Pancreatic schwannomas are extremely rare benign nerve-sheath tumors. Fewer than ~70 cases have been reported, and diagnostic accuracy prior to surgery is low. Therapeutic approaches remain highly heterogeneous in the literature.

We present the case of a 64-year-old female, who presented with left hypochondrial pain radiating posteriorly. MRI and EUS revealed a ~20 mm, well-circumscribed, hypoechoic, firm lesion at elastography in the pancreatic uncinate process. EUS-FNB biopsy demonstrated spindle-cell proliferation with typical Antoni A/B areas. Immunohistochemistry showed S100 and SOX10 positivity, with CD34, DOG1, GFAP, SMA, AE1/AE3 and chromogranin negativity, Ki-67 <1%, consistent with a pancreatic schwannoma.

Given the lesion’s small size, its benign histologic profile, and the absence of high-risk features, and considering that the only surgical option is a pancreaticoduodenectomy (Whipple procedure), the patient declined the surgical approach and instead opted to pursue structured imagistic and EUS surveillance.

Pancreatic schwannomas show variable imaging appearances and often mimic neuroendocrine tumors or solid-pseudopapillary neoplasms. Management strategies described in case series range from observation and enucleation to major resections, reflecting a lack of consensus.

This case highlights the rarity of pancreatic schwannoma and the discordance in therapeutic approaches. When histology confirms benign features, close imagistic and EUS follow-up may be a safe alternative to radical surgery, especially for small, well-delimited lesions.