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pheochromocytoma

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#