logo logo
Search: Organ site Search: Tumor / Syndrome
or
WHO Classification of Tumours
Gangliocytic paraganglioma
Adrenal gland


Definition

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
Click for details
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

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




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




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



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



and pancreas
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
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

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
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.