Home > G. Tumoral pathology > pheochromocytoma
pheochromocytoma
Monday 5 March 2012
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Images
pheochromocytoma
Digital cases
JRC:478 : Pheochromocytoma
JRC:479 : Pheochromocytoma
JRC:14922 : Pheochromocytoma
JRC:16410 : Adrenal pheochromocytoma (very basophilic)
JRC:14612 : Adrenal pheochromocytoma
JRC:18773 : Adrenal pheochromocytoma (F; 55 y/o)
JRC:18774 : Adrenal pheochromocytoma (M; 34 y/o)
Subtypes
IHC
Positive IHC
- Chromogranin, synaptophysin (also present in adrenal cortical carcinomas),
- -S100 (sustentacular cells)
- tenascin (strong in clinically malignant tumors, Am J Surg Pathol 2001;25:1419)
- catecholamines, neuron specific enolase, neurofilament
Variable IHC
- vimentin, bcl2 and focal HMB45 (Arch Pathol Lab Med 1992;116:151)
Negative IHC
- MelanA / Mart1, inhibin, keratin (usually) and calretinin
Differential diagnosis
adrenocortical carcinoma: inhibin+, MelanA+ and calretinin+
small blue cell tumors
- carcinoid tumor
- Ewing sarcoma / PNET
- lymphomas
- monomorphic Wilm tumor
- neuroblastoma
- medulloblastoma
- rhabdomyosarcomas
- small cell carcinomas
- neuroendocrine carcinomas
- small cell osteosarcomas
Molecular biology
Pheochromocytomas are associated with mutations in genes coding for subunits of succinate dehydrogenase , loss of heterozygosity at 1p34-36 and the VHL gene (3p25).
It is also strongly associated with the MEN2A and MEN2B familial syndromes, and found in less than 5% of cases of VHL, Sturge-Weber and NF-1.
See also
paraganglioma
adrenal tumors
SDH-deficient tumors
Open references
