized, controlled trial to determine the efficacy of surgery for this condition has been performed, Gelos et al2Gelos M. Behringer D. Philippou S. et al.Pancreatic carcinosarcoma. Case report of multimodal therapy and review of the literature.JOP. 2008; 9: 50-55PubMed Google Scholar identified that the average postoperative survival interval was 6 months. A standard chemotherapy protocol has also not yet been developed.