Difference between revisions of "Adrenal gland"

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==Neuroblastoma==
==Neuroblastoma==
:See also: ''[[olfactory neuroblastoma]]''.
{{Main|Neuroblastoma}}
===General===
Epidemiology:
*Usually paediatric population.
 
Laboratory findings:
*Increased urine homovanillic acid.
 
Predictors of a poor prognosis:<ref name=Ref_PCPBoD8_254>{{Ref PCPBoD8|254}}</ref>
*High mitotic-karyorrhectic index.
*Lack of schwannian stroma.
*>18 months.
*Near ploidy.
*N-MYC amplification.
*Lymph node spread.
*Distant spread.
 
Classification:
*In a grouping known as ''neuroblastic tumours'' which includes:<ref name=pmid10421272>{{cite journal |author=Shimada H, Ambros IM, Dehner LP, Hata J, Joshi VV, Roald B |title=Terminology and morphologic criteria of neuroblastic tumors: recommendations by the International Neuroblastoma Pathology Committee |journal=Cancer |volume=86 |issue=2 |pages=349–63 |year=1999 |month=July |pmid=10421272 |doi= |url=}}</ref>
**[[Ganglioneuroma]] (benign).
**[[Ganglioneuroblastoma]] (intermediate).
**Neuroblastoma (aggressive).
 
===Gross===
*Typically an abdominal mass.
**~40% arise in the [[adrenal gland]].<ref name=Ref_PCPBoD8_253>{{Ref PCPBoD8|253}}</ref>
 
===Microscopic===
Features:<ref name=pmid18635637>{{cite journal |author=Chung EM, Murphey MD, Specht CS, Cube R, Smirniotopoulos JG |title=From the Archives of the AFIP. Pediatric orbit tumors and tumorlike lesions: osseous lesions of the orbit |journal=Radiographics |volume=28 |issue=4 |pages=1193–214 |year=2008 |pmid=18635637 |doi=10.1148/rg.284085013 |url=}}</ref>
*[[small round cell tumour|Small round blue cell]]s separated by thin (pink) fibrous septa.
*Homer-Wright rosettes.
**Rosette with a small (~100 micrometers - diameter) meshwork of fibers (neuropil) at the centre.<ref name=pmid16551982>{{cite journal |author=Wippold FJ, Perry A |title=Neuropathology for the neuroradiologist: rosettes and pseudorosettes |journal=AJNR Am J Neuroradiol |volume=27 |issue=3 |pages=488–92 |year=2006 |month=March |pmid=16551982 |doi= |url=}}</ref>
*Neuropil-like stroma = paucicellular stroma with a cotton candy-like appearance; see comparison below.
**>50% neuropil-like stroma -- otherwise it's a [[ganglioneurona]] or ganglioblastoma.
 
Notes:
*The fibrous septa are especially useful for differentiation from lymphoma.
 
DDx:
*[[Small round cell tumours]].
**[[Wilms tumour]].
**Lymphoma.
**[[Hepatoblastoma]].
 
Images:
<gallery>
Image:Adrenal Neuroblastoma MP CTR.jpg|Neuroblastoma - medium power]]
Image:Adrenal Neuroblastoma 2 MP CTR.jpg|Neuroblastoma - medium power (SKB)
Image:Adrenal Neuroblastoma M2P PA.JPG|Neuroblastoma  - medium power]]
Image:Adrenal Neuroblastoma VascularInvasion MP CTR.jpg|thumb|Neuroblastoma  - vascular invasion - medium power]]
Image:Adrenal Neuroblastoma MP3 PA.JPG|Neuroblastoma  - medium power]]
Image:Adrenal Neuroblastoma MP PA.JPG|Neuroblastoma  - medium power]]
Image:Adrenal Neuroblastoma 2 HP CTR.jpg|Neuroblastoma - high power (SKB)
Image:Adrenal Neuroblastoma 3 HP CTR.jpg|Neuroblastoma  - high power - blue cells arrayed around a core of fluffy pink neuropil (SKB)
Image:Adrenal Neuroblastoma HP2 CTR.jpg|Neuroblastoma -  high power]]
Image:Adrenal Neuroblastoma HP CTR.jpg|Neuroblastoma  -  high power]]
</gallery>
*[http://radiographics.rsna.org/content/28/4/1193/F42.expansion Neuroblastoma (radiographics.rsna.org)].<ref>URL: [http://radiographics.rsna.org/content/28/4/1193.full http://radiographics.rsna.org/content/28/4/1193.full]. Accessed on: 12 January 2011.</ref>
*[http://commons.wikimedia.org/wiki/File:Neuroblastoma_rosettes.jpg Neuroblastoma (WC)].
*[http://farm3.static.flickr.com/2259/2274260465_afbea05f9b.jpg Neuroblastoma (flickr.com)].
 
====Schwannian vs. neuropil====
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
| Feature
| Schwannian
| Neuropil
|-
| Cellularity
| high ~ spacing of cells < 30 µm
| low ~ spacing of cells > 100 µm
|-
| Fibrillary
| yes, long fine strands
| no
|-
| Associations
| ganglion cells
| neuroblasts
|-
| Cytoplasmic vacuolation
| yes
| ?
|-
|}
 
====Classification/grading====
Commonly grouped by the ''Shimada classification'', which depends on the presence a number of things including:
*Mitoses/karyorrhectic cells.
*Molecular abnormalities.
 
===IHC===
*PGP 9.5 +ve.<ref>{{Cite journal  | last1 = Ootsuka | first1 = S. | last2 = Asami | first2 = S. | last3 = Sasaki | first3 = T. | last4 = Yoshida | first4 = Y. | last5 = Nemoto | first5 = N. | last6 = Shichino | first6 = H. | last7 = Chin | first7 = M. | last8 = Mugishima | first8 = H. | last9 = Suzuki | first9 = T. | title = Useful markers for detecting minimal residual disease in cases of neuroblastoma. | journal = Biol Pharm Bull | volume = 31 | issue = 6 | pages = 1071-4 | month = Jun | year = 2008 | doi =  | PMID = 18520032 }}</ref>
**PGP = protein gene product.
*NB-84 +ve.<ref name=pmid9500774>{{Cite journal  | last1 = Miettinen | first1 = M. | last2 = Chatten | first2 = J. | last3 = Paetau | first3 = A. | last4 = Stevenson | first4 = A. | title = Monoclonal antibody NB84 in the differential diagnosis of neuroblastoma and other small round cell tumors. | journal = Am J Surg Pathol | volume = 22 | issue = 3 | pages = 327-32 | month = Mar | year = 1998 | doi =  | PMID = 9500774 }}</ref>
**More sensitive that synaptophysin.
*Synaptophysin +ve.
*CD99 -ve.
 
===EM===
Distinctive EM appearance:<ref name=pmid1196755>{{Cite journal  | last1 = Mackay | first1 = B. | last2 = Masse | first2 = SR. | last3 = King | first3 = OY. | last4 = Butler | first4 = J. | title = Diagnosis of neuroblastoma by electron microscopy of bone marrow aspirates. | journal = Pediatrics | volume = 56 | issue = 6 | pages = 1045-9 | month = Dec | year = 1975 | doi =  | PMID = 1196755 }}</ref>
*Dendritic processes with longitudinally oriented microtubules.
*Membrane bound electron-dense granules (contain catecholamines).
*Desmosomes
**Not seen in [[EWS]], [[RMS]], lymphomas.
*Membrane densities.
 
Pertinent negative:<ref name=pmid1196755/>
*No glycogen.
**Seen in [[EWS]].


=See also=
=See also=

Revision as of 03:47, 16 November 2015

A drawing of the adrenal glands.

Adrenal gland is a little organ that hangs-out above the kidney. Pathologists rarely see it. It uncommonly is affected by tumours.

Anatomy & histology

Adrenal cortical rest redirects here.

Anatomy

  • Cortex.
  • Medulla.

Note:

  • Adrenal tissue may be associated with gonads or between gonads and adrenal gland proper.[1]

Microscopic

It is composed of a cortex and a medulla.

Cortex

It has three layers - mnemonic: GFR (from superficial to deep):

  1. Zona glomerulosa - salt (e.g. aldosterone).
    • Eosinophilic cytoplasm. (???)
    • Layer normally discontinuous.
  2. Zona fasciculata - sugar (e.g. cortisol).
    • Clear cytoplasm - key feature.
    • Largest part of the cortex ~ 70%.
    • Cells in cords/nests. (???)
  3. Zona reticularis - steroid (e.g. dehydroepiandrosterone).
    • Marked eosinophilia of cytoplasm - key feature.
    • Granular/reticular cytoplasm.

Note:

  • Normal cortex may not be completely encapsulated, i.e. the adrenal capsule may have defects.[2]
    • In other words: the cortex may "spill" into the surrounding fat.

Medulla

It consists of two cell types:[3]

  1. Chromaffin cells.
    • Arise of neural crest.
  2. Sustentacular cells (supporting cells).

Produce NED: norepinephrine, epinephrine, dopamine.

Images

  • Adrenal medulla. (WC)

  • Adrenal cortex & medulla (right of image), and tumour (left of image). (WC/Nephron)

www:

IHC

Adrenal cortex:[4]

  • Chromogranin A -ve.
  • Synaptophysin +ve.
  • Alpha-inhibin +ve.
  • Vimentin +ve.
  • Melan A +ve.
  • AE1/AE3 -ve.

Clinical

Patients getting a bilateral adrenalectomy get pre-treatment with steroids.[5]

Adrenal insufficiency is an immediate danger post-op.[6]

Benign

The section covers non-neoplastic pathologies of the adrenal gland. These uncommonly come to the pathologist.

  • Adrenal incidentalomas[7]
    • Adrenal tumors
    • Greater than 1 cm
    • Identified on imaging performed for other indications
  • Found in up to 10% of patients undergoing abdominal imaging.
  • Management problematic
    • Guidelines incorporate lesion size, functional status and imaging features.
    • Resection is generally advocated for
      • Functioning lesions.
      • Radiographic features suggestive of malignancy.
      • Growth during observation.

Stress response

Spironolactone bodies

Main article: Spironolactone bodies

Hemorrhagic adrenalitis

  • AKA Waterhouse-Friderichsen syndrome.

General

  • Classically thought to be only due to Neisseria meningitidis; however, more recently also associated with Staphylococcus aureus,[9] and Streptococcus pneumoniae.[10]

Gross

Features:

  • Massive haemorrhage within the substance of the adrenal gland.

DDx (autopsy):

  • Post-mortem changes.

Microscopic

Features:

  • Massive haemorrhage within the substance of the adrenal gland.

Image: Haemorrhage in adrenal (nih.gov).

  • Adrenal hemorrhage -low power - E. coli sepsis (SKB)

  • Adrenal hemorrhage - medium power - E. coli sepsis (SKB)

Adrenal cytomegaly

  • AKA adrenocortical cytomegaly.
  • AKA adrenal gland with cytomegaly.

General

May be associated with:[11]

Microscopic

Features:

  • Large cells in the adrenal cortex.[12]

Addison disease

General

  • Chronic adrenocortical insufficiency.

Clinical:

  • Brown skin - due POMC (a precursor of ACTH and melanocyte stimulating hormone (MSH)).[13]
  • Hypotension.
  • Nausea and vomiting.

DDx:[14]

Notes:

  • Secondary adrenocortical insufficiency (due to pituitary pathology):[15]
    • No hyperpigmentation (as no POMC).
    • Aldosterone usu. normal.

Microscopic

Features:[13]

  • Atrophy adrenal cortex - specifically zona fasciculata and zona reticularis.

Notes:

  • There is preservation of zona glomerulosa and medulla.

Benign neoplasms

Adrenal hemangioma

Radiographic incidentalomas but may be large and calcified raising a radiographic ddx of adrenal cortical carcinoma.

  • Rare.
  • 40 and 70 years.
  • 2:1 female-to-male ratio

  • Adrenal hemangioma - low power (SKB)

  • Adrenal hemangioma - medium power (SKB)

Adrenal cortical adenoma

Main article: Adrenal cortical adenoma

Pheochromocytoma

Main article: Pheochromocytoma

Adrenal ganglioneuroma

Main article: Ganglioneuroma

General

Gross

  • Solid.
  • White.
  • Firm.
  • Well-circumscribed.
  • May be nodular.

DDx (gross):

Images:

Microscopic

Features:

  • Ganglion cells - key feature.
    • Large cells with large nucleus.
      • Prominent nucleolus.
  • Disordered fibrinous material.

Images:

Adrenal myelolipoma

Main article: Adrenal myelolipoma

Adenomatoid tumour

See: Adenomatoid tumours (uterine tumours).

Malignant neoplasms

Adrenocortical carcinoma

  • AKA adrenal cortical carcinoma.
  • Abbreviated ACC.
Main article: Adrenocortical carcinoma

Neuroblastoma

Main article: Neuroblastoma

See also

References

  1. ↑ Barwick, TD.; Malhotra, A.; Webb, JA.; Savage, MO.; Reznek, RH. (Sep 2005). "Embryology of the adrenal glands and its relevance to diagnostic imaging.". Clin Radiol 60 (9): 953-9. doi:10.1016/j.crad.2005.04.006. PMID 16124976.
  2. ↑ Mills, Stacey E. (2012). Histology for Pathologists (4th ed.). Lippincott Williams & Wilkins. pp. 1236. ISBN 978-1451113037.
  3. ↑ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1159. ISBN 978-1416031215.
  4. ↑ De Padua, M.; Rajagopal, V. (May 2008). "Myxoid adrenal adenoma with focal pseudoglandular pattern.". Indian J Med Sci 62 (5): 199-203. PMID 18579979.
  5. ↑ URL: http://www3.interscience.wiley.com/cgi-bin/fulltext/119909358/PDFSTART. Accessed on: 21 August 2010.
  6. ↑ URL: http://ats.ctsnetjournals.org/cgi/content/full/62/5/1516. Accessed on: 21 August 2010.
  7. ↑ Aljabri, KS.; Bokhari, SA.; Alkeraithi, M.. "Adrenal hemangioma in a 19-year-old female.". Ann Saudi Med 31 (4): 421-3. doi:10.4103/0256-4947.76411. PMID 21293064.
  8. ↑ Becker MJ, Becker AE (September 1976). "Fat distribution in the adrenal cortex as an indication of the mode of intrauterine death". Hum. Pathol. 7 (5): 495–504. PMID 964978.
  9. ↑ Adem PV, Montgomery CP, Husain AN, et al. (September 2005). "Staphylococcus aureus sepsis and the Waterhouse-Friderichsen syndrome in children". N. Engl. J. Med. 353 (12): 1245–51. doi:10.1056/NEJMoa044194. PMID 16177250.
  10. ↑ Hamilton D, Harris MD, Foweraker J, Gresham GA (February 2004). "Waterhouse-Friderichsen syndrome as a result of non-meningococcal infection". J. Clin. Pathol. 57 (2): 208–9. PMC 1770213. PMID 14747454. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1770213/.
  11. ↑ URL: http://www.humpath.com/?adrenal-cytomegaly. Accessed on: 3 January 2012.
  12. ↑ 12.0 12.1 Aterman, K.; Kerenyi, N.; Lee, M. (1972). "Adrenal cytomegaly.". Virchows Arch A Pathol Pathol Anat 355 (2): 105-22. PMID 4336262.
  13. ↑ 13.0 13.1 Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1157. ISBN 978-1416031215.
  14. ↑ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1155. ISBN 978-1416031215.
  15. ↑ 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. 585. ISBN 978-1416054542.
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