An uncommon intersection: Familial adenomatous polyposis and solid pseudopapillary neoplasm of the pancreas: A case report and review of the literature

The co-occurrence of both Familial Adenomatous Polyposis (FAP) and Solid Pseudopapillary Neoplasms (SPN) of the pancreas is extremely uncommon, with limited reports published in the literature. FAP is a rare inherited disorder caused by a mutation in the adenomatous polyposis coli (APC) gene, while SPN is generally a low-grade malignant pancreatic lesion. We present the case of a 33-year-old female with a familial history of FAP, who initially presented with breast fibromatoses and was subsequently found to have colonic polyps, consistent with FAP, along with rare events like pancreatic SPNs in the head and tail of the pancreas and large desmoid tumors. It is a unique case that has never been reported in the literature and we provide findings of computed tomography (CT) and volume rendering to correlate the radiological features with pathology for an optimized diagnosis.