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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. | ||
==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><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> | ===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]]. | ||
*[[Wilms tumour]] ([[AKA]] nephroblastoma). | *[[Wilms tumour]] ([[AKA]] nephroblastoma). | ||
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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). | 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== | ||
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==Tabular comparison== | ==Tabular comparison== | ||
Adapted from Thorner:<ref>PST. 14 February 2011.</ref> | Adapted from Thorner:<ref>PST. 14 February 2011.</ref> | ||
{| class="wikitable" | {| class="wikitable sortable" | ||
! '''Tumour''' | |||
! '''Key histologic features''' | |||
! '''History''' | |||
! '''Key [[IHC]] / <br>special stains''' | |||
|- | |- | ||
| [[Hepatoblastoma]] | | [[Hepatoblastoma]] | ||
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| nests, thin fibrovascular septae, lymphocytes, ganglion-like cells (prominent nucleolus), neuropil (eosinophilic, fluffy, finely vacuolated) | | nests, thin fibrovascular septae, lymphocytes, ganglion-like cells (prominent nucleolus), neuropil (eosinophilic, fluffy, finely vacuolated) | ||
| adrenal gland, sympathetic chain; usu. < 3 years old | | adrenal gland, sympathetic chain; usu. < 3 years old | ||
| NB-84+, NSE+, | | NB-84+, NSE+, [[S-100]]- | ||
|- | |- | ||
| [[Nephroblastoma]] (Wilms tumour) | | [[Nephroblastoma]] (Wilms tumour) | ||
Line 80: | Line 348: | ||
| '''nests with "jigsaw" border''' | | '''nests with "jigsaw" border''' | ||
| peritoneum (abdomen or plevis); male > female; locally invasive | | peritoneum (abdomen or plevis); male > female; locally invasive | ||
| keratin+, EMA+, desmin+ !!! | | [[keratin]]+, [[EMA]]+, desmin+ !!! | ||
|- | |- | ||
| Small cell carcinoma | | [[Small cell carcinoma]] | ||
| '''nuclear moulding''', stippled chromatin | | '''nuclear moulding''', stippled chromatin | ||
| adults, smokers | | adults, smokers | ||
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| '''dyscohesive cells''', usu. ~2x normal (resting) lymphocyte, usu. nucleolus | | '''dyscohesive cells''', usu. ~2x normal (resting) lymphocyte, usu. nucleolus | ||
| usu. lymphadenopathy | | usu. lymphadenopathy | ||
| CD45, CD20, TdT, CD3 | | CD45, [[CD20]], TdT, [[CD3]] | ||
|- | |- | ||
|} | |} | ||
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==IHC panel== | ==IHC panel== | ||
Gino's panel:<ref> | Gino's panel:<ref>G. Somers. 22 March 2011.</ref> | ||
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto" | {| class="wikitable sortable" style="margin-left:auto;margin-right:auto" | ||
! Lesion | ! Lesion | ||
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| -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]] |
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