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WHO Classification of Tumours
Gangliocytic paraganglioma
Region of the ampulla of Vater


Unlike the conventional paraganglioma, this tumor has a peculiar morphologic appearance; it consists of a mixture of epithelioid neuroendocrine cells, Schwann-like cells, and ganglion cell-like elements.

Gangliocytic paraganglioma of the ampulla of Vater
Gangliocytic paragangliomas account for 20–25% of ampullary-periampullary neuroendocrine neoplasms. They are mainly found in the periampullary region where they produce polypoid lesions protruding into the duodenal lumen. These neoplasms are characterized by their triphasic cellular differentiation, consisting of neuroendocrine cells, spindle-shaped cells with Schwannian differentiation, and ganglion cells, which vary in terms of relative abundance and distribution within each neoplasm. Some gangliocytic paraganglioma have been associated with NF1

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Kheir SM, Halpern NB (1984)
Paraganglioma of the duodenum in association with congenital neurofibromatosis. Possible relationship.
Cancer 53: 2491-6

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Stephens M, Williams GT, Jasani B, Williams ED (1987)
Synchronous duodenal neuroendocrine tumours in von Recklinghausen's disease--a case report of co-existing gangliocytic paraganglioma and somatostatin-rich glandular carcinoid.
Histopathology 11: 1331-40

. Gangliocytic paragangliomas usually follow a benign course; however, occasional large neoplasms (size, > 2 cm) may spread to local lymph nodes, mainly manifesting as to the neuroendocrine component of the lesion
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Büchler M, Malfertheiner P, Baczako K, Krautzberger W, Beger HG (1985)
A metastatic endocrine-neurogenic tumor of the ampulla of Vater with multiple endocrine immunoreaction--malignant paraganglioma?
Digestion 31: 54-9

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Inai K, Kobuke T, Yonehara S, Tokuoka S (1989)
Duodenal gangliocytic paraganglioma with lymph node metastasis in a 17-year-old boy.
Cancer 63: 2540-5

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Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010


Commentary: Neuroendocrine tumours of the digestive system