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Small round cell tumours (view source)

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[[Image:Ewing sarcoma - very high mag.jpg|thumb|right|[[Micrograph]] showing a small round cell tumour ([[Ewing sarcoma]]) in the lung. [[H&E stain]].]]
'''Small round cell tumours''' (SRCT), also '''small round blue cell tumours''' (SRBCT), are a group of tumours that have a similar histologic appearance.   
'''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.
==General==
*Group of tumours that is typically seen in childhood and younger adults.
*Most common small round cell tumour is non-Hodgkin lymphoma.<ref name=pmid23998069/>


DDx:<ref>URL: [http://www.thedoctorsdoctor.com/diseases/small_round_blue_cell_tumor.htm http://www.thedoctorsdoctor.com/diseases/small_round_blue_cell_tumor.htm]. Accessed on: 2 July 2010.</ref>
===A short differential diagnosis of small round cell tumours===
A short DDx of SRCTs:<ref>URL: [http://www.thedoctorsdoctor.com/diseases/small_round_blue_cell_tumor.htm http://www.thedoctorsdoctor.com/diseases/small_round_blue_cell_tumor.htm]. Accessed on: 2 July 2010.</ref><ref>{{cite journal |author=Chen QR, Vansant G, Oades K, ''et al.'' |title=Diagnosis of the small round blue cell tumors using multiplex polymerase chain reaction |journal=J Mol Diagn |volume=9 |issue=1 |pages=80–8 |year=2007 |month=February |pmid=17251339 |pmc=1867426 |doi=10.2353/jmoldx.2007.060111 |url=}}</ref>
*[[Neuroblastoma]].
*[[Neuroblastoma]].
*Wilm's tumour ([[AKA]] nephroblastoma).
*[[Wilms tumour]] ([[AKA]] nephroblastoma).
*Alveolar rhabdomyosarcoma.
*[[Alveolar rhabdomyosarcoma]].
*[[Ewing sarcoma]]/PNET - this entity is dealt with in the ''[[bone tumours]]'' article.
*[[Ewing sarcoma]] / primative neuroectodermal tumour (PNET).
*[[Lymphoma]] - large cell lymphomas, e.g. [[diffuse large B cell lymphoma]], anaplastic large cell lymphoma.
*[[Lymphoma]] - non-Hogkin's lymphoma, usu. large cell lymphomas (e.g. [[diffuse large B cell lymphoma]], anaplastic large cell lymphoma), Burkitt's lymphoma.
*[[Retinoblastoma]].
*[[Retinoblastoma]].
*[[Liver neoplasms|Hepatoblastoma]].
*[[Liver neoplasms|Hepatoblastoma]].
*Desmoplastic small round cell tumour (DSRCT).
*[[Desmoplastic small round cell tumour]] (DSRCT).
*[[Small cell carcinoma]].


Memory device: 4 -blastoma (hepato-, neuro-, nephro-, retino-), PNET/Ewing sarcoma (medulloblastoma), DSRCT, lymphoma, alveolar rhabdomyosarcoma.
Others:
*[[Small cell osteosarcoma]].
*[[Synovial sarcoma]].
 
Memory device: 4 -blastomas (hepato-, neuro-, nephro-, retino-), PNET/Ewing sarcoma (medulloblastoma), LADSSS (lymphoma, alveolar rhabdomyosarcoma, DSRCT, small cell carcinoma, small cell osteosarcoma, synovial sarcoma).
 
===A long differential diagnosis of small blue cell tumours===
Adapted from ''Miller'' with modifications:<ref name=miller>Miller RT. (Sep 2013). ''Atlantic Diagnostic Immunohistochemistry Symposium''. St. John's, NL, Canada.</ref>
{| class="wikitable sortable"
! Tumour
! Group
! Notes/comments
|-
| [[Malignant melanoma]], small cell
| melanoma
| 
|-
| [[PEComa]]
| mesenchymal lesion
| 
|-
| Carcinoma not otherwise specified
| carcinoma
| 
|-
| [[Small cell carcinoma]]
| carcinoma
| 
|-
| [[Merkel cell carcinoma]]
| carcinoma
| 
|-
| [[Islet cell tumour]] ([[carcinoid]])
| carcinoma
| 
|-
| [[Medullary thyroid carcinoma]]
| carcinoma
| 
|-
| [[Desmoplastic small round cell tumour]]
| sarcoma
| 
|-
| [[Polyphenotypic small cell tumour]]
| other
| 
|-
| [[Papillary-cystic tumour of pancreas]]
| other
| 
|-
| [[Pancreatoblastoma]]
| other
| 
|-
| [[Acinar cell carcinoma]]
| carcinoma
| 
|-
| [[Adenoid cystic carcinoma]]
| carcinoma
| 
|-
| Cutaneous adnexal tumours ([[spiradenoma]], [[acrospiroma]])
| other
| 
|-
| [[Wilms tumour]]
| other
| 
|-
| [[Thymoma]]
| epithelial lesion
| 
|-
| [[Metanephric adenoma|Metanephric adenoma of urinary tract]]
| epithelial lesion
| 
|-
| Basaloid carcinoma / [[Basal cell carcinoma]]
| carcinoma
| 
|-
| [[Small cell mesothelioma]]
| carcinoma
| <ref name=pmid1310669>{{Cite journal  | last1 = Mayall | first1 = FG. | last2 = Gibbs | first2 = AR. | title = The histology and immunohistochemistry of small cell mesothelioma. | journal = Histopathology | volume = 20 | issue = 1 | pages = 47-51 | month = Jan | year = 1992 | doi =  | PMID = 1310669 }}</ref>
|-
| [[Pulmonary blastoma]]
| other
| 
|-
| [[Cutaneous myoepithelioma]]
| epithelial lesion
|<ref>{{Cite journal | last1 = Kutzner| first1 = H.| last2 = Mentzel| first2 = T.| last3 = Kaddu| first3 = S.| last4 = Soares| first4 = LM.| last5 = Sangueza| first5 = OP.| last6 = Requena| first6 = L.| title = Cutaneous myoepithelioma: an under-recognized cutaneous neoplasm composed of myoepithelial cells.| journal = Am J Surg Pathol| volume = 25| issue = 3| pages = 348-55| month = Mar| year = 2001| doi = | PMID = 11224605 }}</ref>
|-
| [[Ewing sarcoma]] / [[PNET]]
| neural tumours
| 
|-
| [[Neuroblastoma]] / [[esthesioneuroblastoma]]
| neural tumours
| 
|-
| [[Sclerosing perineurioma]]
| neural tumours
| 
|-
| [[Schwannoma]]
| neural tumours
| 
|-
| [[Meningioma]]
| neural tumours
| 
|-
| [[Medulloblastoma]]
| neural tumours
| 
|-
| [[Central neurocytoma]]
| neural tumours
| 
|-
| [[Medulloepithelioma]]
| neural tumours
| 
|-
| [[Retinoblastoma]]
| neural tumours
| 
|-
| [[Oligodendroglioma]]
| neural tumours
| 
|-
| [[Pineocytoma]]
| neural tumours
| 
|-
| [[Hemangioblastoma]]
| neural tumours
| may be seen extraneural
|-
| [[Neurogenic sarcoma]]
| neural tumours
| 
|-
| [[Lymphoma]]
| lymphoid
| 
|-
| [[Extramedullary leukemia]]
| lymphoid
| 
|-
| [[Plasma cell myeloma]]
| lymphoid
| 
|-
| [[Mastocytosis]]
| lymphoid
| 
|-
| [[Plasmacytoid dendritic cell tumour]]
| lymphoid
|CD4 +ve, CD56 +ve<ref name=pmid16160468>{{Cite journal | last1 = Reichard| first1 = KK.| last2 = Burks| first2 = EJ.| last3 = Foucar| first3 = MK.| last4 = Wilson| first4 = CS.| last5 = Viswanatha| first5 = DS.| last6 = Hozier| first6 = JC.| last7 = Larson| first7 = RS.| title = CD4(+) CD56(+) lineage-negative malignancies are rare tumors of plasmacytoid dendritic cells.| journal = Am J Surg Pathol| volume = 29| issue = 10| pages = 1274-83| month = Oct| year = 2005| doi = | PMID = 16160468 }}</ref>
|-
| [[Granuloma annulare]]
| histiocytic lesion
| 
|-
| [[Seminoma]]
| germ cell tumour
| degenerative cases
|-
| [[Granulosa cell tumour]]
| sex cord tumour
| 
|-
| [[Gynandroblastoma]]
| sex cord tumour
| Sertoli cell & granulosa cell
|-
| [[Sertoli-Leydig tumour]]
| sex cord tumour
| 
|-
| [[Endometrial stromal sarcoma]]
| sarcoma
| may be extragenital
|-
| [[Adenosarcoma]]
| sarcoma
| glandular component may not be sampled
|-
| [[Rhabdomyosarcoma]]
| muscle
| 
|-
| Skeletal muscle regeneration
| muscle
| mimics [[rhabdomyosarcoma]]<ref name=pmid9762546>{{Cite journal | last1 = Guillou| first1 = L.| last2 = Coquet| first2 = M.| last3 = Chaubert| first3 = P.| last4 = Coindre| first4 = JM.| title = Skeletal muscle regeneration mimicking rhabdomyosarcoma: a potential diagnostic pitfall.| journal = Histopathology| volume = 33| issue = 2| pages = 136-44| month = Aug| year = 1998| doi = | PMID = 9762546 }}</ref>
|-
| [[Small cell leiomyosarcoma]]
| sarcoma
| 
|-
| [[Small cell osteosarcoma]]
| chondro-osseous tumours
| 
|-
| [[Mesenchymal chondrosarcoma]]
| chondro-osseous tumours
| 
|-
| [[Myxoid chondrosarcoma]]
| chondro-osseous tumours
| 
|-
| [[Proliferative myositis]]
| muscle
| 
|-
| [[Fetal or genital rhabdomyoma]]
| other
| 
|-
| [[Glomus tumour]]
| other
| 
|-
| [[Glomangiopericytoma]]
| other
| 
|-
| [[Synovial sarcoma]]
| sarcoma
| 
|-
| [[Hemangiopericytoma]]
| soft tissue lesions
| 
|-
| [[angiosarcoma|Small cell angiosarcoma]]
| vascular lesions
| 
|-
| [[Epithelioid hemangioendothelioma]]
| vascular lesions
| 
|-
| [[Solitary fibrous tumour]]
| soft tissue lesions
| can be myxoid
|-
| [[Myofibroblastoma]]
| soft tissue lesions
| 
|-
| [[Angiomyofibroblastoma]]
| soft tissue lesions
| 
|-
|}
Notes:
#This [[DDx]] includes small cells that may be spindled.
#The DDx for large (non-pleomorphic) epithelioid cells is found in ''[[large epithelioid tumours]]''.


==Microscopic==
==Microscopic==
Features:
Features:
*Small cells ~ 2X [[RBC]] diameter - '''key feature'''.
*Scant cytoplasm/high [[NC ratio]].
*Sheets of cells, very cellular.
*Sheets of cells, very cellular.
*Small cells ~ 2X RBC diameter.
*Scant cytoplasm.
*Coarse chromatin.
*Coarse chromatin.
*+/-Nucleolus. (???)
*+/-Nucleolus; usu. not prominent.
*+/-Vascular.
*+/-Vascularity.
 
==Tabular comparison==
Adapted from Thorner:<ref>PST. 14 February 2011.</ref>
{| class="wikitable sortable"
! '''Tumour'''
! '''Key histologic features'''
! '''History'''
! '''Key [[IHC]] / <br>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+, [[S-100]]-
|-
| [[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<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+
|-
| [[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 lesion; +/-RB1 gene mutations (familial)
|
|-
| [[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]]; '''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]]<sup>§</sup>
| '''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 A, CD56, keratins
|-
| [[Lymphoma]], non-Hogkin's, usu. large cell (e.g. [[DLBCL]])
| '''dyscohesive cells''', usu. ~2x normal (resting) lymphocyte, usu. nucleolus
| usu. lymphadenopathy
| CD45, [[CD20]], TdT, [[CD3]]
|-
|}
Notes:
*<sup>§</sup> Uncommonly has the small round cell tumour morphology - included for comparison to ''[[alveolar rhabdomyosarcoma]]''.
 
==IHC panel==
Gino's panel:<ref>G. Somers. 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
|}
 
More general panel:<ref name=pmid23998069>{{Cite journal  | last1 = D'cruze | first1 = L. | last2 = Dutta | first2 = R. | last3 = Rao | first3 = S. | last4 = R | first4 = A. | last5 = Varadarajan | first5 = S. | last6 = Kuruvilla | first6 = S. | title = The role of immunohistochemistry in the analysis of the spectrum of small round cell tumours at a tertiary care centre. | journal = J Clin Diagn Res | volume = 7 | issue = 7 | pages = 1377-82 | month = Jul | year = 2013 | doi = 10.7860/JCDR/2013/5127.3132 | PMID = 23998069 }}</ref>
*CD45, CD20, CD3, CD99, desmin, EMA, pankeratin, synaptophysin, chromogranin, GFAP.


==See also==
==See also==
*[[Soft tissue lesions]].
*[[Soft tissue lesions]].
*[[Basics]].
*[[Large epithelioid tumours]].
*[[Pleomorphic tumours]].


==References==
==References==
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[[Category:Weird stuff]]
[[Category:Weird stuff]]
[[Category:Pediatric pathology]]
Michael
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