Difference between revisions of "Adrenal gland"

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==Adrenal cortical adenoma==
==Adrenal cortical adenoma==
===General===
{{Main|Adrenal cortical adenoma}}
Epidemiology:
*Often an incidental finding.
 
Pathologic/clinical:
*May be hormonally active.
*Can be a cause of [[hypertension]].<ref name=pmid18584586/>
 
*Radiologists are good at identifying adenomas, as they are usually lipid rich and have a characteristic low HU signal.<ref>URL: [http://emedicine.medscape.com/article/376240-overview http://emedicine.medscape.com/article/376240-overview].</ref>
**Microadenomas may be missed.<ref name=pmid18584586/><ref name=pmid20881759>{{Cite journal  | last1 = Fujiwara | first1 = M. | last2 = Murao | first2 = K. | last3 = Imachi | first3 = H. | last4 = Yoshida | first4 = K. | last5 = Muraoka | first5 = T. | last6 = Ohyama | first6 = T. | last7 = Kushida | first7 = Y. | last8 = Haba | first8 = R. | last9 = Kakehi | first9 = Y. | title = Misdiagnosis of two cases of primary aldosteronism owing to failure of computed tomography to detect adrenal microadenoma. | journal = Am J Med Sci | volume = 340 | issue = 4 | pages = 335-7 | month = Oct | year = 2010 | doi = 10.1097/MAJ.0b013e3181e95587 | PMID = 20881759 }}</ref>
 
Indications for excision:<ref name=pmid10870039>{{Cite journal  | last1 = Luton | first1 = JP. | last2 = Martinez | first2 = M. | last3 = Coste | first3 = J. | last4 = Bertherat | first4 = J. | title = Outcome in patients with adrenal incidentaloma selected for surgery: an analysis of 88 cases investigated in a single clinical center. | journal = Eur J Endocrinol | volume = 143 | issue = 1 | pages = 111-7 | month = Jul | year = 2000 | doi =  | PMID = 10870039 }}
</ref><ref name=pmid19035218>{{Cite journal  | last1 = Liu | first1 = XK. | last2 = Liu | first2 = XJ. | last3 = Dong | first3 = X. | last4 = Kong | first4 = CZ. | title = [Clinical research about treatment for adrenal incidentalomas] | journal = Zhonghua Wai Ke Za Zhi | volume = 46 | issue = 11 | pages = 832-4 | month = Jun | year = 2008 | doi =  | PMID = 19035218 }}</ref>
*Lesions >30 mm.
*Hormonally active.
*Non-incidental finding. (???)
*Adrenal vein sampling (AVS) suggestive of adenoma.<ref name=pmid18584586>{{Cite journal  | last1 = Myint | first1 = KS. | last2 = Watts | first2 = M. | last3 = Appleton | first3 = DS. | last4 = Lomas | first4 = DJ. | last5 = Jamieson | first5 = N. | last6 = Taylor | first6 = KP. | last7 = Coghill | first7 = S. | last8 = Brown | first8 = MJ. | title = Primary hyperaldosteronism due to adrenal microadenoma: a curable cause of refractory hypertension. | journal = J Renin Angiotensin Aldosterone Syst | volume = 9 | issue = 2 | pages = 103-6 | month = Jun | year = 2008 | doi = 10.3317/jraas.2008.015 | PMID = 18584586 }}</ref>
 
Notes:
*[[Cushing disease]] is due to the ACTH over-production by the [[pituitary]].
*In cortisol producing tumours (''Cushing syndrome''): atrophy of the non-hyperplastic cortex (due to feedback inhibition from the [[pituitary gland]]).
 
===Microscopic===
Classic features:
*Well-defined cell borders.
*Clear cells (abundant, finely vacuolated cytoplasm)
*Polygonal pink cells.
*Most of the nuclei are bland, central and round.
*May have foci of [[necrosis]]/degeneration and nuclear atypia.
 
<gallery>
Image: Adrenal CorticalAdenoma DSCN5001 PA.JPG|Adrenal Cortical Adenoma (SKB)
Image: Adrenal CorticalAdenoma DSCN5002 PA.JPG|Adrenal Cortical Adenoma (SKB)
Image: Adrenal CorticalAdenoma DSCN5004 PA.JPG|Adrenal Cortical Adenoma (SKB)
Image: Adrenal CorticalAdenoma DSCN5005 PA.JPG|Adrenal Cortical Adenoma (SKB)
Image: Adrenal CorticalAdenoma MP CTR.jpg|Adrenal Cortical Adenoma - Medium power (SKB)
Image: Adrenal CorticalAdenoma HP CTR.jpg|Adrenal Cortical Adenoma - High power.  Abundant clear cytoplasm.  Round, regular nuclei. (SKB)
Image: Adrenal CorticalAdenoma MP PA.JPG|Adrenal Cortical Adenoma - Some pleomorphism - Medium power (SKB)
Image: Adrenal LipoAdenoma MP PA.JPG|Adrenal cortical adenoma with fat - "lipoadenoma" (SKB)
</gallery>
 
Note:
*In aldosterone producing tumours:
**May extend outside of the capsule (should ''not'' be diagnosed as ''[[adrenal cortical carcinoma]]'').
**No atrophy of non-hyperplastic cortex.
**May show spironolactone bodies if hypertension treated with spironolactone prior to surgery.
 
DDx:
*Adrenal cortical nodule.<ref name=Ref_EP200>{{Ref EP|200}}</ref>
*[[Adrenal cortical hyperplasia]].
**Hyperplasia is multifocal.<ref>IAV. 18 February 2009.</ref>
*[[Adrenal cortical carcinoma]].


==Pheochromocytoma==
==Pheochromocytoma==

Revision as of 05:53, 9 May 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.

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.[1]
    • In other words: the cortex may "spill" into the surrounding fat.

Medulla

It consists of two cell types:[2]

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

Produce NED: norepinephrine, epinephrine, dopamine.

Images

  • Adrenal medulla. (WC)

  • Adrenal cortex, medulla and tumour. (WC/Nephron)

www:

IHC

Adrenal cortex:[3]

  • 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.[4]

Adrenal insufficiency is an immediate danger post-op.[5]

Benign

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

  • Adrenal incidentalomas[6]
    • 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,[8] and Streptococcus pneumoniae.[9]

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:[10]

Microscopic

Features:

  • Large cells in the adrenal cortex.[11]

Addison disease

General

  • Chronic adrenocortical insufficiency.

Clinical:

  • Brown skin - due POMC (a precursor of ACTH and melanocyte stimulating hormone (MSH)).[12]
  • Hypotension.
  • Nausea and vomiting.

DDx:[13]

Notes:

  • Secondary adrenocortical insufficiency (due to pituitary pathology):[14]
    • No hyperpigmentation (as no POMC).
    • Aldosterone usu. normal.

Microscopic

Features:[12]

  • 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

See also: olfactory neuroblastoma.

General

Epidemiology:

  • Usually paediatric population.

Laboratory findings:

  • Increased urine homovanillic acid.

Predictors of a poor prognosis:[15]

  • High mitotic-karyorrhectic index.
  • Lack of schwannian stroma.
  • >18 months.
  • Near ploidy.
  • N-MYC amplification.
  • Lymph node spread.
  • Distant spread.

Classification:

Gross

Microscopic

Features:[18]

  • Small round blue cells separated by thin (pink) fibrous septa.
  • Homer-Wright rosettes.
    • Rosette with a small (~100 micrometers - diameter) meshwork of fibers (neuropil) at the centre.[19]
  • 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:

Images:

  • Neuroblastoma - medium power]]

  • Neuroblastoma - medium power (SKB)

  • Neuroblastoma - medium power]]

  • Neuroblastoma - vascular invasion - medium power]]

  • Neuroblastoma - medium power]]

  • Neuroblastoma - medium power]]

  • Neuroblastoma - high power (SKB)

  • Neuroblastoma - high power - blue cells arrayed around a core of fluffy pink neuropil (SKB)

  • Neuroblastoma - high power]]

  • Neuroblastoma - high power]]

Schwannian vs. neuropil

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.[21]
    • PGP = protein gene product.
  • NB-84 +ve.[22]
    • More sensitive that synaptophysin.
  • Synaptophysin +ve.
  • CD99 -ve.

EM

Distinctive EM appearance:[23]

  • Dendritic processes with longitudinally oriented microtubules.
  • Membrane bound electron-dense granules (contain catecholamines).
  • Desmosomes
    • Not seen in EWS, RMS, lymphomas.
  • Membrane densities.

Pertinent negative:[23]

  • No glycogen.

See also

References

  1. ↑ Mills, Stacey E. (2012). Histology for Pathologists (4th ed.). Lippincott Williams & Wilkins. pp. 1236. ISBN 978-1451113037.
  2. ↑ 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.
  3. ↑ De Padua, M.; Rajagopal, V. (May 2008). "Myxoid adrenal adenoma with focal pseudoglandular pattern.". Indian J Med Sci 62 (5): 199-203. PMID 18579979.
  4. ↑ URL: http://www3.interscience.wiley.com/cgi-bin/fulltext/119909358/PDFSTART. Accessed on: 21 August 2010.
  5. ↑ URL: http://ats.ctsnetjournals.org/cgi/content/full/62/5/1516. Accessed on: 21 August 2010.
  6. ↑ 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.
  7. ↑ 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.
  8. ↑ 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.
  9. ↑ 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/.
  10. ↑ URL: http://www.humpath.com/?adrenal-cytomegaly. Accessed on: 3 January 2012.
  11. ↑ 11.0 11.1 Aterman, K.; Kerenyi, N.; Lee, M. (1972). "Adrenal cytomegaly.". Virchows Arch A Pathol Pathol Anat 355 (2): 105-22. PMID 4336262.
  12. ↑ 12.0 12.1 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.
  13. ↑ 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.
  14. ↑ 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.
  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. 254. ISBN 978-1416054542.
  16. ↑ Shimada H, Ambros IM, Dehner LP, Hata J, Joshi VV, Roald B (July 1999). "Terminology and morphologic criteria of neuroblastic tumors: recommendations by the International Neuroblastoma Pathology Committee". Cancer 86 (2): 349–63. PMID 10421272.
  17. ↑ 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. 253. ISBN 978-1416054542.
  18. ↑ Chung EM, Murphey MD, Specht CS, Cube R, Smirniotopoulos JG (2008). "From the Archives of the AFIP. Pediatric orbit tumors and tumorlike lesions: osseous lesions of the orbit". Radiographics 28 (4): 1193–214. doi:10.1148/rg.284085013. PMID 18635637.
  19. ↑ Wippold FJ, Perry A (March 2006). "Neuropathology for the neuroradiologist: rosettes and pseudorosettes". AJNR Am J Neuroradiol 27 (3): 488–92. PMID 16551982.
  20. ↑ URL: http://radiographics.rsna.org/content/28/4/1193.full. Accessed on: 12 January 2011.
  21. ↑ Ootsuka, S.; Asami, S.; Sasaki, T.; Yoshida, Y.; Nemoto, N.; Shichino, H.; Chin, M.; Mugishima, H. et al. (Jun 2008). "Useful markers for detecting minimal residual disease in cases of neuroblastoma.". Biol Pharm Bull 31 (6): 1071-4. PMID 18520032.
  22. ↑ Miettinen, M.; Chatten, J.; Paetau, A.; Stevenson, A. (Mar 1998). "Monoclonal antibody NB84 in the differential diagnosis of neuroblastoma and other small round cell tumors.". Am J Surg Pathol 22 (3): 327-32. PMID 9500774.
  23. ↑ 23.0 23.1 Mackay, B.; Masse, SR.; King, OY.; Butler, J. (Dec 1975). "Diagnosis of neuroblastoma by electron microscopy of bone marrow aspirates.". Pediatrics 56 (6): 1045-9. PMID 1196755.
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