Published online Oct 7, 2021. doi: 10.3748/wjg.v27.i37.6322
Peer-review started: April 6, 2021
First decision: June 17, 2021
Revised: June 28, 2021
Accepted: September 3, 2021
Article in press: September 3, 2021
Published online: October 7, 2021
Pancreatic paragangliomas (PPGL) are rare benign neuroendocrine neoplasms but malignancy can occur. PPGL are often misdiagnosed as pancreatic neuroendocrine tumor or pancreatic adenocarcinoma.
We reviewed 47 case reports of PPGL published in PubMed to date. Fifteen patients (15/47) with PPGL underwent endoscopic ultrasound-guided fine needle aspiration (EUS-FNA). Only six (6/15) were correctly diagnosed as PPGL. All patients with PPGL underwent surgical resection except three (one patient surgery was aborted because of hypertensive crisis, two patients had metastasis or involvement of major vessels). Our patient remained on close surveillance as she was asymptomatic.
Accurate preoperative diagnosis of PPGL can be safely achieved by EUS-FNA with immunohistochemistry. Multidisciplinary team approach should be consi
Core Tip: The morphologic overlap between pancreatic paraganglioma and neuroendocrine tumor is significant. An accurate diagnosis by endoscopic ultrasound-guided fine needle aspiration requires firstly that the possibility of paraganglioma is considered and secondly that a cell block is available for immunohistochemical stains. A patient-centered approach supported by a multidisciplinary team of radiologists, advanced endoscopists, endocrinologists, pathologists, oncologists, and surgeons is paramount in the management of pancreatic paraganglioma.