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

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.

Keywords: duodenum; gangliocytic paraganglioma; immunohistochemistry; paraganglioma-ganglioneuroma; progesterone receptor.

Figure 1

A sessile polyp human melanoma black-45 (HMB45) measured about 1 cm in diameter with a smooth surface was found in gastrointestinal endoscopy.

Figure 2

Histology of the tumor. (A) The tumor was well-circumscribed and non-encapsulated and located in the submucosa. (B) The tumor infiltrated the lamina propria focally. (C) The tumor was composed of epithelioid, ganglion-like, and spindle cells. Note the muscularis mucosae in the upper right that are different from the neoplastic spindle cells. (D) The epithelioid cells have abundant eosinophilic cytoplasm and a round nucleus. Note a ganglion-like cell in the center, which was larger in shape, and a nucleus with prominent eccentric nuclei. [(A, B), H&E ×20, (C), H&E ×100, (D), H&E ×400].

Figure 3

Immunohistochemistry of the tumor. (A) Synaptophysin (Syn) was diffusely positive for epithelioid cells and highlighted the ganglion-like cells. (B) Chromogranin A (CgA) was positive for epithelioid cells and highlighted the ganglion-like cells, but the number of positive cells was less than that in Syn. (C) Neurofilament (NF) stained some ganglion-like cells and neoplastic spindle cells. (D) S-100 stained the sustentacular cells around the epithelioid cells and the neoplastic spindle cells. (E) Progesterone receptor (PR) was positive in some epithelioid cells. (F) Desmin stained the muscularis mucosa, which displayed that the tumor infiltrated the lamina propria. [(A–F), ×100].