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#redirect [[Adrenal_gland#Pheochromocytoma]]
'''Pheochromocytoma''' is a tumour of the [[adrenal gland]] medulla. It may be benign of malignant.
 
==General==
*Considered to be a [[paraganglioma]].<ref name=Ref_EP327>{{Ref EP|327}}</ref>
*Literally means "dusky" (pheo) "colour" (chromo) - dull appearance on gross.
*Tumour arises from adrenal medulla - chromaffin cells.<ref name=Ref_PCPBoD8_586>{{Ref PCPBoD8|586}}</ref>
 
Memory device - the rule of 10s:<ref name=Ref_PCPBoD8_586>{{Ref PCPBoD8|586}}</ref>
*10% extra-adrenal (e.g. carotid body, organ of Zuckerkandl (neighourhood of aortic bifuration/IMA branch point)).
*10% bilateral.
*10% malignant.
*10% no hypertension.
*25% associated within a syndrome:
*#[[Multiple endocrine neoplasia]] 2A and 2B.
*#[[von Hippel-Lindau syndrome]].
*#[[Neurofibromatosis]] type 1.
*#Familial paraganglioma syndromes - several.
 
===Clinical===
*Classic finding: hypertension.
*Paroxysms (i.e. episodes) of tachycardia, headache, anxiety, [[hypertension]].
 
Laboratory findings (urine):
*Vanillylmandelic acid (VMA).
*Metanephrines.
 
==Microscopic==
Features:<ref>{{Ref PBoD8|1161}}</ref>
*Chief cells:
**Usu. polygonal cells, may be spindled.
**Arranged in cell nests - "Zellballen" (literally ''cell balls'') - '''key feature'''.
**Stippled chromatin ([[AKA]] salt and pepper chromatin) - coarsely granular chromatin.
**Granular cytoplasm, often basophilic - '''important'''.
*Sustentacular cells (structural support cell).
*Often haemorrhagic - highly vascular.
*+/-Nuclear pleomorphism.
 
Notes:
*The nested architecture (Zellballen) is useful for differentiating from [[ACC]].
*[[Metastasis]] sole criteria of malignancy.<ref name=Ref_PCPBoD8_586>{{Ref PCPBoD8|586}}</ref>
*Surrounding adrenal cortex is typically compressed.<ref>URL: [http://www.pathpedia.com/Education/eAtlas/Histopathology/Adrenal/Pheochromocytoma.aspx http://www.pathpedia.com/Education/eAtlas/Histopathology/Adrenal/Pheochromocytoma.aspx]. Accessed on: 27 May 2013.</ref>
 
DDx:
*[[Adrenal cortical carcinoma]] - ''[[pheochromocytoma versus adrenal cortical carcinoma]]''.
 
===Images===
<gallery>
Image:Carotid_body_tumour_2_low_mag.jpg | Carotid body tumour - low mag. (WC/Nephron)
Image:Carotid_body_tumour_2_high_mag.jpg | Carotid body tumour - high mag. (WC/Nephron)
</gallery>
 
====Pheochromocytoma versus adrenal cortical carcinoma====
*Pheochromocytoma and adrenal cortical carcinoma overlap histologically.<ref name=pmid20154585>{{Cite journal  | last1 = Sangoi | first1 = AR. | last2 = McKenney | first2 = JK. | title = A tissue microarray-based comparative analysis of novel and traditional immunohistochemical markers in the distinction between adrenal cortical lesions and pheochromocytoma. | journal = Am J Surg Pathol | volume = 34 | issue = 3 | pages = 423-32 | month = Mar | year = 2010 | doi = 10.1097/PAS.0b013e3181cfb506 | PMID = 20154585 }}</ref>
 
Favour pheochromocytoma:
*Small chickenwire-pattern blood vessels, nests, salt-and-pepper chromatin, red blood cell extravasation.
 
Favour adrenal cortical carcinoma:
*Nucleolus, sheeting.
 
===Malignant pheochromoctyoma===
#''Robbins'' says metastases are the sole criteria of malignancy.<ref name=Ref_PCPBoD8_586>{{Ref PCPBoD8|586}}</ref>
#''Thompson'' suggests one can differentiate benign from malignant with the aid of the following:<ref name=Ref_EP259>{{Ref EP|259}}</ref>
#*Marked nuclear atypia.
#*Invasion:
#**Capsular.
#**Vascular.
#*Necrosis.
#*Cellular monotony.
#*Mitoses:
#**Rate.
#**Atypical mitosis.
 
==IHC==
*Chief cells:
**Chromogranin A +ve.
**Synaptophysin +ve.
*Sustentacular cells:
**S100 +ve.
 
==[[Electron microscopy]]==
*Membrane-bound secretory granules.
 
==Sign out==
<pre>
ADRENAL MASS, RIGHT, ADRENALECTOMY:
- PHEOCHROMOCYTOMA.
- SURGICAL MARGIN NEGATIVE FOR PHEOCHROMOCYTOMA.
 
COMMENT:
The tumour cells stains for chromogranin and synaptophysin. S-100 marks the sustentacular cells.
Inhibin is negative in the tumour cells. The immunostaining pattern is consistent with a
pheochromocytoma.
</pre>
 
====Micro====
The sections shows a partially hemorrhagic lesion in the medulla of the adrenal gland that is arranged in nests (Zellballen).  The tumour cells have abundant grey/blue granular cytoplasm, and nuclei with granular chromatin (salt and pepper chromatin). The lesion is surrounded by a compressed rim of adrenal cortex and fibrosis tissue.  The core of the lesion is fibrotic and has clusters of hemosiderin-laden macrophages.
 
There is no capsular invasion. Vascular invasion is not identified.  There is no necrosis. Mitotic activity is not appreciated. 
 
The adrenal cortex is unremarkable.
 
==See also==
*[[Paraganglioma]].
*[[Adrenal gland]].
 
==References==
{{Reflist|2}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Adrenal gland]]

Revision as of 02:49, 23 August 2014

Pheochromocytoma is a tumour of the adrenal gland medulla. It may be benign of malignant.

General

  • Considered to be a paraganglioma.[1]
  • Literally means "dusky" (pheo) "colour" (chromo) - dull appearance on gross.
  • Tumour arises from adrenal medulla - chromaffin cells.[2]

Memory device - the rule of 10s:[2]

Clinical

  • Classic finding: hypertension.
  • Paroxysms (i.e. episodes) of tachycardia, headache, anxiety, hypertension.

Laboratory findings (urine):

  • Vanillylmandelic acid (VMA).
  • Metanephrines.

Microscopic

Features:[3]

  • Chief cells:
    • Usu. polygonal cells, may be spindled.
    • Arranged in cell nests - "Zellballen" (literally cell balls) - key feature.
    • Stippled chromatin (AKA salt and pepper chromatin) - coarsely granular chromatin.
    • Granular cytoplasm, often basophilic - important.
  • Sustentacular cells (structural support cell).
  • Often haemorrhagic - highly vascular.
  • +/-Nuclear pleomorphism.

Notes:

  • The nested architecture (Zellballen) is useful for differentiating from ACC.
  • Metastasis sole criteria of malignancy.[2]
  • Surrounding adrenal cortex is typically compressed.[4]

DDx:

Images

  • Carotid body tumour - low mag. (WC/Nephron)
    Carotid body tumour - low mag. (WC/Nephron)
  • Carotid body tumour - high mag. (WC/Nephron)
    Carotid body tumour - high mag. (WC/Nephron)

Pheochromocytoma versus adrenal cortical carcinoma

  • Pheochromocytoma and adrenal cortical carcinoma overlap histologically.[5]

Favour pheochromocytoma:

  • Small chickenwire-pattern blood vessels, nests, salt-and-pepper chromatin, red blood cell extravasation.

Favour adrenal cortical carcinoma:

  • Nucleolus, sheeting.

Malignant pheochromoctyoma

  1. Robbins says metastases are the sole criteria of malignancy.[2]
  2. Thompson suggests one can differentiate benign from malignant with the aid of the following:[6]
    • Marked nuclear atypia.
    • Invasion:
      • Capsular.
      • Vascular.
    • Necrosis.
    • Cellular monotony.
    • Mitoses:
      • Rate.
      • Atypical mitosis.

IHC

  • Chief cells:
    • Chromogranin A +ve.
    • Synaptophysin +ve.
  • Sustentacular cells:
    • S100 +ve.

Electron microscopy

  • Membrane-bound secretory granules.

Sign out

ADRENAL MASS, RIGHT, ADRENALECTOMY:
- PHEOCHROMOCYTOMA.
- SURGICAL MARGIN NEGATIVE FOR PHEOCHROMOCYTOMA.

COMMENT:
The tumour cells stains for chromogranin and synaptophysin. S-100 marks the sustentacular cells.
Inhibin is negative in the tumour cells. The immunostaining pattern is consistent with a 
pheochromocytoma.

Micro

The sections shows a partially hemorrhagic lesion in the medulla of the adrenal gland that is arranged in nests (Zellballen). The tumour cells have abundant grey/blue granular cytoplasm, and nuclei with granular chromatin (salt and pepper chromatin). The lesion is surrounded by a compressed rim of adrenal cortex and fibrosis tissue. The core of the lesion is fibrotic and has clusters of hemosiderin-laden macrophages.

There is no capsular invasion. Vascular invasion is not identified. There is no necrosis. Mitotic activity is not appreciated.

The adrenal cortex is unremarkable.

See also

References

  1. ↑ Thompson, Lester D. R. (2006). Endocrine Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 327. ISBN 978-0443066856.
  2. ↑ 2.0 2.1 2.2 2.3 Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 586. ISBN 978-1416054542.
  3. ↑ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1161. ISBN 978-1416031215.
  4. ↑ URL: http://www.pathpedia.com/Education/eAtlas/Histopathology/Adrenal/Pheochromocytoma.aspx. Accessed on: 27 May 2013.
  5. ↑ Sangoi, AR.; McKenney, JK. (Mar 2010). "A tissue microarray-based comparative analysis of novel and traditional immunohistochemical markers in the distinction between adrenal cortical lesions and pheochromocytoma.". Am J Surg Pathol 34 (3): 423-32. doi:10.1097/PAS.0b013e3181cfb506. PMID 20154585.
  6. ↑ Thompson, Lester D. R. (2006). Endocrine Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 259. ISBN 978-0443066856.
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