Difference between revisions of "Adrenal gland"
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*Cortex. | *Cortex. | ||
*Medulla. | *Medulla. | ||
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*Adrenal tissue may be associated with gonads or between gonads and adrenal gland proper.<ref>{{Cite journal | last1 = Barwick | first1 = TD. | last2 = Malhotra | first2 = A. | last3 = Webb | first3 = JA. | last4 = Savage | first4 = MO. | last5 = Reznek | first5 = RH. | title = Embryology of the adrenal glands and its relevance to diagnostic imaging. | journal = Clin Radiol | volume = 60 | issue = 9 | pages = 953-9 | month = Sep | year = 2005 | doi = 10.1016/j.crad.2005.04.006 | PMID = 16124976 }}</ref> | |||
===Microscopic=== | ===Microscopic=== |
Revision as of 04:11, 17 July 2015
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):
- Zona glomerulosa - salt (e.g. aldosterone).
- Eosinophilic cytoplasm. (???)
- Layer normally discontinuous.
- 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.
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]
- Chromaffin cells.
- Arise of neural crest.
- Sustentacular cells (supporting cells).
Produce NED: norepinephrine, epinephrine, dopamine.
Images
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
- In fetuses - fat content increases due to stress[8] -- see: Fetal_autopsy#Adrenal_fetal_fat_pattern.
- In newborns/children/adults - fat content decreases due to stress.
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 cytomegaly
General
May be associated with:[11]
- Beckwith-Wiedemann syndrome.
- Prematurity.
- Rh-incompatibility.[12]
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]
- POMC presence implies the pituitary gland intact.
- Hypotension.
- Nausea and vomiting.
DDx:[14]
- Autoimmune.
- Tuberculosis.
- AIDS.
- Malignancy.
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 cortical adenoma
Pheochromocytoma
Adrenal ganglioneuroma
General
- May be retroperitoneal.
- Multiple ganglioneuromas may be due to multiple endocrine neoplasia IIb.
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.
- Large cells with large nucleus.
- Disordered fibrinous material.
Images:
Adrenal myelolipoma
Adenomatoid tumour
See: Adenomatoid tumours (uterine tumours).
Malignant neoplasms
Adrenocortical carcinoma
- AKA adrenal cortical carcinoma.
- Abbreviated ACC.
Neuroblastoma
- See also: olfactory neuroblastoma.
General
Epidemiology:
- Usually paediatric population.
Laboratory findings:
- Increased urine homovanillic acid.
Predictors of a poor prognosis:[16]
- 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:[17]
- Ganglioneuroma (benign).
- Ganglioneuroblastoma (intermediate).
- Neuroblastoma (aggressive).
Gross
- Typically an abdominal mass.
- ~40% arise in the adrenal gland.[18]
Microscopic
Features:[19]
- 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.[20]
- 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:
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.[22]
- PGP = protein gene product.
- NB-84 +ve.[23]
- More sensitive that synaptophysin.
- Synaptophysin +ve.
- CD99 -ve.
EM
Distinctive EM appearance:[24]
- Dendritic processes with longitudinally oriented microtubules.
- Membrane bound electron-dense granules (contain catecholamines).
- Desmosomes
- Membrane densities.
Pertinent negative:[24]
- No glycogen.
- Seen in EWS.
See also
References
- ↑ 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.
- ↑ Mills, Stacey E. (2012). Histology for Pathologists (4th ed.). Lippincott Williams & Wilkins. pp. 1236. ISBN 978-1451113037.
- ↑ 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.
- ↑ De Padua, M.; Rajagopal, V. (May 2008). "Myxoid adrenal adenoma with focal pseudoglandular pattern.". Indian J Med Sci 62 (5): 199-203. PMID 18579979.
- ↑ URL: http://www3.interscience.wiley.com/cgi-bin/fulltext/119909358/PDFSTART. Accessed on: 21 August 2010.
- ↑ URL: http://ats.ctsnetjournals.org/cgi/content/full/62/5/1516. Accessed on: 21 August 2010.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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/.
- ↑ URL: http://www.humpath.com/?adrenal-cytomegaly. Accessed on: 3 January 2012.
- ↑ 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.
- ↑ 13.0 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Wippold FJ, Perry A (March 2006). "Neuropathology for the neuroradiologist: rosettes and pseudorosettes". AJNR Am J Neuroradiol 27 (3): 488–92. PMID 16551982.
- ↑ URL: http://radiographics.rsna.org/content/28/4/1193.full. Accessed on: 12 January 2011.
- ↑ 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.
- ↑ 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.
- ↑ 24.0 24.1 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.