Difference between revisions of "Liposarcoma"

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#redirect [[Adipocytic_tumours#Liposarcoma]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Dedifferentiated_liposarcoma_-_cropped_-_very_high_mag.jpg
| Width      =
| Caption    = Liposarcoma. [[H&E stain]].
| Synonyms  =
| Micro      = lipoblasts - '''key feature''', chicken wire-like vascular, +/-[[myxoid]] background, cell size variation, thick fibrous septa (important low power feature)
| 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]]
| Stains    =
| IHC        = S-100 +ve
| EM        =
| Molecular  = t(12;16)(q13;p11) / TLS-CHOP -- for myxoid liposarcoma
| IF        =
| Gross      =
| Grossing  =
| Site      = [[soft tissue lesions|soft tissue]] esp. retroperiteoneum
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence = most common retroperitoneal sarcoma, uncommon overall
| Bloodwork  = D-dimer elevated
| Rads      = thick septae or nodules in a fatty appearing lesion
| Endoscopy  =
| Prognosis  = poor
| Other      =
| ClinDDx    =
| Tx        =
}}
'''Liposarcoma''' is a [[malignant]] [[Adipocytic tumours|adipocytic tumour]].
 
==General==
*Most common malignant sarcoma in the retroperitoneum.
*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>
**D-dimer (serum) useful; elevated levels in keeping with liposarcoma.
***Cut-off value 0.41 μg/ml for metastasis: sensitivity 0.83 and specificity 0.57.<ref name=pmid22044610>{{cite journal |authors=Morii T, Mochizuki K, Tajima T, Ichimura S, Satomi K |title=D-dimer levels as a prognostic factor for determining oncological outcomes in musculoskeletal sarcoma |journal=BMC Musculoskelet Disord |volume=12 |issue= |pages=250 |date=November 2011 |pmid=22044610 |pmc=3226444 |doi=10.1186/1471-2474-12-250 |url=}}</ref>
***Cut-off value 0.80 μg/ml for total survival: sensitivity 0.80 and specificity 0.75.<ref name=pmid22044610/>
 
Notes:
*Retroperitoneal sarcomas: #1: liposarcoma, #2: [[pleomorphic undifferentiated sarcoma]], #3: [[leiomyosarcoma]], #4: [[MPNST]].
**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==
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.
*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:
*[[Angiolipoma]].
*[[Pleomorphic undifferentiated sarcoma]].
 
===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>
Image:Myxoid_liposarcoma_%2806%29.JPG | Myxoid liposarcoma. (WC)
Image:Myxoid_liposarcoma_%2805%29.JPG | Myxoid liposarcoma. (WC)
</gallery>
www:
*[http://www.john-libbey-eurotext.fr/e-docs/00/04/09/14/texte_alt_jleejd00046_gr5.jpg Lipoblasts (john-libbey-eurotext.fr)].
*[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==
*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.
 
==See also==
*[[Adipocytic tumours]].
 
==References==
{{Reflist|2}}
 
 
[[Category:Diagnosis]]
[[Category:Adipocytic tumours]]

Latest revision as of 14:12, 11 April 2022

Liposarcoma
Diagnosis in short

Liposarcoma. H&E stain.
LM lipoblasts - key feature, chicken wire-like vascular, +/-myxoid background, cell size variation, thick fibrous septa (important low power feature)
Subtypes dedifferentiated liposarcoma, myxoid liposarcoma (includes round cell liposarcoma), mixed-type liposarcoma, pleomorphic liposarcoma, liposarcoma not otherwise specified (NOS), spindle cell liposarcoma
LM DDx pleomorphic undifferentiated sarcoma
IHC S-100 +ve
Molecular t(12;16)(q13;p11) / TLS-CHOP -- for myxoid liposarcoma
Site soft tissue esp. retroperiteoneum
Prevalence most common retroperitoneal sarcoma, uncommon overall
Blood work D-dimer elevated
Radiology thick septae or nodules in a fatty appearing lesion
Prognosis poor

Liposarcoma is a malignant adipocytic tumour.

General

  • Most common malignant sarcoma in the retroperitoneum.
  • Not all (large) retroperitoneal adipocytic tumours are liposarcomas.
  • Prognosis - dependent on the location.
    • Retroperitoneal: poor.
    • Extremity: good - disease specific 5-year survival ~ 92%.[1]
    • D-dimer (serum) useful; elevated levels in keeping with liposarcoma.
      • Cut-off value 0.41 μg/ml for metastasis: sensitivity 0.83 and specificity 0.57.[2]
      • Cut-off value 0.80 μg/ml for total survival: sensitivity 0.80 and specificity 0.75.[2]

Notes:

Gross

  • Thick fibrous septa

Radiology:[3]

  • Fibrous septa or nodules.[4]
  • Myoid liposarcoma - high signal (due to high water content).[4]

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.
  • Thick fibrous septa - important low power feature.[3]

DDx:

Images

  • Dediff. liposar. - shows dediff. component - intermed. mag. (WC)

  • Dediff. liposarc. - high mag. (WC)

  • Dediff. liposarc. - very high mag. (WC)

  • Dediff. liposarc. - lipoblasts - very high mag. (WC)

  • Myxoid liposarcoma. (WC)

  • Myxoid liposarcoma. (WC)

www:

Subtypes

Main subtypes:[5]

  • Dedifferentiated liposarcoma.
  • Myxoid liposarcoma.
    • Round cell liposarcoma - a subtype of myxoid liposarcoma[6] that has a worse prognosis;[7] characterized by regions of high cellularity.
  • Mixed-type liposarcoma.
  • Pleomorphic liposarcoma.
  • Liposarcoma not otherwise specified (NOS).

Additional reported type:

  • Spindle cell liposarcoma.[8]

Myxoid liposarcoma

  • AKA myxoid/round cell liposarcoma.
  • AKA round cell liposarcoma.
Gross

Location:[9]

  • 90% in lower limb.
  • 81% deep.
Microscopic

Features:

  • Chickenwire-type blood vessels.
  • Clear spaces.
  • Adipocytes - may be rare.

DDx:

Images:

Molecular

Typically has a translocation:

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

  • MDM2 +ve.[13]
  • CDK4 +ve.[13]
  • S-100 +ve ~1/3 of the time.

See also

References

  1. ↑ Lietman, SA.; Barsoum, WK.; Goldblum, JR.; Marks, KE.; Mascha, E.; Sundaram, M.; Muschler, G. (Mar 2007). "A 20-year retrospective review of surgically treated liposarcoma at the Cleveland Clinic.". Orthopedics 30 (3): 227-34. PMID 17375550.
  2. ↑ 2.0 2.1 Morii T, Mochizuki K, Tajima T, Ichimura S, Satomi K (November 2011). "D-dimer levels as a prognostic factor for determining oncological outcomes in musculoskeletal sarcoma". BMC Musculoskelet Disord 12: 250. doi:10.1186/1471-2474-12-250. PMC 3226444. PMID 22044610. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3226444/.
  3. ↑ 3.0 3.1 Hosono, M.; Kobayashi, H.; Fujimoto, R.; Kotoura, Y.; Tsuboyama, T.; Matsusue, Y.; Nakamura, T.; Itoh, T. et al. (Mar 1997). "Septum-like structures in lipoma and liposarcoma: MR imaging and pathologic correlation.". Skeletal Radiol 26 (3): 150-4. PMID 9108224.
  4. ↑ 4.0 4.1 4.2 Murphey, MD.; Arcara, LK.; Fanburg-Smith, J.. "From the archives of the AFIP: imaging of musculoskeletal liposarcoma with radiologic-pathologic correlation.". Radiographics 25 (5): 1371-95. doi:10.1148/rg.255055106. PMID 16160117.
  5. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 601. ISBN 978-0781765275.
  6. ↑ Smith, TA.; Easley, KA.; Goldblum, JR. (Feb 1996). "Myxoid/round cell liposarcoma of the extremities. A clinicopathologic study of 29 cases with particular attention to extent of round cell liposarcoma.". Am J Surg Pathol 20 (2): 171-80. PMID 8554106.
  7. ↑ Conyers, R.; Young, S.; Thomas, DM. (2011). "Liposarcoma: molecular genetics and therapeutics.". Sarcoma 2011: 483154. doi:10.1155/2011/483154. PMID 21253554.
  8. ↑ Dei Tos, AP.; Mentzel, T.; Newman, PL.; Fletcher, CD. (Sep 1994). "Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases.". Am J Surg Pathol 18 (9): 913-21. PMID 8067512.
  9. ↑ Moreau, LC.; Turcotte, R.; Ferguson, P.; Wunder, J.; Clarkson, P.; Masri, B.; Isler, M.; Dion, N. et al. (Apr 2012). "Myxoid\Round Cell Liposarcoma (MRCLS) Revisited: An Analysis of 418 Primarily Managed Cases.". Ann Surg Oncol 19 (4): 1081-1088. doi:10.1245/s10434-011-2127-z. PMID 22052112.
  10. ↑ Sung, MS.; Kang, HS.; Suh, JS.; Lee, JH.; Park, JM.; Kim, JY.; Lee, HG.. "Myxoid liposarcoma: appearance at MR imaging with histologic correlation.". Radiographics 20 (4): 1007-19. doi:10.1148/radiographics.20.4.g00jl021007. PMID 10903690.
  11. ↑ Knight, JC.; Renwick, PJ.; Dal Cin, P.; Van den Berghe, H.; Fletcher, CD. (Jan 1995). "Translocation t(12;16)(q13;p11) in myxoid liposarcoma and round cell liposarcoma: molecular and cytogenetic analysis.". Cancer Res 55 (1): 24-7. PMID 7805034.
  12. ↑ Suzuki, K.; Matsui, Y.; Endo, K.; Kubo, T.; Hasegawa, T.; Kimura, T.; Ohtani, O.; Yasui, N. (Nov 2010). "Myxoid liposarcoma with EWS-CHOP type 1 fusion gene.". Anticancer Res 30 (11): 4679-83. PMID 21115923.
  13. ↑ 13.0 13.1 Mariño-Enríquez, A.; Hornick, JL.; Dal Cin, P.; Cibas, ES.; Qian, X. (Feb 2014). "Dedifferentiated liposarcoma and pleomorphic liposarcoma: a comparative study of cytomorphology and MDM2/CDK4 expression on fine-needle aspiration.". Cancer Cytopathol 122 (2): 128-37. doi:10.1002/cncy.21362. PMID 24227706.
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