Objective:

To report a case of pancreatic acinar cell carcinoma (ACC) misdiagnosed as solid pseudopapillary neoplasm (SPN) and review existing literature.

Approach:

• Case Report: A 72-year-old female patient with elevated serum alpha-fetoprotein (AFP) levels and a pancreatic mass was initially misdiagnosed. After multidisciplinary team evaluation, she underwent radical Pancreaticoduodenectomy, confirming ACC postoperatively.

Key Findings:

• The patient had a serum AFP level of 2321.18 ng/mL.
• Imaging findings suggested a solid pseudopapillary neoplasm, leading to misdiagnosis.
• Final histopathological diagnosis confirmed pancreatic acinar cell carcinoma.

Interpretation:

Elevated serum AFP levels in patients with pancreatic masses should prompt consideration of ACC, especially when imaging features are misleading.

Limitations:

• The rarity of ACC limits the availability of standardized diagnostic and treatment guidelines.
• This case study is based on a single patient experience.

Conclusion:

Integrating tumor marker analysis, imaging findings, and multidisciplinary evaluation is essential to minimize preoperative misdiagnosis of rare pancreatic tumors.