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INTRODUCTION: Primary signet ring cell carcinoma of the pancreas (PSRCCP) is an extremely rare histologic variant of pancreatic carcinoma, which cells are characterized by large mucin vacuoles and peripheric nucleus that producing a signet ring aspect.
CASE REPORT: We present a case about 66 years old man, with history of chronic pancreatitis and Wirsung stenosis of unknown origin, came to our attention for abdominal pain compatible with pancreatitis exacerbation. A TC scan showed a head of pancreas’ neoformation, and a MRI showed numerous metastatic liver lesions confirming the presence of pancreatic lesion (52×46) mm. Fine needle aspiration biopsy of liver was suggestive of signet ring cell carcinoma. As second step, an ERCP was performed in order to get a biopsy of pancreatic lesion and drain the biliary tract. No surgical approach was possible as well as the progressive complications arised after diagnosis contraindicated chemotherapy. The patient died 3 month after diagnosis.
CONCLUSION: PSRCCP is an aggressive malignancy with low survival rate, because of high metastatization rate. Only 9 cases are described in literature at December 2019, so we consider our work a precious contribute to knowledge.