Pancreatic gangliocytic paraganglioma harboring lymph node metastasis: a case report and literature review

Abstract

BackgroundGangliocytic paraganglioma (GP) is a rare neuroendocrine neoplasm, which occurs mostly in the periampullary portion of the duodenum; the majority of the reported cases of duodenal GP has been of benign nature with a low incidence of regional lymph node metastasis. GP arising from the pancreas is extremely rare. To date, only three cases have been reported and its clinical characteristics are largely unknown.Case presentationA nodule located in the pancreatic head was incidentally detected in an asymptomatic 68-year-old woman. Computed tomography revealed 18-, 8-, and 12-mm masses in the pancreatic head, the pancreatic tail, and the left adrenal gland, respectively. Subsequent genetic examination revealed an absence of mutations in the MEN1 and VHL genes. Macroscopically, the tumor located in the pancreatic head was 22 mm in size and displayed an ill-circumscribed margin along with yellowish-white color. Microscopically, it was composed of three cell components: epithelioid cells, ganglion-like cells, and spindle cells, which led to the diagnosis of GP. The tumor was accompanied by a peripancreatic lymph node metastasis. The tumor in the pancreatic tail was histologically classified as a neuroendocrine tumor (NET) G1 (grade 1, WHO 2010), whereas the tumor in the left adrenal gland was identified as an adrenocortical adenoma. The patient was disease-free at the 12-month follow-up examination.ConclusionsPancreatic GP is associated with a higher incidence of metastasis and larger tumor size than duodenal GPs, suggesting that the primary organ of GP is an important prognostic factor.

Rectal Paraganglioma

“…Among the published reports of rare primary sites, cases of pancreas (12) and primary cases of the mesenterium (13) are found. We found only one case of rectal primary disease (11).…”

Diagnosis, Pathological Findings, and Clinical Management of Gangliocytic Paraganglioma: A Systematic Review

“…Gangliocytic paraganglioma (GP) is considered a rare neuroendocrine tumor (NET) that typically arises in the second part of the duodenum ( 1 ). Although a few investigators have reported cases of liver metastasis ( 2 – 4 ) and, in one case, a fatal duodenal GP ( 4 ), this tumor is usually associated with an excellent prognosis ( 5 ).…”

Primary pancreatic paraganglioma harboring lymph node metastasis: A case report

“…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 ]. Primary paragangliomas derived from the pancreas are particularly rare, and according to our comprehensive literature review, only 19 cases have been reported worldwide from 1998 till today[ 1 , 5 - 20 ]. Three patients showed evidence of lymph node metastasis[ 10 , 11 , 20 ] or invasion of vascular and peripancreatic adipose tissue[ 20 ], and one patient showed multiple liver metastases[ 14 ].…”

“…Extra-adrenal paragangliomas can arise at sites where paraganglia are known to occur, including the parasympathetic nervous system of the head and neck, mediastinal compartments of the chest, and retroperitoneum of the abdomen[ 2 - 4 ]. Primary paragangliomas arising in the pancreas are extremely rare, as there have been only 19 cases reported worldwide to date[ 1 , 5 - 20 ], with four of these cases accompanied by lymph node metastasis or distant metastasis[ 10 , 11 , 14 , 20 ] (Table 1 ). The criteria for malignancy based on histopathological examination are not well defined; therefore, the presence of specific local invasion or metastasis is a reliable diagnostic feature[ 21 ].…”