Difference between revisions of "Adrenal gland"

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==Pheochromocytoma==
==Pheochromocytoma==
===General===
{{Main|Pheochromocytoma}}
*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.


==Adrenal ganglioneuroma==
==Adrenal ganglioneuroma==

Revision as of 02:47, 23 August 2014

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.

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,[7] and Streptococcus pneumoniae.[8]

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 cytomegaly

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

General

May be associated with:[9]

Microscopic

Features:

  • Large cells in the adrenal cortex.[10]

Addison disease

General

  • Chronic adrenocortical insufficiency.

Clinical:

  • Brown skin - due POMC (a precursor of ACTH and melanocyte stimulating hormone (MSH)).[11]
  • Hypotension.
  • Nausea and vomiting.

DDx:[12]

Notes:

  • Secondary adrenocortical insufficiency (due to pituitary pathology):[13]
    • No hyperpigmentation (as no POMC).
    • Aldosterone usu. normal.

Microscopic

Features:[11]

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

Notes:

  • There is preservation of zona glomerulosa and medulla.

Benign neoplasms

Adrenal cortical adenoma

General

Epidemiology:

  • Often an incidental finding.

Pathologic/clinical:

  • May be hormonally active.
  • Radiologists are good at identifying adenomas, as they are usually lipid rich and have a characteristic low HU signal.[14]

Indications for excision:[15][16]

  • Lesions >30 mm.
  • Hormonally active.
  • Non-incidental finding. (???)

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 cytoplasm.
  • May have foci of necrosis/degeneration and nuclear atypia.

Note:

  • In aldosterone producing tumours:

DDx:

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

Myelolipoma redirects here.

General

  • Benign and rare.
  • Typically asymptomatic and hormonally inactive.[19]
    • Symptoms: back or abdominal pain.
  • Diagnosis - usu. by abdominal CT.

Treatment:

  • Watchful waiting if small (<=7 cm) and asymptomatic.[19]

Microscopic

Features:[20]

  • Adipose tissue.
  • Hematopoietic elements from all three lineages:
    1. Erythroid.
    2. Myeloid.
    3. Megakaryocytic.
  • +/-Calcification.[19]

DDx:[21]

Images

  • Myelolipoma (WC/Mattopaedia)

www:

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:[22]

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

Classification:

Gross

Microscopic

Features:[25]

  • 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.[26]
  • 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:

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.[28]
    • PGP = protein gene product.
  • NB-84 +ve.[29]
    • More sensitive that synaptophysin.
  • Synaptophysin +ve.
  • CD99 -ve.

EM

Distinctive EM appearance:[30]

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

Pertinent negative:[30]

  • 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. ↑ 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.
  7. ↑ 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.
  8. ↑ 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/.
  9. ↑ URL: http://www.humpath.com/?adrenal-cytomegaly. Accessed on: 3 January 2012.
  10. ↑ 10.0 10.1 Aterman, K.; Kerenyi, N.; Lee, M. (1972). "Adrenal cytomegaly.". Virchows Arch A Pathol Pathol Anat 355 (2): 105-22. PMID 4336262.
  11. ↑ 11.0 11.1 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.
  12. ↑ 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.
  13. ↑ 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.
  14. ↑ URL: http://emedicine.medscape.com/article/376240-overview.
  15. ↑ Luton, JP.; Martinez, M.; Coste, J.; Bertherat, J. (Jul 2000). "Outcome in patients with adrenal incidentaloma selected for surgery: an analysis of 88 cases investigated in a single clinical center.". Eur J Endocrinol 143 (1): 111-7. PMID 10870039.
  16. ↑ Liu, XK.; Liu, XJ.; Dong, X.; Kong, CZ. (Jun 2008). "[Clinical research about treatment for adrenal incidentalomas]". Zhonghua Wai Ke Za Zhi 46 (11): 832-4. PMID 19035218.
  17. ↑ Thompson, Lester D. R. (2006). Endocrine Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 200. ISBN 978-0443066856.
  18. ↑ IAV. 18 February 2009.
  19. ↑ 19.0 19.1 19.2 Daneshmand, S.; Quek, ML. (2006). "Adrenal myelolipoma: diagnosis and management.". Urol J 3 (2): 71-4. PMID 17590837.
  20. ↑ 20.0 20.1 Cha, JS.; Shin, YS.; Kim, MK.; Kim, HJ. (Aug 2011). "Myelolipomas of both adrenal glands.". Korean J Urol 52 (8): 582-5. doi:10.4111/kju.2011.52.8.582. PMC 3162227. PMID 21927708. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3162227/.
  21. ↑ Lam, KY.; Lo, CY. (Sep 2001). "Adrenal lipomatous tumours: a 30 year clinicopathological experience at a single institution.". J Clin Pathol 54 (9): 707-12. PMID 11533079.
  22. ↑ 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.
  23. ↑ 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.
  24. ↑ 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.
  25. ↑ 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.
  26. ↑ Wippold FJ, Perry A (March 2006). "Neuropathology for the neuroradiologist: rosettes and pseudorosettes". AJNR Am J Neuroradiol 27 (3): 488–92. PMID 16551982.
  27. ↑ URL: http://radiographics.rsna.org/content/28/4/1193.full. Accessed on: 12 January 2011.
  28. ↑ 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.
  29. ↑ 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.
  30. ↑ 30.0 30.1 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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