Undifferentiated Carcinoma of the Pancreas With Osteoclast-Like Giant Cells: A Comprehensive Review of Clinical Features, Molecular Pathology, and Therapeutic Advances

Undifferentiated carcinoma of the pancreas with osteoclast-like giant cells is a rare variant of malignant pancreatic tumor, accounting for less than 1% of all pancreatic tumors. The efficacy of neoadjuvant and adjuvant chemotherapy in patients with undifferentiated carcinoma of the pancreas with osteoclast-like giant cells remains unclear. Preliminary case reports have suggested pembrolizumab may be effective in some metastatic patients, though this requires validation in larger studies. Furthermore, patients with pure undifferentiated carcinoma of the pancreas with osteoclast-like giant cells demonstrate better overall survival rates than those with mixed-type undifferentiated carcinoma of the pancreas with osteoclast-like giant cells containing a pancreatic ductal adenocarcinoma histopathological subtype. Programmed cell death ligand-1 (PD-L1) expression has been identified as a significant determinant that shortens survival in patients with undifferentiated carcinoma of the pancreas with osteoclast-like giant cells, suggesting that anti-PD-1/PD-L1 agents may represent a potential therapeutic approach for this tumor type. This article reviews recent advances in the diagnosis, differential diagnosis, treatment, and prognosis of undifferentiated carcinoma of the pancreas with osteoclast-like giant cells to enhance understanding of this rare tumor entity.