Preoperative Diagnosis of Abdominal Extra-Adrenal Paragangliomas with Fine-Needle Biopsy

Abstract

Paragangliomas are rare, non-epithelial neuroendocrine neoplasms originating in paraganglia, for instance the adrenal medulla, or at extra-adrenal locations. The aim of this study was to review the literature regarding abdominal extra-adrenal paragangliomas diagnosed pre-operatively with fine-needle biopsy (FNA and/or FNB). The PubMed database was searched to identify such cases, using a specific algorithm and inclusion/exclusion criteria. An unpublished case from our practice was also added to the rest of the data, resulting in a total of 36 cases for analysis. Overall, 24 (67%) lesions were found in females, whereas 12 (33%) in males. Most (21/36; 58.33%) were identified around and/or within the pancreatic parenchyma. FNA and/or FNB reached or suggested a paraganglioma diagnosis in 17/36 cases (47.22%). Of the preoperative misdiagnoses, the most common was an epithelial neuroendocrine tumor (NET). Regarding follow-up, most patients were alive with no reported recurrence; however, 5/36 patients exhibited a recurrence or a widespread disease, whereas one patient died 48 months following her diagnosis. In two patients, transient hypertension was reported during the EUS-FNA procedure. In conclusion, this study showed that the preoperative diagnosis of these lesions is feasible and, while diagnostic pitfalls exist, they could significantly be avoided with the application of immunochemistry.

Visual analytics identifies key miRNAs for differentiating peripancreatic paraganglioma and pancreatic neuroendocrine tumors

“…Singhi et al ( 6 ), reported that 3 of 9 (33%) of patients diagnosed with peripancreatic PGL developed metastases within 5 years of surgery, and 2 of them died because of their disease. In another report ( 47 ), 13.9% exhibited a recurrence or widespread disease and one patient died 48 months following diagnosis. Additionally, it is important to note that more than 40% of abdominal PGLs carry a germline pathogenic gene variant, which has significant clinical implications for personalized surveillance and treatment and for the patient’s family.…”

“…Retroperitoneal PGLs, although rare, can occur in the vicinity of or within the pancreas, resembling primary pancreatic lesions. To date, literature records have documented approximately 56 peripancreatic PGLs (2,6,(40)(41)(42)(43)(44)(45)(46). Distinguishing between PANNETs and PGLs is crucial for appropriate patient management, both preoperatively and postoperatively.…”