Liposarcoma
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| Liposarcoma | |
|---|---|
| Diagnosis in short | |
 Liposarcoma. H&E stain. | |
| LM DDx | pleomorphic undifferentiated sarcoma |
| Site | soft tissue esp. retroperiteoneum |
|
| |
| Prevalence | most common retroperitoneal sarcoma, uncommon overall |
| Prognosis | poor |
Liposarcoma is a malignant 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:
Images
Myxoid liposarcoma. (WC)
Myxoid liposarcoma. (WC)
.JPG)
.JPG)
Dediff. liposarcoma - lipoblasts - very high mag. (WC)
Dediff. liposarcoma - shows dediff. component - intermed. mag. (WC)
www:
Subtypes
Main subtypes:[1]
- Dedifferentiated liposarcoma.
- Myxoid liposarcoma.
- Mixed-type liposarcoma.
- Pleomorphic liposarcoma.
- Liposarcoma not otherwise specified (NOS).
Additional reported type:
- Spindle cell liposarcoma.[4]
Myxoid liposarcoma
Gross
Location:[5]
- 90% in lower limb.
- 81% deep.
Microscopic
Features:
- Chickenwire-type blood vessels.
- Clear spaces.
- Adipocytes - may be rare.
DDx:
Images:
- Myxoid liposarcoma (rsna.org).[6]
- Myxoid liposarcoma - among other things (rsna.org).[7]
- Myxoid liposarcoma (cap.org).
Molecular
Typically has a translocation:
- t(12;16)(q13;p11) TLS-CHOP.[8]
- May have the translocation usually seen in clear cell sarcoma:
- t(12;22)(q13;q12) EWS-ATF1.[9]
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
References
- ↑ 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.
- ↑ 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.
- ↑ Conyers, R.; Young, S.; Thomas, DM. (2011). "Liposarcoma: molecular genetics and therapeutics.". Sarcoma 2011: 483154. doi:10.1155/2011/483154. PMID 21253554.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.