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Difference between revisions of "Pheochromocytoma"
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{{ Infobox diagnosis | {{ Infobox diagnosis | ||
| Name = {{PAGENAME}} | | Name = {{PAGENAME}} | ||
| Image = | | Image = Pheochromocytoma high mag.jpg | ||
| Width = | | Width = | ||
| Caption = | | Caption = Pheochromocytoma. [[H&E stain]]. | ||
| Synonyms = | | Synonyms = | ||
| Micro = | | Micro = Zellballen (nests of cells), fibrovascular septae, salt-and-pepper nuclei, +/-hemorrhage (very common) | ||
| Subtypes = | | Subtypes = | ||
| LMDDx = [[adrenocortical carcinoma]], [[paraganglioma]] | | LMDDx = [[adrenocortical carcinoma]], [[paraganglioma]] | ||
| Line 15: | Line 15: | ||
| Gross = | | Gross = | ||
| Grossing = | | Grossing = | ||
| Site = [[adrenal gland]] (same tumour at other sites known as ''[[paraganglioma]]'') | | Site = [[adrenal gland]] (same tumour arising at other sites known as ''[[paraganglioma]]'') | ||
| Assdx = | | Assdx = | ||
| Syndromes = [[Multiple endocrine neoplasia]] 2A and 2B, [[von Hippel-Lindau syndrome]],[[Neurofibromatosis]] type 1, familial paraganglioma syndromes (several) | | Syndromes = [[Multiple endocrine neoplasia]] 2A and 2B, [[von Hippel-Lindau syndrome]],[[Neurofibromatosis]] type 1, familial paraganglioma syndromes (several) | ||
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Memory device - the rule of 10s:<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, | *10% extra-adrenal (e.g. carotid body, ''Organ of Zuckerkandl'' (neighourhood of aortic bifuration/IMA branch point)). | ||
*10% bilateral. | *10% bilateral. | ||
*10% malignant. | *10% malignant. | ||
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*#[[von Hippel-Lindau syndrome]]. | *#[[von Hippel-Lindau syndrome]]. | ||
*#[[Neurofibromatosis]] type 1. | *#[[Neurofibromatosis]] type 1. | ||
*#Familial paraganglioma syndromes - several. | *#Familial paraganglioma syndromes - several - see ''[[paraganglioma]]'' article. | ||
===Clinical=== | ===Clinical=== | ||
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*Vanillylmandelic acid (VMA). | *Vanillylmandelic acid (VMA). | ||
*Metanephrines. | *Metanephrines. | ||
==Macroscopic== | |||
*Medullary tumour | |||
*Round to oval mass | |||
*Dusky red and possibly haemorrhagic | |||
==Microscopic== | ==Microscopic== | ||
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*Often haemorrhagic - highly vascular. | *Often haemorrhagic - highly vascular. | ||
*+/-Nuclear pleomorphism. | *+/-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: | Notes: | ||
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Image:Carotid_body_tumour_2_low_mag.jpg | Carotid body tumour - low mag. (WC/Nephron) | 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: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> | </gallery> | ||
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===Malignant pheochromoctyoma=== | ===Malignant pheochromoctyoma=== | ||
#''Robbins'' says metastases are the sole criteria of malignancy.<ref name=Ref_PCPBoD8_586>{{Ref PCPBoD8|586}}</ref> | #''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> | #''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. | #*Marked nuclear atypia. | ||
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*Sustentacular cells: | *Sustentacular cells: | ||
**S100 +ve. | **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]]== | ==[[Electron microscopy]]== | ||
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==Sign out== | ==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> | <pre> | ||
ADRENAL MASS, RIGHT, ADRENALECTOMY: | ADRENAL MASS, RIGHT, ADRENALECTOMY: | ||