Introduction Serous cystadenocarcinoma (SCAc) of the pancreas is the rare malignant counterpart of serous cystadenoma, with fewer than 40 reported cases. Its clinical behavior and optimal management remain poorly defined. This systematic review summarizes the presentation, diagnosis, treatment, and outcomes of pancreatic SCAc. Methods A systematic search of PubMed, Scopus, Web of Science, and Embase was performed from inception through December 2025. Thirty-six studies describing 38 histologically confirmed cases met inclusion criteria. Data on demographics, tumor characteristics, imaging, management, and outcomes were extracted and summarized descriptively. Results Median age at diagnosis was 66.5 years (IQR 55.8-71.0), and 71% of patients were female. Abdominal pain and incidental imaging findings were the most common presentations. Tumors were typically large and located in the pancreatic body or tail. Local invasion occurred in 74% of cases. Distant metastases were present in 68% (26 of 38), most commonly to the liver. All patients underwent surgical resection. Chemotherapy use was rare. Recurrence was reported in 26% of cases, with a median disease-free interval of roughly 50 months. Disease-related mortality was infrequently reported. Conclusions Despite frequent invasion and metastasis, SCAc often demonstrates an indolent course, and long-term survival is common after resection. Surgical management remains central to treatment. Given its rarity and diagnostic limitations, individualized management and prolonged surveillance are warranted.
Wilhelm et al. (Tue,) studied this question.