https://imcjms.com Ibrahim Medical College Journal of Medical Science https://imcjms.com/registration/journal_full_text/59 Tue, 02 Aug 2016 11:33:56 +0000 Pancreatolithiasis is a risk factor for developing pancreatic cancer. We report here a rare case of sarcomatoid carcinoma of the pancreas in a 55-year old diabetic male associated with pancreatolithiasis. CT scan of abdomen revealed a large operable mass occupying the distal body and tail of the pancreas. Per-operative survey revealed a small metastatic nodule in the surface of hepatic segment IVa. Histopathology of the distal pancreatic lesion revealed sarcomatoid carcinoma. Hepatic nodule was a metastatic adenocarcinoma. Distal pancreatectomy and splenectomy was done en-mass, along with non-anatomical resection of the hepatic metastatic nodule. Combined with six cycles of chemotherapy, the patient survived a total of another fourteen months. Introduction   A 55 year male college teacher, known diabetic for 25 years and on insulin for 6 years, was diagnosed as a case of pancreatolithiasis for last two years. Pancreatolithiasis was diagnosed by plain X-ray of the abdomen which showed multiple large stones in the pancreas (Fig:1a). He presented to Hepato-Biliary out-patient department with occasional colicky pain in the left hypochondrium, anorexia and weight loss for last one year. His built and nutrition was average, but he was anxious for pain because it was hampering his regular activities. He was not anemic or icteric, and vital signs and symptoms were stable. Abdominal examination revealed mild tenderness at left hypochondrium with no organomegally or ascites. Haematological and biochemical parameters including liver function tests and pancreatic enzymes were within normal limits. Ultrasonography of the abdomen revealed pancreatolithiasis, dilated major pancreatic duct (main pancreatic duct diameter 17mm) and a mass lesion occupying the distal body and tail of the pancreas. Computerized tomography (CT) scan of abdomen showed a fairly large mass (6.5 X 5.0 X 5.0 cm) occupying the distal pancreas (Fig1b). CA 19-9 level was 183 u/L (n= <37 u/L). CT guided fine needle aspiration cytology (FNAC) was done and it was positive for malignant cells.   Fig. 1 a) Plain X-ray of abdomen showing pancreatolithiasis;  1 b) CT scan of abdomen showing distal pancreatic mass Our impression waspancreatolithiasis with a malignant lesion in the distal pancreas. Plan of management was to perform distal pancreatectomy and splenectomy en-mass along withpancreatolithotomy and Roux-en-Y pancreatojejunostomy. Patient was prepared accordingly and was vaccinated against Streptococcus pneumoniae, Haemophilus influenzae and Neisseria meningitides as per schedule.         First follow up was after one month and subsequently the patient was followed up for next one year. During this period he received six cycles of chemotherapy and was under the care of an oncologist. After one year, there was recurrence of the tumor in the operative bed. With consultation of the oncologist, chemotherapy was restarted, but the patient died of disseminated disease after two months. Discussion The overall survival for ACP is poor when compared with pancreatic duct adenocarcinomas (PDA) but several studies suggest that operative resection provide little benefit.11,12 A Mayo Clinic Institutional Review Board approved cohort study using the Surveillance, Epidemiology and End Results (SEER)cancer registry databasehas shown that males are more affected by ACP than females, the size of ACP is larger at presentation than other PDA (median tumor size 6.0 cm vs. 3.5 cm) and PDA are located more in the head region of the pancreas whereas ACP are located more distally in the body and tail region.13 However, local recurrence of the disease is very high in ACP and Strobel and colleagues (2011)5 has shown in a study that after curative resection of ACP, median survival is only 7.1 months. Yamaguchi et al. (1998) has shown a zero one-year survival for patients with ACP.12 Therefore, it is suggested that pre-diagnosed case of ACP should not undergo surgery. In our case, the decision of operation was influenced by FNAC report, but subtype categorization of pancreatic carcinoma was difficult with FNAC because of the small sample examined microscopically.14 Recently, Clark et al. (2012)11 has mentioned in his study that patients of ACP who underwent pancreatic resection had an overall survival comparable to patients with PDA. Therefore, they recommended that patients with ACP should be offered pancreatic resection when technically feasible. So the operation was justified in our case even with the hepatic metastasis and the patient got a total of fourteen months survival benefit.   1.    Rashid M, Ahmed T, Alam H et al. Evaluation, surgical approaches & results of treatment of pancreatolithiasis in 120 patients. J Surgical Scien 2006; 10: 21–26. 3.    Barretoa SG, Shuklab PJ and Shrikhandeb SV. Tumors of the Pancreatic Body and Tail. Cancer Facts & Figures. Atlanta, GA: American Cancer Society, 2004. 5.    Strobel O, HartwigW, Bergmann F et al. Anaplastic pancreatic cancer: presentation, surgical management, and outcome. Surgery 2011; 149: 200–208. 7.    Lewandrowski KB, Weston L, Dickersin GR et al. Giant cell tumor of the pancreas of mixed osteoclastic and pleomorphic cell type: evidence for a histogenetic relationship and mesenchymal differentiation. Hum Pathol 1990; 21: 1184–1187. 9.    Manduch M, Dexter DF, Jalink DW et al. Undifferentiated pancreatic carcinoma with osteoclast-like giant cells: report of a case with osteochondroid differentiation. Pathol Res Pract 2009; 205: 353–359. 11.  Clark CJ, Graham RP, Arun JS, Harmsen WS and Reid-Lombardo KM. Clinical Outcomes for Anaplastic Pancreatic Cancer: A Population-Based Study. J American Coll Surgeons 2012; 215: 627-634. 13.  Surveillance, Epidemiology and End Results (SEER). National Cancer Institute. Bethesda, MD. Available at: http://seer. cancer.gov. 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