Difference between revisions of "Small round cell tumours"

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==Tabular comparison==
==Tabular comparison==
Adapted from Thorner:<ref>PST. 14 February 2011.</ref>
{| class="wikitable"
{| class="wikitable"
| '''Tumour'''
| '''Tumour'''
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|-
|-
| Hepatoblastoma
| Hepatoblastoma
|
| fetal hepatocytes (~ 1:3 NC ratio, eosinophilic cytoplasm)
| liver
| liver
| AFP
| AFP
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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
| kidney lesion, various syndromes
| WT-1+
| WT-1+
|-
|-
| Retinoblastoma
| Retinoblastoma
|
| Flexner-Wintersteiner rosette (rosette with empty centre (donut hole))<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>
| eye
| eye lesion; +/-RB1 gene mutations (familial)
|
|
|-
|-
| 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>
|  
| +/-nevoid basal cell carcinoma syndrome
|
|
|-
|-

Revision as of 16:29, 28 February 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]

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

Microscopic

Features:

  • Sheets of cells, very cellular.
  • Small cells ~ 2X RBC diameter.
  • Scant cytoplasm.
  • Coarse chromatin.
  • +/-Nucleolus. (???)
  • +/-Vascular.

Tabular comparison

Adapted from Thorner:[2]

Tumour Key histologic features History Key IHC /
special stains
Hepatoblastoma fetal hepatocytes (~ 1:3 NC ratio, eosinophilic cytoplasm) liver AFP
Neuroblastoma nests, thin fibrovascular septae, lymphocytes, ganglion-like cells (prominent nucleolus), neurophil (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 WT-1+
Retinoblastoma Flexner-Wintersteiner rosette (rosette with empty centre (donut hole))[3] eye lesion; +/-RB1 gene mutations (familial)
Medulloblastoma Homer-Wright rosettes (rosette with a meshwork of fibers (neuropil) at the centre)[3] +/-nevoid basal cell carcinoma syndrome
Ewing sarcoma/PNET clear cytoplasm (glycogen) bone lesion, adolescents PAS+/PASD-, CD99 diffuse membranous
Rhabdomyosarcoma, alveolar rhabdomyocytes (eosinophilic cytoplasm +/-cross striations (uncommon), eccentric nucleus) adolescents, young adults; often mets at presentation "DAM" = desmin, actin, myogenin
Rhabdomyosarcoma, embryonal rhabdomyocytes (elongated/cigar-shaped cells, eosinophilic cytoplasm +/-cross striations, eccentric nucleus), nests with rounded border <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 stippled chromatin, nuclear moulding adults, smokers synaptophysin, chromogranin, CD56, keratins
Lymphoma (large cell) dyscohesive cells, ~2x normal (resting) lymphocyte, nucleolus lymphadenopathy (???) CD45

See also

References

  1. URL: http://www.thedoctorsdoctor.com/diseases/small_round_blue_cell_tumor.htm. Accessed on: 2 July 2010.
  2. PST. 14 February 2011.
  3. 3.0 3.1 Wippold FJ, Perry A (March 2006). "Neuropathology for the neuroradiologist: rosettes and pseudorosettes". AJNR Am J Neuroradiol 27 (3): 488–92. PMID 16551982.