gangliocytic paraganglioma

gangliocytic paraganglioma (duodenum)

A benign tumour of uncertain histogenesis, variously regarded as a hamartoma or neoplasm associated with neurofibromatosis type 1, most commonly in the 2nd part of the duodenum.

Clinical findings
Patients present with peptic ulcer disease-like symptoms, nausea, vomiting and upper GI bleeding; peak incidence is in middle-aged adults; male:female ratio, 2:1.
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References in periodicals archive ?
We wish to comment on the article by Park and Han (1) on gangliocytic paraganglioma (GP) with lymph node metastasis.
Gangliocytic paraganglioma has been regarded as an extremely rare tumor, but 40% (4 of 10) of patients diagnosed with duodenal NET G1 actually had GP in the previous investigation.
Corticotropin-producing pulmonary gangliocytic paraganglioma associated with Cushing's syndrome.
The recent WHO 2010 classification categorized all NETs from the GI and pancreatobiliary tracts as malignant tumors, except for gangliocytic paraganglioma and pancreatic neuroendocrine microadenomas, which are classified as benign tumors, and L-cell-type (glucagon-like peptide [GLP] and peptide YY [PYY]-producing) NETs and tubular carcinoids, which are classified as uncertain malignancies.
B, Gangliocytic paraganglioma of the duodenum showing epithelioid neuroendocrine cells (left), schwannian cells (center), and ganglion cell (right) components.
Gangliocytic paraganglioma (GP) is characterized by the triphasic cellular differentiation; epithelioid neuroendocrine cells, spindle cells with Schwann cell-like differentiation, and ganglion cells comprise the tumor.
Gangliocytic paraganglioma occurs most commonly in the second portion of the duodenum, with rare cases reported in the jejunum, pylorus, esophagus, pancreas, appendix, and lung.
Gangliocytic paraganglioma of the duodenum composed of spindled, epithelioid, and ganglion cells (arrow in [B] points to ganglion cells) (hematoxylin-eosin, original magnifications X50 [A] and X400 [B]).
The third most common group of GI mesenchymal tumors after GI stromal tumors (GISTs) and leiomyomas, (1) neurogenic polyps encompass a variety of clinically and pathologically distinct entities including schwannoma, granular cell tumor, perineurioma, neurofibroma, ganglioneuroma, and gangliocytic paraganglioma.
Gangliocytic paraganglioma (GP) is a rare and distinctive neoplasm of controversial histogenesis and biologic behavior.
38) In this setting, corticomedullary tumors, cauda equina paragangliomas showing ependymal differentiation, as well as gangliocytic paragangliomas that include Schwann-like cells and ganglion cells, do not qualify as composite tumors.