Published online Sep 26, 2021. doi: 10.12998/wjcc.v9.i27.8071
Peer-review started: February 24, 2021
First decision: May 6, 2021
Revised: May 19, 2021
Accepted: August 5, 2021
Article in press: August 5, 2021
Published online: September 26, 2021
Primary pancreatic paragangliomas are extremely rare tumors. Limited by the diagnostic efficacy of histopathological examination, their malignant behavior is thought to be associated with local invasion or metastasis, with only four malignant cases reported in the literature to date. As pancreatic paragangliomas share similar imaging features with other types of pancreatic neuroendocrine neoplasms, they are difficult to diagnose accurately without the support of pathological evidence. As primary pancreatic paragangliomas are rare, especially those accompanied by lymph node metastasis, there is currently no consensus on treatment. Herein, we report a case of primary pancreatic paraganglioma with lymph node metastasis.
A mass located in the pancreatic body was incidentally discovered on computed tomography in a 41-year-old Tibetan man. Distal pancreatectomy was subsequently performed and a 4.1 cm × 4.2 cm tumor was found embedded in the body of the pancreas during surgery. Histological examination confirmed the characteristics of paraganglioma in which the neoplastic chief cells were arranged in a classic Zellballen pattern under hematoxylin-eosin staining. Further, immunohistochemistry demonstrated that the sustentacular cells in the tumor tissue were positive for S-100 protein, and neoplastic cells and pancreatic draining lymph nodes were positive for chromogranin A and synaptophysin; thus, the presence of lymph node metastasis (two of the eight resected pancreatic draining lymph nodes) was also confirmed. A diagnosis of primary pancreatic paraganglioma with lymph node metastasis was finally established. The patient remained disease-free for 1 year after the surgery.
A definite diagnosis of pancreatic paraganglioma mainly depends on posto
Core Tip: Paragangliomas are rare neuroendocrine tumors that originate from autonomic nervous systems, with a morbidity of only 2 to 8 in 1000000 of the population. Paragangliomas arising from the pancreas are even less, local invasion or metastasis are thought to be associated with malignant behaviors, and only four of these cases have been reported to date. It is difficult to make an accurate preoperative diagnosis of pancreatic paraganglioma especially when the tumor is non-functioning. No standard therapeutic consensus has been reached yet. Herein, we present a case of primary pancreatic paraganglioma with lymph node metastasis and discuss the related issues.