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Difference between revisions of "Liposarcoma"
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# | '''Liposarcoma''' is a [[malignant]] [[Adipocytic tumours|adipocytic tumour]]. | ||
==General== | |||
*Most common malignant sarcoma in the retroperitoneum. | |||
*Not all (large) retroperitoneal adipocytic tumours are liposarcomas. | |||
Notes: | |||
*Retroperitoneal sarcomas: #1: liposarcoma, #2: [[pleomorphic undifferentiated sarcoma]], #3: leiomyosarcoma, #4: [[MPNST]]. | |||
**Extremely rare in retroperitoneum: [[synovial sarcoma]]. | |||
==Microscopic== | |||
Features: | |||
*Lipoblasts - '''key feature'''. | |||
**Large sharply demarcated vacuole. | |||
**Nucleus: | |||
***Hyperchromatic (dark staining) nucleus. | |||
***Eccentric location. | |||
***Nuclear indentation. | |||
*Chicken wire-like vascular. | |||
*+/-[[Myxoid]] background. | |||
*Cell size variation. | |||
DDx: | |||
*[[Angiolipoma]]. | |||
*[[Pleomorphic undifferentiated sarcoma]]. | |||
Images: | |||
*[http://commons.wikimedia.org/wiki/File:Myxoid_liposarcoma_%2806%29.JPG Myxoid liposarcoma (WC)]. | |||
*[http://commons.wikimedia.org/wiki/File:Myxoid_liposarcoma_%2805%29.JPG Myxoid liposarcoma (WC)]. | |||
*[http://www.john-libbey-eurotext.fr/e-docs/00/04/09/14/texte_alt_jleejd00046_gr5.jpg Lipoblasts (john-libbey-eurotext.fr)]. | |||
*[http://commons.wikimedia.org/wiki/File:Dedifferentiated_liposarcoma_-_cropped_-_very_high_mag.jpg Dediff. liposarcoma - lipoblasts - very high mag. (WC)]. | |||
*[http://commons.wikimedia.org/wiki/File:Dedifferentiated_liposarcoma_-_intermed_mag.jpg Dediff. liposarcoma - shows dediff. component - intermed. mag. (WC)]. | |||
*[http://path.upmc.edu/cases/case302.html Poorly diff. liposarcoma - several images (upmc.edu)]. | |||
===Subtypes=== | |||
Main subtypes:<ref name=Ref_WMSP_601>{{Ref WMSP|601}}</ref> | |||
*Dedifferentiated liposarcoma. | |||
*Myxoid liposarcoma. | |||
**Round cell liposarcoma - a subtype of myxoid liposarcoma<ref name=pmid8554106>{{Cite journal | last1 = Smith | first1 = TA. | last2 = Easley | first2 = KA. | last3 = Goldblum | first3 = JR. | title = Myxoid/round cell liposarcoma of the extremities. A clinicopathologic study of 29 cases with particular attention to extent of round cell liposarcoma. | journal = Am J Surg Pathol | volume = 20 | issue = 2 | pages = 171-80 | month = Feb | year = 1996 | doi = | PMID = 8554106 }} | |||
</ref> that has a worse prognosis;<ref name=pmid21253554>{{Cite journal | last1 = Conyers | first1 = R. | last2 = Young | first2 = S. | last3 = Thomas | first3 = DM. | title = Liposarcoma: molecular genetics and therapeutics. | journal = Sarcoma | volume = 2011 | issue = | pages = 483154 | month = | year = 2011 | doi = 10.1155/2011/483154 | PMID = 21253554 }}</ref> characterized by regions of high cellularity. | |||
*Mixed-type liposarcoma. | |||
*Pleomorphic liposarcoma. | |||
*Liposarcoma not otherwise specified (NOS). | |||
Additional reported type: | |||
*Spindle cell liposarcoma.<ref name=pmid8067512>{{Cite journal | last1 = Dei Tos | first1 = AP. | last2 = Mentzel | first2 = T. | last3 = Newman | first3 = PL. | last4 = Fletcher | first4 = CD. | title = Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases. | journal = Am J Surg Pathol | volume = 18 | issue = 9 | pages = 913-21 | month = Sep | year = 1994 | doi = | PMID = 8067512 }}</ref> | |||
====Myxoid liposarcoma==== | |||
*[[AKA]] ''myxoid/round cell liposarcoma''. | |||
*[[AKA]] ''round cell liposarcoma''. | |||
=====Gross===== | |||
Location:<ref name=pmid22052112>{{Cite journal | last1 = Moreau | first1 = LC. | last2 = Turcotte | first2 = R. | last3 = Ferguson | first3 = P. | last4 = Wunder | first4 = J. | last5 = Clarkson | first5 = P. | last6 = Masri | first6 = B. | last7 = Isler | first7 = M. | last8 = Dion | first8 = N. | last9 = Werier | first9 = J. | title = Myxoid\Round Cell Liposarcoma (MRCLS) Revisited: An Analysis of 418 Primarily Managed Cases. | journal = Ann Surg Oncol | volume = 19 | issue = 4 | pages = 1081-1088 | month = Apr | year = 2012 | doi = 10.1245/s10434-011-2127-z | PMID = 22052112 }}</ref> | |||
*90% in lower limb. | |||
*81% deep. | |||
=====Microscopic===== | |||
Features: | |||
*Chickenwire-type blood vessels. | |||
*Clear spaces. | |||
*Adipocytes - may be rare. | |||
DDx: | |||
*[[Low-grade fibromyxoid sarcoma]]. | |||
Images: | |||
*[http://pubs.rsna.org/doi/full/10.1148/radiographics.20.4.g00jl021007 Myxoid liposarcoma (rsna.org)].<ref name=pmid10903690>{{Cite journal | last1 = Sung | first1 = MS. | last2 = Kang | first2 = HS. | last3 = Suh | first3 = JS. | last4 = Lee | first4 = JH. | last5 = Park | first5 = JM. | last6 = Kim | first6 = JY. | last7 = Lee | first7 = HG. | title = Myxoid liposarcoma: appearance at MR imaging with histologic correlation. | journal = Radiographics | volume = 20 | issue = 4 | pages = 1007-19 | month = | year = | doi = 10.1148/radiographics.20.4.g00jl021007 | PMID = 10903690 }}</ref> | |||
*[http://pubs.rsna.org/doi/figure/10.1148/rg.255055106 Myxoid liposarcoma - among other things (rsna.org)].<ref name=pmid16160117>{{Cite journal | last1 = Murphey | first1 = MD. | last2 = Arcara | first2 = LK. | last3 = Fanburg-Smith | first3 = J. | title = From the archives of the AFIP: imaging of musculoskeletal liposarcoma with radiologic-pathologic correlation. | journal = Radiographics | volume = 25 | issue = 5 | pages = 1371-95 | month = | year = | doi = 10.1148/rg.255055106 | PMID = 16160117 }}</ref> | |||
*[http://www.cap.org/apps/cap.portal?_nfpb=true&cntvwrPtlt_actionOverride=%2Fportlets%2FcontentViewer%2Fshow&_windowLabel=cntvwrPtlt&cntvwrPtlt{actionForm.contentReference}=cap_foundation%2FcaseOfMonth%2Ffeb13%2Ffeb_2013_cotm_diagnosis.html&_state=maximized&_pageLabel=cntvwr Myxoid liposarcoma (cap.org)]. | |||
=====Molecular===== | |||
Typically has a [[translocations|translocation]]: | |||
*t(12;16)(q13;p11) TLS-CHOP.<ref name=pmid7805034>{{Cite journal | last1 = Knight | first1 = JC. | last2 = Renwick | first2 = PJ. | last3 = Dal Cin | first3 = P. | last4 = Van den Berghe | first4 = H. | last5 = Fletcher | first5 = CD. | title = Translocation t(12;16)(q13;p11) in myxoid liposarcoma and round cell liposarcoma: molecular and cytogenetic analysis. | journal = Cancer Res | volume = 55 | issue = 1 | pages = 24-7 | month = Jan | year = 1995 | doi = | PMID = 7805034 }}</ref> | |||
*May have the translocation usually seen in [[clear cell sarcoma]]: | |||
*t(12;22)(q13;q12) EWS-ATF1.<ref name=pmid21115923>{{Cite journal | last1 = Suzuki | first1 = K. | last2 = Matsui | first2 = Y. | last3 = Endo | first3 = K. | last4 = Kubo | first4 = T. | last5 = Hasegawa | first5 = T. | last6 = Kimura | first6 = T. | last7 = Ohtani | first7 = O. | last8 = Yasui | first8 = N. | title = Myxoid liposarcoma with EWS-CHOP type 1 fusion gene. | journal = Anticancer Res | volume = 30 | issue = 11 | pages = 4679-83 | month = Nov | year = 2010 | doi = | PMID = 21115923 }}</ref> | |||
====Dedifferentiated liposarcoma==== | |||
*Has an undifferentiated component that, if seen alone, would be diagnosed as [[pleomorphic undifferentiated sarcoma]]. | |||
*The diagnosis depends on the presence of the differentiated component of the tumour, i.e. the presence of lipoblasts. | |||
==IHC== | |||
*IHC is of limited value. | |||
*S-100 +ve ~1/3 of the time. | |||
*Reticulin ???. | |||
==See also== | |||
*[[Adipocytic tumours]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Adipocytic tumours]] | |||