Pancreatic paraganglioma mimicking pancreatic neuroendocrine tumor

Abstract

Extra-adrenal paragangliomas are rare tumors arising from the chromaffin cells of the autonomic nervous system. Retroperitoneal paragangliomas may present as a pancreatic mass. We present a case of a 61-year-old woman with an incidentally found pancreatic mass (7.2 × 6.5 cm) in the CT scan. EUS- guided FNA result was compatible with pancreatic neuroendocrine tumor. Patient underwent pancreaticoduodenectomy and histopathologic assessment revealed the mass was an extra-adrenal paraganglioma. Preoperative diagnosis of pancreatic paragangliomas can be challenging due to imaging and histopathologic similarities with pancreatic neuroendocrine tumors.

Primary pancreatic paraganglioma harboring lymph node metastasis: A case report

“…Paragangliomas that arise from the adrenal medulla are referred to as pheochromocytomas, which are the most common type of paraganglioma[ 23 ]. The majority of extra-adrenal paragangliomas tend to originate in the middle ear, jugular foramen, carotid body, aorticopulmonary region, posterior mediastinum, and para-aortic region (especially Zuckerkandl’s body)[ 4 ].…”

Peripancreatic paraganglioma: A diagnostic dilemma resolved on endoscopic ultrasound guided fine needle aspiration cytology

“…5,6 There are limited case reports describing cytopathologic diagnosis of paraganglioma. 7 Majority of case reports have suggested a diagnosis of atypical epithelial cells, 8 poorly differentiated carcinoma, 9 or neuroendocrine tumour 10 on EUS FNAC of pancreatic/peripancreatic PG. The overlapping cytomorphology and immunomarker expression creates a fertile ground for diagnostic errors.…”

Pheochromocytoma presenting as a pancreatic mass: Avoiding a dangerous pitfall in endoscopic ultrasound‐guided fine needle aspiration biopsy using GATA3 immunostain

“…1,7 There are a number of reports of cytological diagnosis of peripancreatic paraganglioma (PP), with a high rate of alternative diagnoses: pancreatic neuroendocrine tumour (PanNET), various carcinomas (adenocarcinoma, acinar cell carcinoma, neuroendocrine carcinoma, renal cell carcinoma), spindle cell neoplasms, and pseudocysts. [1][2][3]7,8 There are so far no cytopathological examples of primary pheochromocytoma presenting as a pancreatic tumour. One cytopathological diagnosis of a "neuroendocrine tumour, pancreatic?"…”

“…Sympathetic paragangliomas are rare tumours arising from chromaffin cells of the autonomic nervous system, distributed along the paravertebral axis in the thorax, abdomen, and pelvis, producing catecholamines in various amounts. [1][2][3] An intra-adrenal sympathetic paraganglioma is designated a pheochromocytoma. 4,5 The two entities share similar clinical, morphological, and immunohistochemical features.…”

“…The tumour morphology can be very pleomorphic, without necessarily indicating a metastatic spread, making an exact cytopathological diagnosis challenging 1,7 . There are a number of reports of cytological diagnosis of peripancreatic paraganglioma (PP), with a high rate of alternative diagnoses: pancreatic neuroendocrine tumour (PanNET), various carcinomas (adenocarcinoma, acinar cell carcinoma, neuroendocrine carcinoma, renal cell carcinoma), spindle cell neoplasms, and pseudocysts 1‐3,7,8 . There are so far no cytopathological examples of primary pheochromocytoma presenting as a pancreatic tumour.…”