Difference between revisions of "Small round cell tumours"

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(IHC table)
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| [[Nephroblastoma]] (Wilms tumour)
| [[Nephroblastoma]] (Wilms tumour)
| '''triphasic''' - (1) blue cells (blastema), (2) spindle cells (stroma), (3) tubular structures (epithelial)
| '''triphasic''' - (1) blue cells (blastema), (2) spindle cells (stroma), (3) tubular structures (epithelial)
| kidney lesion, various syndromes
| kidney lesion, various syndromes (e.g. Beckwith-Wiedemann syndrome<ref>URL: [http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002168/ http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002168/]. Accessed on: 4 April 2011.</ref>)
| WT-1+
| WT-1+
|-
|-
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| [[Medulloblastoma]]
| [[Medulloblastoma]]
| '''Homer-Wright rosettes''' ([[rosette]] with a 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>
| '''Homer-Wright rosettes''' ([[rosette]] with a 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>
| +/-[[nevoid basal cell carcinoma syndrome]]
| +/-[[nevoid basal cell carcinoma syndrome]]; '''must''' arise in cerebellum
|
|
|-
|-
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|-
|-
| [[Alveolar rhabdomyosarcoma]]
| [[Alveolar rhabdomyosarcoma]]
| '''alveolar-like spaces''' (small blue cells line spaces supported by fibrous tissue), rhabdomyocytes (eosinophilic cytoplasm +/-cross striations (uncommon), eccentric nucleus)
| '''alveolar-like spaces''' (small blue cells line spaces supported by fibrous tissue), rhabdomyoblasts (eosinophilic cytoplasm, +/-cross striations (uncommon), eccentric nucleus, +/-elongated/cigar-shaped cells (uncommon))
| adolescents, young adults; often mets at presentation
| adolescents, young adults; often mets at presentation
| "DAM" = desmin, actin, myogenin
| "DAM" = desmin, actin, myogenin
|-
|-
| [[Embryonal rhabdomyosarcoma]]<sup>§</sup>
| [[Embryonal rhabdomyosarcoma]]<sup>§</sup>
| '''nests with rounded border''', rhabdomyocytes (+/-elongated/cigar-shaped cells, eosinophilic cytoplasm +/-cross striations, eccentric nucleus)  
| '''nests with rounded border''', rhabdomyoblasts (+/-elongated/cigar-shaped cells, eosinophilic cytoplasm, +/-cross striations, eccentric nucleus)  
| <10 years old; usu. localized
| <10 years old; usu. localized
| "DAM" = desmin, actin, myogenin
| "DAM" = desmin, actin, myogenin
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Notes:
Notes:
*<sup>§</sup> Uncommonly has the small round cell tumour morphology - included for comparison to ''[[alveolar rhabdomyosarcoma]]''.
*<sup>§</sup> Uncommonly has the small round cell tumour morphology - included for comparison to ''[[alveolar rhabdomyosarcoma]]''.
==IHC panel==
Gino's panel:<ref>GS. 22 March 2011.</ref>
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
! Lesion
! NB84
! Myogenin
! Desmin
! CD99
! CD45
|-
| Lymphoma
| -ve
| -ve
| -ve
| +ve/-ve
| +
|-
| Ewing sarcoma
| +ve/-ve
| -ve
| -ve
| +ve (membranous)
| -
|-
| Neuroblastoma
| +ve
| -ve
| -ve
| +ve/-ve
| -ve
|-
| Rhabdomyosarcoma
| -ve
| +ve
| +ve
| -ve
| -ve
|}


==See also==
==See also==

Revision as of 03:21, 5 April 2011

Small round cell tumours (SRCT), also small round blue cell tumours (SRBCT), are a group of tumours that have a similar histologic appearance.

It is a group of tumours that is seen more often in childhood than adulthood.

DDx:[1][2]

Memory device: 4 -blastoma (hepato-, neuro-, nephro-, retino-), PNET/Ewing sarcoma (medulloblastoma), DSRCT, lymphoma, alveolar rhabdomyosarcoma.

Microscopic

Features:

  • Small cells ~ 2X RBC diameter - key feature.
  • Scant cytoplasm/high NC ratio.
  • Sheets of cells, very cellular.
  • Coarse chromatin.
  • +/-Nucleolus; usu. not prominent.
  • +/-Vascularity.

Tabular comparison

Adapted from Thorner:[3]

Tumour Key histologic features History Key IHC /
special stains
Hepatoblastoma fetal hepatocytes (~ 1:3 NC ratio, eosinophilic cytoplasm) liver lesion, usu. < 3 years old AFP
Neuroblastoma nests, thin fibrovascular septae, lymphocytes, ganglion-like cells (prominent nucleolus), neuropil (eosinophilic, fluffy, finely vacuolated) adrenal gland, sympathetic chain; usu. < 3 years old NB-84+, NSE+, S100-
Nephroblastoma (Wilms tumour) triphasic - (1) blue cells (blastema), (2) spindle cells (stroma), (3) tubular structures (epithelial) kidney lesion, various syndromes (e.g. Beckwith-Wiedemann syndrome[4]) WT-1+
Retinoblastoma Flexner-Wintersteiner rosette (rosette with empty centre (donut hole))[5] eye lesion; +/-RB1 gene mutations (familial)
Medulloblastoma Homer-Wright rosettes (rosette with a meshwork of fibers (neuropil) at the centre)[5] +/-nevoid basal cell carcinoma syndrome; must arise in cerebellum
Ewing sarcoma/PNET clear cytoplasm (glycogen) bone lesion, adolescents PAS+/PASD-, CD99 diffuse membranous
Alveolar rhabdomyosarcoma alveolar-like spaces (small blue cells line spaces supported by fibrous tissue), rhabdomyoblasts (eosinophilic cytoplasm, +/-cross striations (uncommon), eccentric nucleus, +/-elongated/cigar-shaped cells (uncommon)) adolescents, young adults; often mets at presentation "DAM" = desmin, actin, myogenin
Embryonal rhabdomyosarcoma§ nests with rounded border, rhabdomyoblasts (+/-elongated/cigar-shaped cells, eosinophilic cytoplasm, +/-cross striations, eccentric nucleus) <10 years old; usu. localized "DAM" = desmin, actin, myogenin
Desmoplastic small round cell tumour (DSRCT) nests with "jigsaw" border peritoneum (abdomen or plevis); male > female; locally invasive keratin+, EMA+, desmin+ !!!
Small cell carcinoma nuclear moulding, stippled chromatin adults, smokers synaptophysin, chromogranin, CD56, keratins
Lymphoma, non-Hogkin's, usu. large cell (e.g. DLBCL) dyscohesive cells, usu. ~2x normal (resting) lymphocyte, usu. nucleolus lymphadenopathy (???) CD45

Notes:

IHC panel

Gino's panel:[6]

Lesion NB84 Myogenin Desmin CD99 CD45
Lymphoma -ve -ve -ve +ve/-ve +
Ewing sarcoma +ve/-ve -ve -ve +ve (membranous) -
Neuroblastoma +ve -ve -ve +ve/-ve -ve
Rhabdomyosarcoma -ve +ve +ve -ve -ve

See also

References

  1. URL: http://www.thedoctorsdoctor.com/diseases/small_round_blue_cell_tumor.htm. Accessed on: 2 July 2010.
  2. Chen QR, Vansant G, Oades K, et al. (February 2007). "Diagnosis of the small round blue cell tumors using multiplex polymerase chain reaction". J Mol Diagn 9 (1): 80–8. doi:10.2353/jmoldx.2007.060111. PMC 1867426. PMID 17251339. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1867426/.
  3. PST. 14 February 2011.
  4. URL: http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002168/. Accessed on: 4 April 2011.
  5. 5.0 5.1 Wippold FJ, Perry A (March 2006). "Neuropathology for the neuroradiologist: rosettes and pseudorosettes". AJNR Am J Neuroradiol 27 (3): 488–92. PMID 16551982.
  6. GS. 22 March 2011.