Purpose: Pancreatic ductal adenocarcinoma (PDAC) is an aggressive malignancy with poor response to chemotherapy. High-frequency microsatellite instability (MSI-H) is a rare biological phenomenon in conventional PDAC, being more frequently described in tumors with medullary or mucinous features.

Methods And Results: In this manuscript, we report the case of a patient with an MSI-H pancreatic carcinoma with medullary features (medullary carcinoma of the pancreas-MCP) that achieved a complete pathological response after neoadjuvant modified FOLFIRINOX. Additionally, we summarize the available evidence on the clinical, pathological, and molecular features of patients with MCP, along with survival outcomes.

Conclusions: MCPs present significant sensitivity not only to immune checkpoint inhibitors, but also to systemic chemotherapy and that the latter treatment modality should not be overlooked. They also present different pathological and molecular features compared with conventional PDAC, meaning they should be considered a separate pathological entity.

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http://dx.doi.org/10.1007/s12029-024-01140-5DOI Listing

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