Adrenal gland

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

Anatomy & histology

Histology

Composed for cortex and medulla.

  • Cortex has three layers - Mnemonic: GFR (from superficial to deep):
    • Zona glomerulosa - salt (e.g. aldosterone)
      • eosinophilic cytoplasm???
      • Normally discontinuous layer.
    • Zona fasciculata - sugar (e.g. cortisol)
      • Clear cytoplasm - key feature.
      • Largest part of the cortex ~ 70%.
      • Cells in cords/nests???
    • Zona reticularis - steroid (e.g. dehydroepiandrosterone).
      • Marked eosinophilia of cytoplasm - key feature.
      • Granular/reticular cytoplasm.
  • Medulla - produces NED: norepinephrine, epinephrine, dopamine.

Clinical

Patients getting a bilat. adrenalectomy get pre-treatment with steroids.[1]

Adrenal insuff. may be immediately post-op.[2]

Benign

Spironolactone bodies

Features:[3]

  • Location: zona glomerulosa (where aldosterone is produced).
  • Appearance: eosinophilic spherical laminated whorls.
  • Etiology: long-term use of spironolactone.

Images:

Hemorrhagic adrenalitis

General

  • AKA Waterhouse-Friderichsen syndrome.
  • Classically thought to be only due to Neisseria meningitidis; however, more recently also associated with Streptococcus aureus.[4][5]

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).

Benign neoplasms

Adenomas

Radiology[6]

  • Radiologists are good at identifying adenomas, as they are usually lipid rich and have a characteristic low HU signal.

Treatment is excision if...[7][8]

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

Hyperplasia vs. adenoma

  • Hyperplasia is multifocal.[9]

Adrenal cortical adenoma

Epidemiology

  • Often an incidental finding.

Pathologic/clinical:

  • May be hormonally active.

Histology

Classic features:

  • Well-defined cell borders.
  • Clear cytoplasm.
  • May have foci of necrosis/degeneration and nuclear atypia.

In aldosterone producing tumours:

  • May extend outside of the capsule (should not be diagnosed as adrenal cortical carcinoma.
  • No atrophy of non-hyperplastic cortex.

In cortisol producing tumours:

  • Atrophy of the non-hyperplastic cortex (due to feedback inhibition from the pituitary gland).

Pheochromocytoma

General

Clinical

  • Paroxysms (i.e. episodic) tachycardia, headache, anxiety.

Epidemiology

  • Tumour arises from medulla
  • Literally means "dusky" (pheo) "colour" (chromo) - dull appearance on gross

Histology

Features:

  • Architecture:
    • Cell nests, auf deutsch: Zellballen (literally Cell balls).
      • Useful for differentiating from ACC.
  • Nuclei.
    • +/-Pleomorphism.
    • Nucleoli may be prominent (not signif. prognostically).
  • Cellular morphology.
    • Polygonal cells.
  • Cytoplasm.
    • Basophilic, granular.
  • Other.
    • Haemorrhagic.

Ganglioneuroma

Microscopic

Features:

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

Images:

See: CNS tumours.

Myelolipoma

Adenomatoid tumour

See: Adenomatoid tumours (uterine tumours).

Malignant neoplasms

Adrenocortical carcinoma

  • AKA adrenal cortical carcinoma.
  • Abbreviated ACC.

General

  • Prognosis sucks.

Gross

  • +/-Encapsulated.
  • Necrotic-appearing.

Image:

Microscopic

Various criteria exist for this diagnosis. The most widely used is the Weiss criteria, which is a big long clunker.

Image:

Notes:

  • Tumour may contain fat.[11]

Adult

Weiss criteria

Three of the following:[12]

  1. High nuclear grade.
  2. High mitotic rate; >5/50 HPF (@ 40X obj.) - definition suffers from HPFitis.
  3. Atypical mitoses.
  4. Cleared cytoplasm in >= 25% of tumour cells.
  5. Sheeting (diffuse architecture) in >= 1/3 of tumour cells.
  6. Necrosis in nests.
  7. Venous invasion.
  8. Adrenal sinusoid invasion; lymphovascular space invasion within the adrenal gland.
  9. Capsular invasion.
Volante criteria

There is a simplified set of criteria by Volante et al. - that is not widely used:[13]

  • Reticular network disruption (with reticulin staining).
  • One of the three following:
    1. Abundant mitoses >5/50 high-power fields - definition suffers from HPFitis.
    2. Necrosis.
    3. Vascular invasion.

Pediatric

The criteria in the pediatric setting are somewhat different. This is discussed by Wieneke et al.[14] and Dehner and Hill.[15]

Dehner and Hill propose a very simple system:[15]

  • "Low risk" < 200 g & confined to the adrenal.
  • "Intermediate risk" 200-400 g, no mets, +/-microscopic disease outside adrenal.
  • "High risk" >400 g, or mets, or gross invasion of adjacent structures.

IHC

  • Vimentin +ve.
  • Melan A +ve.
  • Inhibin-alpha +ve.
  • Cytokeratins +ve/-ve.

Others:

Malignant pheochromoctyoma

  • Like the description in benign neoplasms.
  • Differentiated from benign pheochromocytoma by mets - often aided by radiologic report.
  • Features useful for differentiating benign from malignant:[16]
    • Marked nuclear atypia.
    • Invasion:
      • Capsular.
      • Vascular.
    • Necrosis.
    • Cellular monotony.
    • Mitoses:
      • Rate.
      • Atypical mitosis.

Neuroblastoma

General

  • Clinical: increased urine homovanillic acid.

Epidemiology:

  • Usually paediatric population.

Microscopic

Features:[17]

  • 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.[18]

Notes:

  • The fibrous septa are esp. useful for differentiation from lymphoma.

DDx:

Subtypes

  • Several subtypes exist.[19]

Images:

See also

References

  1. ↑ URL: http://www3.interscience.wiley.com/cgi-bin/fulltext/119909358/PDFSTART. Accessed on: 21 August 2010.
  2. ↑ URL: http://ats.ctsnetjournals.org/cgi/content/full/62/5/1516. Accessed on: 21 August 2010.
  3. ↑ Kovacs K, Horvath E, Singer W (December 1973). "Fine structure and morphogenesis of spironolactone bodies in the zona glomerulosa of the human adrenal cortex". J. Clin. Pathol. 26 (12): 949-57. PMC 477936. PMID 4131694. http://jcp.bmj.com/cgi/pmidlookup?view=long&pmid=4131694.
  4. ↑ 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.
  5. ↑ 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/.
  6. ↑ URL: http://emedicine.medscape.com/article/376240-overview.
  7. ↑ 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.
  8. ↑ 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.
  9. ↑ IAV. 18 February 2009.
  10. ↑ Thompson, Lester D. R. (2006). Endocrine Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 327. ISBN 978-0443066856.
  11. ↑ Heye S, Woestenborghs H, Van Kerkhove F, Oyen R (2005). "Adrenocortical carcinoma with fat inclusion: case report". Abdom Imaging 30 (5): 641–3. doi:10.1007/s00261-004-0281-5. PMID 15688105.
  12. ↑ Jain M, Kapoor S, Mishra A, Gupta S, Agarwal A (2010). "Weiss criteria in large adrenocortical tumors: a validation study". Indian J Pathol Microbiol 53 (2): 222–6. doi:10.4103/0377-4929.64325. PMID 20551521.
  13. ↑ Volante M, Bollito E, Sperone P, et al. (November 2009). "Clinicopathological study of a series of 92 adrenocortical carcinomas: from a proposal of simplified diagnostic algorithm to prognostic stratification". Histopathology 55 (5): 535–43. doi:10.1111/j.1365-2559.2009.03423.x. PMID 19912359.
  14. ↑ Wieneke JA, Thompson LD, Heffess CS (July 2003). "Adrenal cortical neoplasms in the pediatric population: a clinicopathologic and immunophenotypic analysis of 83 patients". Am. J. Surg. Pathol. 27 (7): 867–81. PMID 12826878.
  15. ↑ 15.0 15.1 Dehner LP, Hill DA (2009). "Adrenal cortical neoplasms in children: why so many carcinomas and yet so many survivors?". Pediatr. Dev. Pathol. 12 (4): 284–91. doi:10.2350/08-06-0489.1. PMID 19326954.
  16. ↑ Thompson, Lester D. R. (2006). Endocrine Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 259. ISBN 978-0443066856.
  17. ↑ 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.
  18. ↑ Wippold FJ, Perry A (March 2006). "Neuropathology for the neuroradiologist: rosettes and pseudorosettes". AJNR Am J Neuroradiol 27 (3): 488–92. PMID 16551982.
  19. ↑ 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.
  20. ↑ URL: http://radiographics.rsna.org/content/28/4/1193.full. Accessed on: 12 January 2011.
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