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Pheochromocytoma (view source)

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#redirect [[Adrenal_gland#Pheochromocytoma]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Pheochromocytoma high mag.jpg
| Width      =
| Caption    = Pheochromocytoma. [[H&E stain]].
| Synonyms  =
| Micro      = Zellballen (nests of cells), fibrovascular septae, salt-and-pepper nuclei, +/-hemorrhage (very common)
| Subtypes  =
| LMDDx      = [[adrenocortical carcinoma]], [[paraganglioma]]
| Stains    =
| IHC        = chief cells: chromogranin A +ve, synaptophysin +ve; sustentacular cells: S-100 +ve
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = [[adrenal gland]] (same tumour arising at other sites known as ''[[paraganglioma]]'')
| Assdx      =
| Syndromes  = [[Multiple endocrine neoplasia]] 2A and 2B, [[von Hippel-Lindau syndrome]],[[Neurofibromatosis]] type 1, familial paraganglioma syndromes (several)
| Clinicalhx = hypertension (classic Hx), paroxysms of tachycardia, headache, anxiety, hypertension
| Signs      =
| Symptoms  =
| Prevalence = uncommon
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = usually benign
| Other      =
| ClinDDx    = other adrenal gland masses, [[renal cell carcinoma]], other abdominal masses
| Tx        =
}}
'''Pheochromocytoma''' is a tumour of the [[adrenal gland]] medulla. It may be benign or [[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 - see ''[[paraganglioma]]'' article.
 
===Clinical===
*Classic finding: hypertension.
*Paroxysms (i.e. episodes) of tachycardia, headache, anxiety, [[hypertension]].
 
Laboratory findings (urine):
*Vanillylmandelic acid (VMA).
*Metanephrines.
 
==Macroscopic==
*Medullary tumour
*Round to oval mass
*Dusky red and possibly haemorrhagic
 
==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.
*Rarely pigmented <ref>{{Cite journal  | last1 = Bellezza | first1 = G. | last2 = Giansanti | first2 = M. | last3 = Cavaliere | first3 = A. | last4 = Sidoni | first4 = A. | title = Pigmented "black" pheochromocytoma of the adrenal gland: a case report and review of the literature. | journal = Arch Pathol Lab Med | volume = 128 | issue = 10 | pages = e125-8 | month = Oct | year = 2004 | doi = 10.1043/1543-2165(2004)128<e125:PBPOTA>2.0.CO;2 | PMID = 15387689 }}</ref>
 
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]]''.
*[[Paraganglioma]] - same lesion arising outside of the adrenal gland.
 
===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)
Image:Adrenal Phaeochromocytoma 2 MP2 CTR.jpg|Adrenal Pheochromocytoma - medium power - this particular color of blue purple seems to be farily unique to phaeochromocytoma. (SKB)
Image:Adrenal Phaeochromocytoma 2 MP CTR.jpg|Adrenal Pheochromocytoma - medium power (SKB)
Image:Adrenal Phaeochromocytoma 2 HP CTR.jpg|Adrenal Pheochromocytoma - high power - nuclear pleomorphism (SKB)
Image:Adrenal Phaeochromocytoma 3 LP CTR.jpg|Adrenal Pheochromocytoma - low power (SKB)
Image:Adrenal Phaeochromocytoma 3 MP CTR.jpg|Adrenal Pheochromocytoma - medium power (SKB)
Image:Adrenal Phaeochromocytoma 3 HP CTR.jpg|Adrenal Pheochromocytoma - high power - version with cleared cells - adrenal cortical neoplasms might be a histologic consideration with this lesion (SKB)
Image:Adrenal Phaeochromocytoma 4 MP CTR.jpg|Pheochromocytoma - medium power (SKB)
Image:Adrenal Phaeochromocytoma 4 MP2 CTR.jpg|Pheochromocytoma - medium power (SKB)
Image:Adrenal Phaeochromocytoma 4 HP CTR.jpg|Adrenal Pheochromocytoma - high power - version with eosinophilic cytoplasm and eosinophilic globules (SKB)
Image:Adrenal Phaeochromocytoma 5 LP PA.JPG|Pheochromocytoma - low power - not a great photo but shows the adrenal cortex pushes aside by the medullary pheochromocytoma (SKB)
Image:Adrenal Phaeochromocytoma 5 MP PA.JPG|Pheochromocytoma - medium power (SKB)
Image:Adrenal Phaeochromocytoma MP CTR (3).jpg|Pheochromocytoma - medium power (SKB)
Image:Adrenal Phaeochromocytoma MP2 CTR (2).jpg|Pheochromocytoma - medium power (SKB)
Image:Adrenal Phaeochromocytoma HP CTR (2).jpg|Pheochromocytoma - high power - this grey-blue (probably a faded version of the purple blue is also fairly unique to pheochromocytoma (SKB)
Image:Adrenal Phaeochromocytoma 6 MP PA.JPG|Pheochromocytoma - medium power - this pheo is composed of somewhat spindled cells (SKB)
Image:Adrenal Phaeochromocytoma LP CTR.jpg|Pheochromocytoma - medium power (SKB)
Image:Adrenal Phaeochromocytoma MP CTR.jpg|Pheochromocytoma - medium power (SKB)
Image:Adrenal Phaeochromocytoma MP CTR (2).jpg|Pheochromocytoma - medium power (SKB)
Image:Adrenal Phaeochromocytoma MP2 CTR.jpg|Pheochromocytoma - medium power (SKB)
Image:Adrenal Phaeochromocytoma HP CTR.jpg|Pheochromocytoma - medium power (SKB)
Image:Adrenal Pheochromocytoma 4 LP2 PA.JPG|Pheochromocytoma - low power (SKB)
Image:Adrenal Pheochromocytoma 4 MP2 PA - Copy.JPG|Pheochromocytoma - medium power (SKB)
Image:Adrenal Pheochromocytoma 4 HP2 PA.JPG|Pheochromocytoma - high power (SKB)
Image:Adrenal Pheochromocytoma LP RBWH.JPG|Pheochromocytoma - low power - shows compressed adrenal cortex to the left (SKB)
Image:Adrenal Pheochromocytoma LP2 RBWH.JPG|Pheochromocytoma - low power (SKB)
Image:Adrenal Pheochromocytoma MP RBWH.JPG|Pheochromocytoma  - medium power (SKB)
Image:Adrenal Pheochromocytoma HP RBWH - Copy.JPG|Pheochromocytoma - high power - another with cleared cells (SKB)
Image:Adrenal Pheochromocytoma MP3 RBWH.JPG|Adrenal Pheochromocytoma - medium power (SKB)
Image:Adrenal Pheochromocytoma MP4 RBWH.JPG|Pheochromocytoma - medium power (SKB)
 
</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 (8th Ed.)'' 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.
 
Pheochromocytoma versus [[adrenal cortical carcinoma]] (ACC):<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>
*Melan A -ve.
**Positive in ACC.
*Inhibin -ve.
**Positive in ACC.
*[[Calretinin]] -ve.
**Positive in ACC.
 
A panel:
*S-100, chromogranin, calretinin, EMA, PAX8.
 
==[[Electron microscopy]]==
*Membrane-bound secretory granules.
 
==Sign out==
Mete ''et al.''. created a synoptic report for these cases that may be used.<ref name=pmid24476517>{{cite journal |authors=Mete O, Tischler AS, de Krijger R, McNicol AM, Eisenhofer G, Pacak K, Ezzat S, Asa SL |title=Protocol for the examination of specimens from patients with pheochromocytomas and extra-adrenal paragangliomas |journal=Arch Pathol Lab Med |volume=138 |issue=2 |pages=182–8 |date=February 2014 |pmid=24476517 |pmc=3909881 |doi=10.5858/arpa.2012-0551-OA |url=}}</ref>  The ''College of American Pathologists'' does ''not'' have a [[CAP protocols|synoptic]] for these currently (September 2021).{{fact}}
 
<pre>
Right Adrenal (Mass), Adrenalectomy:
    - Pheochromocytoma, margin clear.
 
Comment:
The tumour stains as follows:
POSITIVE: synaptophysin, chromogranin A, S-100 (sustentacular cells).
NEGATIVE: EMA, inhibin.
Proliferation (Ki-67): <2% of tumour cells.
 
The immunostaining pattern is consistent with a pheochromocytoma.
</pre>
 
 
===Block letters===
<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]]
Michael
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