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pheochromocytoma

Monday 5 March 2012

PO

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

- pediatric pheochromocytoma

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

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

- http://ispub.com/IJEN/8/1/1576

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