Pancreatic schwannoma, an extremely rare and challenging entity: Report of two cases and review of literature.

Pancreatic schwannoma is a rare benign tumor, for which the preoperative and intraoperative definitive diagnosis is quite challenging. We present the clinical, radiological and pathologic features of two primary pancreatic schwannomas identified in our pathology database over a period of 30 years at our tertiary care hospital. To better understand the clinico-pathological and radiological features of this entity, we provide a comprehensive review of 73 cases described in the English literature, along with our two cases. This review will especially focus on preoperative and intraoperative diagnosis to assess their accuracy for pancreatic schwannoma. The three most common preoperative diagnoses based on imaging for pancreatic schwannomas were cystic neoplasm (56%), pancreatic neuroendocrine tumor (29%) and mucinous cystic neoplasm (26%). Imaging could not definitely diagnose pancreatic schwannoma in any of the reported cases. To obtain a definite diagnosis before surgery, 25 cases underwent imaging-guided fine-needle aspiration (FNA)/biopsy, of which 60% were correctly reported as benign with definite diagnosis of pancreatic schwannoma in 48%. A higher diagnostic accuracy was observed in biopsies (71%) than FNA (37%). In addition, an intraoperative frozen section was carried out in 15 cases, and 47% were correctly diagnosed. Despite relatively low accuracy, preoperative histological assessment can be helpful in surgical managment. A core tissue specimen is recommended to improve the diagnostic accuracy in this setting.