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Difference between revisions of "Chondro-osseous tumours"
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→Ewing sarcoma: more
(→Osteosarcoma: +alt. name) |
(→Ewing sarcoma: more) |
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*Second most common malignant bone tumour in children. | *Second most common malignant bone tumour in children. | ||
**Most common malignant bone tumour = osteosarcoma (AKA osteogenic sarcoma). | **Most common malignant bone tumour = osteosarcoma (AKA osteogenic sarcoma). | ||
Poor prognostic factors:<ref>PST. 14 February 2011.</ref> | |||
*Age (18 years-old+). | |||
*Pelvis (extremity = good). | |||
*>8 cm. | |||
*Metastases. | |||
*EWS-FL1 fusion type 2. | |||
====Radiology==== | ====Radiology==== | ||
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===Microscopic=== | ===Microscopic=== | ||
Features:<ref>PST. 22 February 2010.</ref> | Features:<ref>PST. 22 February 2010.</ref> | ||
*Scant clear cytoplasm (contain glycogen - PAS +ve, PAS-D -ve). | *Scant clear cytoplasm (contain glycogen -- PAS +ve, PAS-D -ve). | ||
* | *Bland round small nucleus. | ||
* | **Usu. lack nucleoli. | ||
**Usu. minimal-moderate size variation. | |||
Notes: | Notes: | ||
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===[[IHC]]=== | ===[[IHC]]=== | ||
Features:<ref name=Ref_WMSP651>{{Ref WMSP|651}}</ref> | Features:<ref name=Ref_WMSP651>{{Ref WMSP|651}}</ref> | ||
*CD99 +ve | *CD99 +ve -- 1. diffuse, 2. plasma membrane staining; both required -- most specific. | ||
*CD45 -ve. | *CD45 -ve. | ||
**Done to r/o [[lymphoma]]. | **Done to r/o [[lymphoma]]. | ||
*+/-Neural markers (NSE, synaptophysin, CD57 (??? CD56 ???), S100). | *+/-Neural markers (NSE, synaptophysin, CD57 (??? CD56 ???), S100). | ||
*+/-Cytokeratins. | *+/-Cytokeratins. | ||
*Caveolin-1.<ref>PST. | *Caveolin-1 +ve in ~ 85% of EWS.<ref>PST. 14 February 2011.</ref> | ||
Notes:<ref>PST. 22 February 2010.</ref> | Notes:<ref>PST. 22 February 2010.</ref> | ||
*CD99 +ve (plasma membrane) tumours: | *CD99 +ve (plasma membrane) tumours: | ||
**Lymphoblastic lymphoma/leukemia. | **[[Lymphoblastic lymphoma]]/leukemia. | ||
**Angiomatoid fibrous histiocytoma. | **Angiomatoid fibrous histiocytoma. | ||
**Desmoplastic small round cell tumour. | **[[Desmoplastic small round cell tumour]]. | ||
===Molecular diagnostics=== | ===Molecular diagnostics=== | ||
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*''EWS/FLI-1 fusion gene'' formation due to translocation: ''t(11;22)(q24;q12)''.<ref>URL: [http://atlasgeneticsoncology.org/Tumors/Ewing5010.html http://atlasgeneticsoncology.org/Tumors/Ewing5010.html]. Accessed on: 23 February 2010.</ref><ref name=pmid3163261>{{cite journal |author=Turc-Carel C, Aurias A, Mugneret F, ''et al.'' |title=Chromosomes in Ewing's sarcoma. I. An evaluation of 85 cases of remarkable consistency of t(11;22)(q24;q12) |journal=Cancer Genet. Cytogenet. |volume=32 |issue=2 |pages=229–38 |year=1988 |month=June |pmid=3163261 |doi= |url=}}</ref> | *''EWS/FLI-1 fusion gene'' formation due to translocation: ''t(11;22)(q24;q12)''.<ref>URL: [http://atlasgeneticsoncology.org/Tumors/Ewing5010.html http://atlasgeneticsoncology.org/Tumors/Ewing5010.html]. Accessed on: 23 February 2010.</ref><ref name=pmid3163261>{{cite journal |author=Turc-Carel C, Aurias A, Mugneret F, ''et al.'' |title=Chromosomes in Ewing's sarcoma. I. An evaluation of 85 cases of remarkable consistency of t(11;22)(q24;q12) |journal=Cancer Genet. Cytogenet. |volume=32 |issue=2 |pages=229–38 |year=1988 |month=June |pmid=3163261 |doi= |url=}}</ref> | ||
**Often detected by RT-PCR (with EWS 5' and FLI-1 3' primers). | **Often detected by RT-PCR (with EWS 5' and FLI-1 3' primers). | ||
**Type 1 = EWS exon 7 + FLI-1 exon 6; good prognosis. | |||
**Type 2 = others; poor prognosis. | |||
Notes: | Notes: | ||
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**Neuroblastoma (possibly). | **Neuroblastoma (possibly). | ||
*Several other translocations exist. | *Several other translocations exist. | ||
*Lack of molecular findings does ''not'' exclude Ewing sarcoma. | |||
==Osteosarcoma== | ==Osteosarcoma== | ||