Primary pancreatic signet ring cell carcinoma: a case series from China and literature review.

OBJECTIVE

To report on cases of primary pancreatic signet ring cell carcinoma (PSRCC) from our institution, review the published literature, and evaluate the clinicopathological characteristics.

MATERIAL AND METHODS

We present a recent case of PSRCC and report 11 additional cases from China, utilizing the China National Knowledge Infrastructure (CNKI) database. The literature search encompassed publications from January 1994 to December 2023. We also performed a literature search using PubMed and Web of Science to identify cases of PSRCC outside of China and identified a total of 13 additional cases.

RESULTS

A total of 12 cases from China were identified, with a mean age of 64 years. Among these, 11 patients (91.7%) were male, and 1 patient (8.3%) was female. The tumor was primarily located in the pancreatic head (7 cases, 57.3%), followed by the pancreatic body (3 cases, 25.0%) and tail (2 cases, 16.7%). Six patients (50%) underwent radical curative surgery, while the remaining 6 (50%) received palliative surgical treatment or chemotherapy. In the radical curative surgery group (6 patients), the longest follow-up was 23 months, with only one death reported. In contrast, all patients who received palliative treatment (6 patients) experienced short-term mortality, with the longest follow-up period being 5 months. While most patients were Chinese, similar outcomes have been reported in non-Chinese populations, indicating broader applicability.

CONCLUSION

PSRCC is a highly aggressive and extremely rare pancreatic malignancy, typically originating in the head of the pancreas. Patients diagnosed at an early stage and who undergo radical surgery demonstrate significantly prolonged survival.