Pheochromocytoma (PCC) in children is rare, genetically not well described, and often related to a poor prognosis.
Microscopical synopsis
+/- amyloid stroma
Variants
oncocytic pheochromocytoma (11075859)
composite adrenal pheochromocytoma/adrenal ganglioneuroma (MEN2A) (8990146, 3181953, 10839612)
pigmented paraganglioma (8491482)
gangliocytic paraganglioma (15492999)
Predisposition
von Hippel-Lindau disease (VHL)
CGH (15474154)
losses of chromosomes
- 3p loss (72%)
- Chr.4 loss
- 11p loss (72%)
- 13q loss
- 15q loss
- 16p loss
- 17p loss
- Chr.18 loss
gains
- partial gains of chromosome 7
See also
adrenal tumors
paragangliomas
endocrine tumors
References
Hering A, Guratowska M, Bucsky P, Claussen U, Decker J, Ernst G, Hoeppner W, Michel S, Neumann H, Parlowsky T, Loncarevic I. Characteristic genomic imbalances in pediatric pheochromocytoma. Genes Chromosomes Cancer. 2006 Jun;45(6):602-7. PMID: 16518846
Reviews
Maher ER, Eng C. The pressure rises: update on the genetics of phaeochromocytoma. Hum Mol Genet. 2002 Oct 1;11(20):2347-54. PMID: 12351569
Clarke MR, Weyant RJ, Watson CG, Carty SE. Prognostic markers in pheochromocytoma. Hum Pathol. 1998 May;29(5):522-6. PMID: 9596278