Is Gangliocytic Paraganglioma Designated as a Subtype of Composite Paragangliomas and Originated From Pancreas Islet? A Case Report and Review of Literature.

Jing Li,Pei-Shuang Zhu,Lu-Ping Wang

doi:10.3389/fendo.2022.847632

Abstract

Gangliocytic paraganglioma (GP) is quite rare, and origin and entity remain to be elucidated. A 51-year-old man presented with GP as a sessile polyp with a smooth surface that measured about 1 cm in diameter in the descending portion of duodenum. Pathological examination displayed that a neoplasm was predominantly located in the submucosa and infiltrated mucosa focally. The tumor consisted of epithelioid, ganglion-like, and spindle cells admixing in a haphazard way. The epithelioid cells resembled paraganglioma in cytological and architectural features. The ganglion-like cells were scattered and merged with the bland spindle cells in fascicular clusters, which resembled ganglioneuroma. Synaptophysin (Syn), microtubule-associated protein-2 (MAP-2), and chromogranin A (CgA) were positive in the epithelioid and ganglion-like cells in variety, and neurofilament (NF) staining highlighted the ganglion-like cells. S-100 and SOX-10 were positive in the spindle cell proliferation and around the epithelioid cells. Progesterone receptor (PR) was positive in the epithelioid cells. The polyp was resected, and no adjuvant therapy was given. The patient remained with no recurrence in 2 years’ follow-up. Origin of GP is presumed to be related to pancreas islet. GP is distinguished from neuroendocrine tumor (NET) G1 and designated as paraganglioma-ganglioneuroma, a kind of composite paragangliomas.

Highlights

Paraganglioma (PGL) always involves the extra-adrenal ganglions among the sympathetic or parasympathetic chain
90% of Gangliocytic paraganglioma (GP) were documented to be located in the duodenum, and involvement of other sites such as spinal cord, respiratory system, and digestive tract was reported [2]
GPs range in size from 0.5 to 10 cm with an average of 2.5 cm in maximum diameter

Summary

INTRODUCTION

Paraganglioma (PGL) always involves the extra-adrenal ganglions among the sympathetic or parasympathetic chain. The tumor was predominantly located in the submucosa, infiltrated the lamina propria focally, and covered with duodenal mucosa (Figures 2A, B) It was composed of epithelioid cells, ganglion-like cells, and spindle cells (Figure 2C). The epithelioid cells were large in round or polygonal shape and arranged in nest or zellballen pattern They had abundant eosinophilic cytoplasm and a round nucleus with inconspicuous nucleoli. The ganglion-like cells were even larger with eosinophilic cytoplasm and out-standing eccentric vesicular nuclei and were always scattered and not distinguished from the epithelioid cells (Figure 2D). Both types of cells displayed mild cellular pleomorphism yet did not display mitosis. The patient received no adjuvant therapy and remained with no recurrence in 2 years’ follow-up

DISCUSSION

Findings

ETHICS STATEMENT