Pancreatic and Peripancreatic Neural Crest-Derived Tumors: An Updated Contemporary Single Center Experience

Schwannomas, paragangliomas, and gangliocytic paragangliomas are rare tumors of the pancreas and peripancreas. Diagnosis and management of these tumors remain challenging.

A retrospective review was conducted at a single institution. Nineteen cases were identified from 1990 to 2022. Descriptive statistics summarized the demographic, clinicopathologic, and long-term outcome data.

Schwannomas were encountered within the pancreatic parenchyma, whereas half of the paragangliomas were intraparenchymal and the other half were in the peripancreatic connective tissue. Symptoms varied and occurred in half of the patients encountered. Imaging characteristics were nonspecific. When obtained, EUS-FNA (n = 16) and nuclear imaging (n = 3) assisted in making a preoperative diagnosis. Intraparenchymal tumors were predominantly managed with a pancreatectomy (n = 12) while tumors in the peripancreatic connective tissue were managed with tumor resection without a pancreatectomy (n = 4). The median follow-up for schwannomas and gangliocytic paragangliomas was 66.8 and 13.6 months, respectively, during which time zero recurrences occurred. Paraganglioma patients had a median follow-up of 21.4 months, and tumor recurrence was encountered in 50% of patients.

Schwannomas and gangliocytic paragangliomas are benign tumors. Paragangliomas have malignant potential and should be managed with an oncologic resection. Accurate preoperative diagnosis using EUS-FNA and nuclear imaging is crucial for recommending the appropriate surgical intervention and minimizing surgical morbidity.