8683/0 Gangliocytic paraganglioma


Definitions

Region of the ampulla of Vater
ICD-O-3 topography code: C24.1

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 1
Click to access Pubmed
Kheir SM, Halpern NB (1984)
Paraganglioma of the duodenum in association with congenital neurofibromatosis. Possible relationship.
Cancer 53: 2491-6



2
Click to access Pubmed
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 3
Click to access Pubmed
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



4
Click to access Pubmed
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



5
 
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



.





Small intestine
ICD-O-3 topography code: C17

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.





Adrenal gland
ICD-O-3 topography code: C74

Unlike the conventional paraganglioma, the gangliocytic paraganglioma has a peculiar morphologic appearance; it consists of a mixture of epithelioid neuroendocrine cells, Schwann-like cells, and ganglion cell-like elements. It thereby appears to be a hybrid tumour of a carcinoid, paraganglioma and ganglioneuroma
6
 
Ronald A. DeLellis, Ricardo V. Lloyd, Philipp U. Heitz, Charis Eng
World Health Organization Classification of Tumours. Pathology and Genetics of Tumors of Endocrine Organs
IARC Press: Lyon 2004



.

Gangliocytic paragangliomas almost always arise in the duodenum, particularly in the periampullary region
7
Click to access Pubmed
Altavilla G, Chiarelli S, Fassina A (2001)
Duodenal periampullary gangliocytic paraganglioma: report of two cases with immunohistochemical and ultrastructural study.
Ultrastruct Pathol 25: 137-45



8
Click to access Pubmed
Buetow PC, Levine MS, Buck JL, Pantongrag-Brown L, Emory TS (1997)
Duodenal gangliocytic paraganglioma: CT, MR imaging, and US findings.
Radiology 204: 745-7



9
Click to access Pubmed
Sakhuja P, Malhotra V, Gondal R, Dutt N, Choudhary A (2001)
Periampullary gangliocytic paraganglioma.
J Clin Gastroenterol 33: 154-6



, but have occasionally been reported in the jejunum 10
Click to access Pubmed
Burke AP, Helwig EB (1989)
Gangliocytic paraganglioma.
Am J Clin Pathol 92: 1-9



, pylorus 11
Click to access Pubmed
Burke AP, Helwig EB (1989)
Gangliocytic paraganglioma.
Am J Clin Pathol 92: 1-9



and pancreas 12
Click to access Pubmed
Tomic S, Warner T (1996)
Pancreatic somatostatin-secreting gangliocytic paraganglioma with lymph node metastases.
Am J Gastroenterol 91: 607-8



. One case has been reported in the nasopharynx 13
Click to access Pubmed
Sinkre P, Lindberg G, Albores-Saavedra J (2001)
Nasopharyngeal gangliocytic paraganglioma.
Arch Pathol Lab Med 125: 1098-100



.
Approximately 8% of gangliocytic paragangliomas give rise to regional lymph node metastases
14
Click to access Pubmed
Tomic S, Warner T (1996)
Pancreatic somatostatin-secreting gangliocytic paraganglioma with lymph node metastases.
Am J Gastroenterol 91: 607-8



and one tumour is reported to have infiltrated the mesentery 15
Click to access Pubmed
Dookhan DB, Miettinen M, Finkel G, Gibas Z (1993)
Recurrent duodenal gangliocytic paraganglioma with lymph node metastases.
Histopathology 22: 399-401



. There are no reports of distant metastases. Gangliocytic paragangliomas pursue an indolent course even in the presence of lymph node metastases.