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Difference between revisions of "Liposarcoma"
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| Caption = Liposarcoma. [[H&E stain]]. | | Caption = Liposarcoma. [[H&E stain]]. | ||
| Synonyms = | | Synonyms = | ||
| Micro = | | Micro = lipoblasts - '''key feature''', chicken wire-like vascular, +/-[[myxoid]] background, cell size variation, thick fibrous septa (important low power feature) | ||
| Subtypes = | | Subtypes = [[dedifferentiated liposarcoma]], [[myxoid liposarcoma]] (includes round cell liposarcoma), mixed-type liposarcoma, pleomorphic liposarcoma, liposarcoma not otherwise specified (NOS), spindle cell liposarcoma | ||
| LMDDx = [[pleomorphic undifferentiated sarcoma]] | | LMDDx = [[pleomorphic undifferentiated sarcoma]] | ||
| Stains = | | Stains = | ||
| IHC = | | IHC = S-100 +ve | ||
| EM = | | EM = | ||
| Molecular = | | Molecular = t(12;16)(q13;p11) / TLS-CHOP -- for myxoid liposarcoma | ||
| IF = | | IF = | ||
| Gross = | | Gross = | ||
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| Prevalence = most common retroperitoneal sarcoma, uncommon overall | | Prevalence = most common retroperitoneal sarcoma, uncommon overall | ||
| Bloodwork = | | Bloodwork = | ||
| Rads = | | Rads = thick septae or nodules in a fatty appearing lesion | ||
| Endoscopy = | | Endoscopy = | ||
| Prognosis = poor | | Prognosis = poor | ||
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*Most common malignant sarcoma in the retroperitoneum. | *Most common malignant sarcoma in the retroperitoneum. | ||
*Not all (large) retroperitoneal adipocytic tumours are liposarcomas. | *Not all (large) retroperitoneal adipocytic tumours are liposarcomas. | ||
*Prognosis - dependent on the location. | |||
**Retroperitoneal: poor. | |||
**Extremity: good - disease specific 5-year survival ~ 92%.<ref>{{Cite journal | last1 = Lietman | first1 = SA. | last2 = Barsoum | first2 = WK. | last3 = Goldblum | first3 = JR. | last4 = Marks | first4 = KE. | last5 = Mascha | first5 = E. | last6 = Sundaram | first6 = M. | last7 = Muschler | first7 = G. | title = A 20-year retrospective review of surgically treated liposarcoma at the Cleveland Clinic. | journal = Orthopedics | volume = 30 | issue = 3 | pages = 227-34 | month = Mar | year = 2007 | doi = | PMID = 17375550 }}</ref> | |||
Notes: | Notes: | ||
*Retroperitoneal sarcomas: #1: liposarcoma, #2: [[pleomorphic undifferentiated sarcoma]], #3: leiomyosarcoma, #4: [[MPNST]]. | *Retroperitoneal sarcomas: #1: liposarcoma, #2: [[pleomorphic undifferentiated sarcoma]], #3: [[leiomyosarcoma]], #4: [[MPNST]]. | ||
**Extremely rare in retroperitoneum: [[synovial sarcoma]]. | **Extremely rare in retroperitoneum: [[synovial sarcoma]]. | ||
==Gross== | |||
*Thick fibrous septa | |||
Radiology:<ref name=pmid9108224/> | |||
*Fibrous septa or nodules.<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> | |||
*Myoid liposarcoma - high signal (due to high water content).<ref name=pmid16160117/> | |||
==Microscopic== | ==Microscopic== | ||
Features: | Features: | ||
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*+/-[[Myxoid]] background. | *+/-[[Myxoid]] background. | ||
*Cell size variation. | *Cell size variation. | ||
*Thick fibrous septa - important low power feature.<ref name=pmid9108224>{{Cite journal | last1 = Hosono | first1 = M. | last2 = Kobayashi | first2 = H. | last3 = Fujimoto | first3 = R. | last4 = Kotoura | first4 = Y. | last5 = Tsuboyama | first5 = T. | last6 = Matsusue | first6 = Y. | last7 = Nakamura | first7 = T. | last8 = Itoh | first8 = T. | last9 = Konishi | first9 = J. | title = Septum-like structures in lipoma and liposarcoma: MR imaging and pathologic correlation. | journal = Skeletal Radiol | volume = 26 | issue = 3 | pages = 150-4 | month = Mar | year = 1997 | doi = | PMID = 9108224 }}</ref> | |||
DDx: | DDx: | ||
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===Images=== | ===Images=== | ||
<gallery> | |||
Image: Dedifferentiated liposarcoma - intermed mag.jpg | Dediff. liposar. - shows dediff. component - intermed. mag. (WC) | |||
Image: Dedifferentiated liposarcoma - high mag.jpg | Dediff. liposarc. - high mag. (WC) | |||
Image: Dedifferentiated liposarcoma - very high mag.jpg | Dediff. liposarc. - very high mag. (WC) | |||
Image: Dedifferentiated liposarcoma - cropped - very high mag.jpg | Dediff. liposarc. - lipoblasts - very high mag. (WC) | |||
</gallery> | |||
<gallery> | <gallery> | ||
Image:Myxoid_liposarcoma_%2806%29.JPG | Myxoid liposarcoma. (WC) | Image:Myxoid_liposarcoma_%2806%29.JPG | Myxoid liposarcoma. (WC) | ||
Image:Myxoid_liposarcoma_%2805%29.JPG | Myxoid liposarcoma. (WC) | Image:Myxoid_liposarcoma_%2805%29.JPG | Myxoid liposarcoma. (WC) | ||
</gallery> | </gallery> | ||
www: | www: | ||
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==IHC== | ==IHC== | ||
* | *MDM2 +ve.<ref name=pmid24227706>{{Cite journal | last1 = Mariño-Enríquez | first1 = A. | last2 = Hornick | first2 = JL. | last3 = Dal Cin | first3 = P. | last4 = Cibas | first4 = ES. | last5 = Qian | first5 = X. | title = Dedifferentiated liposarcoma and pleomorphic liposarcoma: a comparative study of cytomorphology and MDM2/CDK4 expression on fine-needle aspiration. | journal = Cancer Cytopathol | volume = 122 | issue = 2 | pages = 128-37 | month = Feb | year = 2014 | doi = 10.1002/cncy.21362 | PMID = 24227706 }}</ref> | ||
*CDK4 +ve.<ref name=pmid24227706/> | |||
*S-100 +ve ~1/3 of the time. | *S-100 +ve ~1/3 of the time. | ||
==See also== | ==See also== | ||