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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| Symptoms  =
| Symptoms  =
| Prevalence = most common retroperitoneal sarcoma, uncommon overall
| Prevalence = most common retroperitoneal sarcoma, uncommon overall
| Bloodwork  =
| Bloodwork  = D-dimer elevated
| 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>
**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:
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>
Image:Dedifferentiated_liposarcoma_-_cropped_-_very_high_mag.jpg | Dediff. liposarcoma - lipoblasts - very high mag. (WC)
Image:Dedifferentiated_liposarcoma_-_intermed_mag.jpg | Dediff. liposarcoma - shows dediff. component - intermed. mag. (WC)
</gallery>
</gallery>
www:
www:
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==IHC==
==IHC==
*IHC is of limited value.
*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.
*Reticulin ???.


==See also==
==See also==

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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