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| *Cortex. | | *Cortex. |
| *Medulla. | | *Medulla. |
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| | Note: |
| | *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> |
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| ===Microscopic=== | | ===Microscopic=== |
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| <gallery> | | <gallery> |
| Image:Adrenal_gland_(medulla).JPG | Adrenal medulla. (WC) | | Image:Adrenal_gland_(medulla).JPG | Adrenal medulla. (WC) |
| Image:Adrenal_cortical_carcinoma_-_low_mag.jpg | Adrenal cortex, medulla and tumour. (WC/Nephron) | | Image:Adrenal_cortical_carcinoma_-_low_mag.jpg | Adrenal cortex & medulla (right of image), and tumour (left of image). (WC/Nephron) |
| </gallery> | | </gallery> |
| www: | | <gallery> |
| | Image:Adrenal rest - epididymis -- low mag.jpg | Adrenal rest - low mag. (WC/Nephron) |
| | Image:Adrenal rest - epididymis -- intermed mag.jpg | Adrenal rest - intermed. mag. (WC/Nephron) |
| | Image:Adrenal rest - epididymis -- high mag.jpg | Adrenal rest - high mag. (WC/Nephron) |
| | </gallery> |
| | |
| | =====www===== |
| *[http://www.webpathology.com/image.asp?case=78&n=5 Adrenal medulla (webpathology.com)]. | | *[http://www.webpathology.com/image.asp?case=78&n=5 Adrenal medulla (webpathology.com)]. |
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| *Melan A +ve. | | *Melan A +ve. |
| *AE1/AE3 -ve. | | *AE1/AE3 -ve. |
| | *RCC -ve (0 +ve of 63 cases<ref name=pmid21490444>{{Cite journal | last1 = Sangoi | first1 = AR. | last2 = Fujiwara | first2 = M. | last3 = West | first3 = RB. | last4 = Montgomery | first4 = KD. | last5 = Bonventre | first5 = JV. | last6 = Higgins | first6 = JP. | last7 = Rouse | first7 = RV. | last8 = Gokden | first8 = N. | last9 = McKenney | first9 = JK. | title = Immunohistochemical distinction of primary adrenal cortical lesions from metastatic clear cell renal cell carcinoma: a study of 248 cases. | journal = Am J Surg Pathol | volume = 35 | issue = 5 | pages = 678-86 | month = May | year = 2011 | doi = 10.1097/PAS.0b013e3182152629 | PMID = 21490444 }}</ref>). |
| | *EMA -ve (0 +ve of 63 cases<ref name=pmid21490444/>). |
| | |
| | A panel that may be useful for [[adrenal cortical adenoma|adenoma]] versus [[adrenal cortical carcinoma|carcinoma]]:<ref name=pmid26317117>{{Cite journal | last1 = Kovach | first1 = AE. | last2 = Nucera | first2 = C. | last3 = Lam | first3 = QT. | last4 = Nguyen | first4 = A. | last5 = Dias-Santagata | first5 = D. | last6 = Sadow | first6 = PM. | title = Genomic and immunohistochemical analysis in human adrenal cortical neoplasia reveal beta-catenin mutations as potential prognostic biomarker. | journal = Discoveries (Craiova) | volume = 3 | issue = 2 | pages = | month = | year = | doi = 10.15190/d.2015.32 | PMID = 26317117 }} |
| | </ref><ref name=pmid11196463>{{Cite journal | last1 = Arola | first1 = J. | last2 = Salmenkivi | first2 = K. | last3 = Liu | first3 = J. | last4 = Kahri | first4 = AI. | last5 = Heikkilä | first5 = P. | title = p53 and Ki67 in adrenocortical tumors. | journal = Endocr Res | volume = 26 | issue = 4 | pages = 861-5 | month = Nov | year = 2000 | doi = | PMID = 11196463 }}</ref> |
| | *Beta-catenin, p53, reticulin, inhibin, melan A, Ki-67. |
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| ==Clinical== | | ==Clinical== |
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| =Benign neoplasms= | | =Benign neoplasms= |
| ==Adrenal cortical adenoma==
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| ===General===
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| Epidemiology:
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| *Often an incidental finding.
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| Pathologic/clinical:
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| *May be hormonally active.
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| *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>
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| 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 }}
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| </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>
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| *Lesions >30 mm.
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| *Hormonally active.
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| *Non-incidental finding. (???)
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| Notes:
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| *[[Cushing disease]] is due to the ACTH over-production by the [[pituitary]].
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| *In cortisol producing tumours (''Cushing syndrome''): atrophy of the non-hyperplastic cortex (due to feedback inhibition from the [[pituitary gland]]).
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|
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| ===Microscopic===
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| Classic features:
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| *Well-defined cell borders.
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| *Clear cells (abundant, finely vacuolated cytoplasm)
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| *Polygonal pink cells.
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| *Most of the nuclei are bland, central and round.
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| *May have foci of [[necrosis]]/degeneration and nuclear atypia.
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| | ==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 |
| <gallery> | | <gallery> |
| Image: Adrenal CorticalAdenoma DSCN5001 PA.JPG|Adrenal Cortical Adenoma (SKB) | | Image:Adrenal Hemangioma LP CTR.jpg|Adrenal hemangioma - low power (SKB) |
| Image: Adrenal CorticalAdenoma DSCN5002 PA.JPG|Adrenal Cortical Adenoma (SKB)
| | Image:Adrenal Hemangioma MP CTR.jpg|Adrenal hemangioma - medium power (SKB) |
| Image: Adrenal CorticalAdenoma DSCN5004 PA.JPG|Adrenal Cortical Adenoma (SKB)
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| Image: Adrenal CorticalAdenoma DSCN5005 PA.JPG|Adrenal Cortical Adenoma (SKB)
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| Image: Adrenal CorticalAdenoma MP CTR.jpg|Adrenal Cortical Adenoma - Medium power (SKB)
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| 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)
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| Image: Adrenal LipoAdenoma MP PA.JPG|Adrenal cortical adenoma with fat - "lipoadenoma" (SKB)
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| </gallery> | | </gallery> |
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| Note:
| | ==Adrenal cortical adenoma== |
| *In aldosterone producing tumours:
| | {{Main|Adrenal cortical adenoma}} |
| **May extend outside of the capsule (should ''not'' be diagnosed as ''[[adrenal cortical carcinoma]]'').
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| **No atrophy of non-hyperplastic cortex.
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| **May show spironolactone bodies if hypertension treated with spironolactone prior to surgery.
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| DDx:
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| *Adrenal cortical nodule.<ref name=Ref_EP200>{{Ref EP|200}}</ref>
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| *[[Adrenal cortical hyperplasia]].
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| **Hyperplasia is multifocal.<ref>IAV. 18 February 2009.</ref>
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| *[[Adrenal cortical carcinoma]].
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| ==Pheochromocytoma== | | ==Pheochromocytoma== |
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| ==Neuroblastoma== | | ==Neuroblastoma== |
| :See also: ''[[olfactory neuroblastoma]]''.
| | {{Main|Neuroblastoma}} |
| ===General===
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| Epidemiology:
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| *Usually paediatric population.
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| Laboratory findings:
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| *Increased urine homovanillic acid.
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| Predictors of a poor prognosis:<ref name=Ref_PCPBoD8_254>{{Ref PCPBoD8|254}}</ref>
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| *High mitotic-karyorrhectic index.
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| *Lack of schwannian stroma.
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| *>18 months.
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| *Near ploidy.
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| *N-MYC amplification.
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| *Lymph node spread.
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| *Distant spread.
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| Classification:
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| *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>
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| **[[Ganglioneuroma]] (benign).
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| **[[Ganglioneuroblastoma]] (intermediate).
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| **Neuroblastoma (aggressive).
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| ===Gross===
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| *Typically an abdominal mass.
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| **~40% arise in the [[adrenal gland]].<ref name=Ref_PCPBoD8_253>{{Ref PCPBoD8|253}}</ref>
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| ===Microscopic===
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| 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>
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| *[[small round cell tumour|Small round blue cell]]s separated by thin (pink) fibrous septa.
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| *Homer-Wright rosettes.
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| **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>
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| *Neuropil-like stroma = paucicellular stroma with a cotton candy-like appearance; see comparison below.
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| **>50% neuropil-like stroma -- otherwise it's a [[ganglioneurona]] or ganglioblastoma.
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| Notes:
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| *The fibrous septa are especially useful for differentiation from lymphoma.
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| DDx:
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| *[[Small round cell tumours]].
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| **[[Wilms tumour]].
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| **Lymphoma.
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| **[[Hepatoblastoma]].
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| Images:
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| <gallery>
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| Image:Adrenal Neuroblastoma MP CTR.jpg|Neuroblastoma - medium power]]
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| Image:Adrenal Neuroblastoma 2 MP CTR.jpg|Neuroblastoma - medium power (SKB)
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| Image:Adrenal Neuroblastoma M2P PA.JPG|Neuroblastoma - medium power]]
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| Image:Adrenal Neuroblastoma VascularInvasion MP CTR.jpg|thumb|Neuroblastoma - vascular invasion - medium power]]
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| Image:Adrenal Neuroblastoma MP3 PA.JPG|Neuroblastoma - medium power]]
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| Image:Adrenal Neuroblastoma MP PA.JPG|Neuroblastoma - medium power]]
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| Image:Adrenal Neuroblastoma 2 HP CTR.jpg|Neuroblastoma - high power (SKB)
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| Image:Adrenal Neuroblastoma 3 HP CTR.jpg|Neuroblastoma - high power - blue cells arrayed around a core of fluffy pink neuropil (SKB)
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| Image:Adrenal Neuroblastoma HP2 CTR.jpg|Neuroblastoma - high power]]
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| Image:Adrenal Neuroblastoma HP CTR.jpg|Neuroblastoma - high power]]
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| </gallery>
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| *[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>
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| *[http://commons.wikimedia.org/wiki/File:Neuroblastoma_rosettes.jpg Neuroblastoma (WC)].
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| *[http://farm3.static.flickr.com/2259/2274260465_afbea05f9b.jpg Neuroblastoma (flickr.com)].
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| | |
| ====Schwannian vs. neuropil====
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| {| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
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| | Feature
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| | Schwannian
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| | Neuropil
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| |-
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| | Cellularity
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| | high ~ spacing of cells < 30 µm
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| | low ~ spacing of cells > 100 µm
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| |-
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| | Fibrillary
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| | yes, long fine strands
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| | no
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| |-
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| | Associations
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| | ganglion cells
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| | neuroblasts
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| |-
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| | Cytoplasmic vacuolation
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| | yes
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| | ?
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| |-
| |
| |}
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| | |
| ====Classification/grading====
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| Commonly grouped by the ''Shimada classification'', which depends on the presence a number of things including:
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| *Mitoses/karyorrhectic cells.
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| *Molecular abnormalities.
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| ===IHC===
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| *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.
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| *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>
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| **More sensitive that synaptophysin.
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| *Synaptophysin +ve.
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| *CD99 -ve.
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| ===EM===
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| 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>
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| *Dendritic processes with longitudinally oriented microtubules.
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| *Membrane bound electron-dense granules (contain catecholamines).
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| *Desmosomes
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| **Not seen in [[EWS]], [[RMS]], lymphomas.
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| *Membrane densities.
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| Pertinent negative:<ref name=pmid1196755/>
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| *No glycogen.
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| **Seen in [[EWS]].
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| =See also= | | =See also= |