gangliocytic

Gangliocytic is an adjective used in pathology to describe cells or tumors that resemble or contain ganglion cells. It is most commonly encountered in reference to gangliocytic paraganglioma, a rare neuroendocrine tumor of the gastrointestinal tract notable for its triphasic histology.

Gangliocytic paraganglioma typically presents in adults and most often arises in the duodenum, frequently near the ampulla of Vater. Lesions are usually submucosal and can appear as a polypoid mass on endoscopy. Patients may report non-specific symptoms such as abdominal pain, gastrointestinal bleeding, or anemia, though many tumors are found incidentally. Metastasis to regional lymph nodes is uncommon, and distant metastasis is rare, contributing to an overall favorable prognosis when the lesion is completely excised.

Histologically, the tumor demonstrates three cellular components: mature ganglion cells; spindle cells with Schwannian features; and epithelioid neuroendocrine cells. Immunohistochemistry supports this composition: epithelioid cells typically express neuroendocrine markers such as synaptophysin and chromogranin A; spindle cells show S-100 positivity; ganglion cells may be NSE-positive. Cytokeratins are usually negative in the tumor, aiding distinction from other neoplasms. The triphasic pattern helps distinguish gangliocytic paraganglioma from other duodenal tumors such as conventional neuroendocrine tumors, gastrointestinal stromal tumors, or schwannomas.

Management generally involves local surgical excision with clear margins. More extensive procedures, like pancreaticoduodenectomy, are reserved for large tumors or diagnostic uncertainty. The overall outlook is favorable, with rare cases of recurrence after complete resection. The term “gangliocytic” underscores the presence of ganglion-like cells within the lesion.