Primary Signet Ring Cell Carcinoma of the Pancreas with a Good Response to Chemotherapy: Case Report and Literature Review

Purpose Although pancreatic cancer is a common malignancy, signet ring cell carcinoma of the pancreas is a very rare histologic type with only 6 cases reported so far. We present a patient with primary signet ring cell carcinoma of the pancreas and a good response to neoadjuvant chemotherapy. Case report A 67-year-old woman presented at a regional hospital with a 2-week history of painless progressive jaundice. Abdominal computed tomography showed a tumor in the head of the pancreas, 4.5 cm in largest diameter. Since endoscopic biopsy and biliary stenting were not available, the patient had undergone palliative choledochoduodenostomy and tru-cut needle biopsy of the lesion. Histopathology revealed primary signet ring cell carcinoma of the pancreas. The tumor was considered borderline resectable and 3-month neoadjuvant chemotherapy with gemcitabine alone was administered due to the low creatinine clearance rates. Follow-up abdominal scan demonstrated very good response to chemotherapy and significant tumor regression to 1.5 cm in largest diameter. Radical cephalic duodenopancreatectomy was performed. Conclusion Significant tumor downsizing in our patient clearly demonstrates good response to neoadjuvant gemcitabine monotherapy. The optimal preoperative chemotherapy leading to tumor reduction and disease control in patients with borderline resectable and locally advanced pancreatic carcinoma is controversial, necessitating further randomized studies including combined chemoradiotherapy and multidrug combination regimens.