Functional paraganglioma of the pancreatic head: A case report and review of literature

Abstract

Background: Pheochromocytoma and paraganglioma (PGL) are a rare group of neuroendocrine neoplasms with characteristic genetic diversity and catecholamine secretion patterns. They arise from non-neuronal and non-epithelial neuroendocrine cells of the paraganglia, and have the highest rate of heritability among all tumors.

Case summary: A 76-year-old woman presented with the complaint of dizziness that had persisted for one week. She had a 30-year history of hypertension. Despite long-term use of antihypertensive drugs, her blood pressure was not effectively controlled. A tumor was subsequently found in the head of the pancreas by computed tomography and magnetic resonance imaging and she was initially diagnosed with an aneurysm. On December 21, 2021, she underwent resection of the retroperitoneal tumor and pancreatic repair surgery. However, after postoperative pathological analysis and immunohistochemistry, the diagnosis was revised to PGL. After two years and eight months of follow-up, the tumor did not recur or metastasize, and her blood pressure returned to normal without taking antihypertensive drugs.

Conclusion: The possibility of PGL should be considered when a tumor is identified and patients have catecholamine secretion related symptoms that are difficult to control with medications.

Keywords: Case report; Immunohistochemistry; Metanephrines; Pancreatic paraganglioma; Pancreatic tumor; Paraganglioma.

Figure 1

Computed tomography and magnetic resonance imaging findings. A: A Non-contrast-enhanced computed tomography scan showing a mass of approximately 3.2 cm × 4.1 cm × 5.0 cm in size at the lower edge of the liver and pancreatic head (orange arrow: Pancreas); B: Computed tomography enhancement showing the passage of blood vessels within the tumor, with patchy areas without enhancement and dilated blood vessels in the center (orange arrow: Pancreas); C: Non-contrast-enhanced magnetic resonance imaging in the pancreatic head; D: Magnetic resonance imaging enhanced arterial scan showed patchy enhancement in the center of the lesion and the delayed scan showed non-enhancement in the strip of patchy areas.

Figure 2

Postoperative microscopic images. A: Heterogeneous cells arranged in organoid or chrysanthemum-shaped clusters [Hematoxylin and eosin (HE): 50 ×]; B: Cells with increased nucleoplasmic ratios and eosinophilic cytoplasm (HE: 100 ×); C: Interstitial fibrous tissue proliferation and myxoid degeneration (HE: 200 ×); D: Irregular nuclear membranes. finechromatin.no necrosis formation (HE: 400 ×).

Figure 3

Immunohistochemistry findings. A: Cluster of differentiation 56 (+); B: Chromogranin A (+); C: Antigen identified by monoclonal antibody Ki-67 (+, 2%-3%); D: Synaptophysin (+) (magnification 200 ×).