Access Keys:
Skip to content (Access Key - 0)

Liposarcoma

DEFINITION AND PATHOGENESIS

  • Takes origin from primitive mesenchymal cells rather than mature adipose cells

IMPORTANCE

  • A fairly common malignancy of fat most common in the thigh, retroperitoneum, and inguinal regions
  • Most common in occurrence rates for soft-tissue sarcomas (20%)
  • Retroperitoneal lesions may occur simultaneously or sequentially to extremity lesions

CLINICAL FEATURES

  • 8 months to 87 years, mean 57 years, M:F = 3:2
  • Rare in subcutaneous fat and GI subserosa
  • Most common sites are thigh (25-30%) and retroperitoneum (?20%)
  • Unusual locations
  • Insidiously growing, deep-seated, ill-defined mass
  • Pain, tenderness only in 10-15%
  • Hemodynamic deteriorationin cardiac and abdominal locations reported
    • Synchronous cardiac and abdominal metastases 13 yrs after a thigh liposarcoma reported
  • Preexisting lipoma is extremely rare
  • Liposarcoma associated with multiple intramuscular lipomas reported in a 71-year-old
  • Has been reported in NF1

RADIOLOGIC FEATURES

  • Depend largely on histological type
  • Well-delineated radiolucency to dense mass
  • May have foci of calcification and ossification (esp well differentiated)
  • Vascularity often seen on bone scan
  • CT may show a low density mass with an area of higher density
    • Myxoid liposarcoma may show a homogenous density between -40 to +30 Hounsfield units
  • MRI shows inhomogeneic regions, some isointense with fat
    • Intermediate T1 signal, bright on T2
  • A liposarcoma of the pectoral muscle has been reported initially detected at mammography (heterogeneous without discrete margins) in a 76 yr-old man with painless gynecomastia

GROSS PATHOLOGY

  • Usually well encapsulated and distinctly lobulated (lobules can become separated)
  • Can be the largest of neoplasms, esp in the retroperitoneal location

HISTOLOGIC/MOLECULAR FEATURES

  • Histological classification of liposarcoma
  • AFIP
    • Well-differentiated liposarcoma (atypical lipoma)
    • Lipoma-like: scattered lipoblasts
    • Sclerosing: inguinal prevalence
    • Inflammatory: prominent inflammatory component
    • Dedifferentiated: well and poorly differentiated together
    • Myxoid liposarcoma (40-50%):lipoblasts, capillaries, myxoid matrix
    • Round cell liposarcoma: tends more to metastasize
    • Pleomorphic liposarcoma: bizarre cells
  • Evans
    • Myxoid
    • Well-differentiated
    • Dedifferentiated
    • Pleomorphic
    • Well-differentiated with a pleomorphic component
  • Lipoblasts the characteristic cell
  • Vacuolated tumor cell with single vacuole and eccentric nucleus or multiple vacuoles and a central nucleus; "signet ring" cell
  • Vacuoles within the cell contain fat (+ oil red O)
  • Cytogenetic studies
    • t(12;16),(q13;p11) (in tumor lipoblasts)
    • Other abnormalities reported include trisomy 8
  • Molecular
    • Chromosome 12 amplificationin well-differentiated and dedifferentiated liposarcomas
      • MDM2 gene
      • CDK4 gene
        • ~10% do not amplify CDK4
        • Alteration in CDKN2A/CDKN2B/CDK4/CCND1 pathwaydemonstrable
          • Array-CGH
          • Real-time genomic PCR
          • Gene expression array
          • Real-time RT-PCR
        • Chromosome 1q21-1q23 is preferential partner of chromosome 12 amplicon in tumors with amplification/overexpression CDK4
    • Over 40 microRNA expression alterations reporteddysregulated in dedifferentiated liposarcomas
      • Upregulated microRNAs
        • miR-21
        • miR-26a
      • Downregulated microRNAs
        • miR-143
          • Restoring miR-143 expression:
            • Inhibited proliferation
            • Induced apoptosis
            • Decreased expression of BCL2
            • Decreased expression of topoisomerase 2A
            • Decreased expression of protein regulator of cytokinesis (PRC1)
            • Decreased expression of polo-like kinase 1 (PLK1)
              • Tx with a PLK1 inhibitor potently induced G(2)-M growth arrest and apoptosis
        • miR-145

DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS

  • If lipoblasts are seen, the dx is made
  • Lipid stains are positive in mesenchymal or epithelial neoplasms
  • Myxoid chondrosarcoma and chordoma
  • Vacuolated forms of lymphoma ("signet-ring" lymphoma)
  • Rarely meningioma, malignant melanoma
  • Intramuscular myxoma, myxoid MFH

DISEASE COURSE AND TREATMENT

  • Wide resection, long course in some low grade lesions
  • Lung metastases (most common) as well as viscera; check retroperitoneum (extrapulmonary metastases, esp with myxoid variant)
  • +/- XRT; preoperative + postoperative boost in higher grade lesions
    • XRT effective in preventing LR but not reported to impact survival
  • Mediastinal and bone metastases have been reported
  • Prognosis
    • 5-yr survivals (with appropriate tx)
    • 74% overall 5-yr survival, all subtypes
    • 80% myxoid liposarcoma, LR <10% with resection and XRT
    • Oral and maxillofacial region
      • Tumor size > 3.6 cm
      • Histopathologic subtype
  • Chemotx trials for lesions >8cm in diameter

 SPECIAL CONSIDERATIONS

  • MYXOID LIPOSARCOMA

 

  • WELL-DIFFERENTIATED LIPOSARCOMA

  • RETROPERITONEAL LIPOSARCOMA  

    •  Any grade or histological type may be found in this location
    • Giant (50 cm and 52 kg) liposarcoma of the retroperitoneal spermatic cord reported 
    • Tumors in this location may become huge and simulate pregnancy

REFERENCES

Aman P, Ron D, Mandahl N, : Rearrangement of the transcription factor gene CHOP in myxoid liposarcomas with T(12;16)(q13;p11). Genes Chromosomes Cancer 5:278- ,1992.

Amir M, Akhtar S, Pervaiz M, Asad-ur-Rahman, Khawaja A, Ahmad I. Giant dedifferentiated retroperitoneal liposarcoma. J Coll Physicians Surg Pak. 2011 Sep;21(9):569-71.

Ariizumi T, Ogose A, Kawashima H, Hotta T, Li G, Xu Y, Hirose T, Endo N. Establishment and characterization of a novel dedifferentiated liposarcoma cell line, NDDLS-1. Pathol Int. 2011 Aug;61(8):461-8. doi: 10.1111/j.1440-1827.2011.02683.x. Pubmed

Barkhof F, Melkert P, Meyer S, Blomjous CEM: Derangement of adipose tissue: a case report of multicentric retroperitoneal liposarcoma, retroperitoneal lipomatosis and multiple subcutaneous lipomas. Eur J Surg Oncol 17:547-50,1991.
Bush CH, Spanier SS, Gillespy T: Imaging of atypical lipomas of the extremities: a report of three cases. Skeletal Radiol 17:472- ,1988.
Castleberry RP, Kelly DR, Wilson ER, Cain WS, Salter MR: Childhood liposarcoma: report of a case and review of the literature. Cancer 54:579-84,1984.

Cha EJ. Dedifferentiated liposarcoma of the small bowel mesentery presenting as a submucosal mass. World J Gastrointest Oncol. 2011 Jul 15;3(7):116-8.

Cheng J, Wang Y, Cheng A, Wang L, Tian Z, Yu H, Wang X, Wu Y, Shen G. Primary liposarcom in oral and maxillofacial region. J Craniofac Surg. 2011 Sep;22(5):1765-71. Pubmed
Crago AM, Singer S. Clinical and molecular approaches to well differentiated and dedifferentiated liposarcoma. Curr Opin Oncol. 2011 Jul;23(4):373-8. Pubmed

Crozat A, Aman P, Mandahl N, Ron D: Fusion of CHOP to a novel RNA-binding protein in human myxoid liposarcoma. Nature 363:640- ,1993.

De Nardi P, Bissolati M, Cristallo M, Staudacher C. Recurrent giant liposarcoma of the spermatic cord. Urology. 2011 Apr 12.

Enzinger FM, Weiss SW: Liposarcoma. In Soft Tissue Tumors. FM Enzinger and SW Weiss (Ed), 3rd ed., C. V. Mosby Co., St. Louis, 1995, pp. 431-466.
Evans HL: Smooth muscle in atypical lipomatous tumors: a report of three cases. Am J Surg Pathol 14:714-8,1990.
Evans HL, Khurana KK, Kemp BL, : Heterologous elements in the dedifferentiated component of dedifferentiated liposarcoma. Am J Surg Pathol 18:1150-7,1994.
Evans HL, Soule EH, Winkelmann RK: Atypical lipoma, atypical intramuscular lipoma, and well differentiated retroperitoneal liposarcoma. A reappraisal of 30 cases formerly classified as well differentiated liposarcoma. Cancer 43:574-84,1979.
Ferbman AA: Retroperitoneal fatty tumors. Report of a case and collective review of the literature from 1937 to 1947. Arch Surg 60:343-62,1950.
Folpe AL, Weiss SW: Lipoleiomyosarcoma (well-differentiated liposarcoma with leiomyosarcomatous differentiation). A clinicopathologic study of nine cases including one with dedifferentiation. Am J Surg Pathol 26:742-9,2002.

Gronchi A, Bui BN, Bonvalot S, Pilotti S, Ferrari S, Hohenberger P, Hohl RJ, Demetri GD, Le Cesne A, Lardelli P, Pérez I, Nieto A, Tercero JC, Alfaro V, Tamborini E, Blay JY. Phase II clinical trial of neoadjuvant trabectedin in patients with advanced localized myxoid liposarcoma. Ann Oncol. 2011 Jun 3.

Gutierrez A, Snyder EL, Marino-Enriquez A, Zhang YX, Sioletic S, Kozakewich E, Grebliunaite R, Ou WB, Sicinska E, Raut CP, Demetri GD, Perez-Atayde AR, Wagner AJ, Fletcher JA, Fletcher CD, Look AT. Aberrant AKT activation drives well-differentiated liposarcoma. Proc Natl Acad Sci U S A. 2011 Sep 27;108(39):16386-91. Pubmed

Henricks WH, Cho YC, Goldblum JR, : Dedifferentiated liposarcoma: a clinicopathological analysis of 155 cases with a proposal for an expanded definition of dedifferentiation. Am J Surg Pathol 21:271-81,1997.
Hisaoka M, Tsuji S, Morimitsu Y, : Detection of TLS/FUS-SHOP fusion transcripts in myxoid and round cell liposarcomas by nested reverse transcription-polymerase chain reaction using archival paraffin-embedded tissues. Diagn Mol Pathol 7:96- ,1998.

Hoffman A, Lazar AJ, Pollock RE, Lev D. New frontiers in the treatment of liposarcoma, a therapeutically resistant malignant cohort. Drug Resist Updat. 2011 Feb;14(1):52-66.

Hosono M, Kobayashi H, Fujimoto R, Kotoura Y, Tsuboyama T, Matsusue Y, Nakamura T, Itoh T, Konishi J: Septum-like structures in lipoma and liposarcoma. MR imaging and pathologic correlation. Skeletal Radiol 26:150-4,1997.

Huh WW, Yuen C, Munsell M, Hayes-Jordan A, Lazar AJ, Patel S, Wang WL, Barahmani N, Okcu MF, Hicks J, Debelenko L, Spunt SL. Liposarcoma in children and young adults: A multi-institutional experience. Pediatr Blood Cancer. 2011 Mar 10. doi: 10.1002/pbc.23095.

Kindblom-Meis JM, Enzinger FM: Color Atlas of Soft Tissue Tumors. Mosby-Wolfe, Portland, 1996, pp 74-105, 138-178.
Jelinek JS, Kransdorf MJ, Shmookler BM, Aboulafia AJ, Malawer MM: Liposarcoma of the extremities: MR and CT findings in the histologic subtypes. Radiology 186:455-9,1993.
Kransdorf MJ, Meis JM, Jelinek JS: Dedifferentiated liposarcoma of the extremities: imaging findings in four patients. AJR 161:127- ,1993.
Kuroda M, Ischida T, Horiuchi H, : Chimeric TLS/FUS-CHOP gene expression and the heterogeneity of its junction in human myxoid and round cell liposarcoma. Am J Pathol 147:1221- ,1995.
Johnston JO, Genant HK, Rosenau W: Soft tissue thigh mass in a 29-year-old man. Clin Orthop 310:278-80,83,1995.
London J, Kim EE, Wallace S, : MR imaging liposarcomas: correlation of MR features and histology. J Comput Assist Tomogr 18:832- ,1989.

Lazopoulos G, Papastavrou L, Kantartzis M. Giant metastatic cardiac and abdominal liposarcomas causing hemodynamic deterioration. Thorac Cardiovasc Surg. 2011 Mar;59(2):121-3.

Louis-Brennetot C, Coindre JM, Ferreira C, Pérot G, Terrier P, Aurias A. The CDKN2a/CDKN2B/CDK4/CCND1 pathway is pivotal in well-differentiated and dedifferentiated liposarcoma oncogenesis: an analysis of 104 tumors. Genes Chromosomes Cancer. 2011 Nov;50(11):896-907. doi: 10.1002/gcc.20909. Pubmed

Okada K, Hasegawa T, Kawai A, Ogose A, Nishida J, Yanagisawa M, Morita T, Tajino T, Tsuchiya T. Primary (de novo) dedifferentiated liposarcoma in the extremities: a multi-institution Tohoku Musculoskeletal Tumor Society study of 18 cases in northern Japan. Jpn J Clin Oncol. 2011 Sep;41(9):1094-100. Pubmed

Matsumoto K, Hukuda S, Ishizawa M, Egawa M, Okabe H: Liposarcoma associated with multiple intramuscular lipomas. A case report. Clin Orthop 373:202-7,2000.

Moreau LC, Turcotte R, Ferguson P, Wunder J, Clarkson P, Masri B, Isler M, Dion N, Werier J, Ghert M, Deheshi B; the Canadian Orthopaedic Oncology Society (CANOOS). Myxoid/Round Cell Liposarcoma (MRCLS) revisited: An analysis of 418 primarily managed cases. Ann Surg Oncol. 2011 Nov 4. Pubmed

Okada K, Hasegawa T, Kawai A, Ogose A, Nishida J, Yanagisawa M, Morita T, Tajino T, Tsuchiya T. Primary (de novo) dedifferentiated liposarcoma in the extremities: a multi-institution Tohoku Musucloskeletal Tumor Society study of 18 cases in northern Japan. Jpn J Clin Oncol. 2011 Sep;41(9):1094-100.

Pearlstone DB, Pisters PWT, Bold RJ, Feig BW, Hunt KK, Yasko AW, Patel S, Pollack A, Benjamin RS, Pollock RE: Patterns of recurrence in extremity liposarcoma: implications for staging and follow-up. Cancer 85:85-92,1999.

Peng T, Zhang P, Liu J, Nguyen T, Bolshakov S, Belousov R, Young ED, Wang X, Brewer K, López-Terrada DH, Oliveira AM, Lazar AJ, Lev D. An experimental model for the study of well-differentiated and dedifferentiated liposarcoma: deregulation of targetable tyrosine kinase receptors. Lab Invest. 2011 Mar;91(3):392-403. Pubmed

Plaat BE, Molenaar WM, Sagrudny J, Bohle RM, Mastik MF, Hoekstra HJ, Van der Graaf WT, Hollema H, van den Berg E. The 16p11 breakpoint in myxoid liposarcomas might affect the expression of the LRP gene on 16p11.2 encoding the multidrug resistance associated major vault protein. Eur J Clin Invest. 2000 May;30(5):447-53.

Polsky MS, Vitenson JH, Wilson JM, Woodhead DM, Weber CH. Retrovesical liposarcoma. Urology. 1974 Feb;3(2):226-8.

Sampson CC, Saunders EH, Green WE, Laurey JR: Liposarcoma developing in a lipoma. Arch Pathol 69:506-10,1960.

Schofer MD, Abu-Safieh MY, Paletta J, Fuchs-Winkelmann S, El-Zayat BF. Liposarcoma of the forearm in a man with type 1 neurofibromatosis: a case report. J Med Case Reports. 2009 Apr 29;3:7071.
So NMC, Yang WT, Metreweli C: Imaging features of liposarcoma of the pectoral muscle. AJR 172:1148-9,1999.

Spittle MF, Newton KA, Mackenzie DH. Liposarcoma. A review of 60 cases. Br J Cancer. 1970 Dec;24(4):696-704.
Springfield D: Liposarcoma. Clin Orthop 289:50-7,1993.
Sreekantaiah C, Karakousis CP, Leong SP, Sandberg AA: Cytogenetic findings in liposarcoma correlate with histopathologic subtypes. Cancer 69:2484- ,1992.
Starkloff GB, Saxton JA, Jr, Johnson RE: Liposarcoma of an extremity associated with multiple subcutaneous lipomas. Report of two cases treated by amputation. Ann Surg 133:261-6,1951.
Sternberg SS: Liposarcoma arising within a subcutaneous lipoma. Cancer 5:975-8,1952.

Stoller FM, Davies DG. Liposarcoma of the neck. Arch Otolaryngol. 1968 Oct;88(4):419-22.

Susini T, Taddei G, Massi D, Massi G. Giant pelvic retroperitoneal liposarcoma. Obstet Gynecol. 2000 Jun;95(6 Pt 2):1002-4.
Suster S, Wong TY, Moran CA: Sarcomas with combined features of liposarcoma and leiomyosarcoma: study of two cases of an unusual soft-tissue tumor showing dual lineage differentiation. Am J Surg Pathol 17:905-11,1993.
Tallini G, Erlandson RA, Brennan MF, : Divergent myosarcomatous differentiation in retroperitoneal liposarcoma. Am J Surg Pathol 17:546-56,1993.
Turc-Carel C, Limon J, Dal Cin P, : Cytogenetic studies of adipose tissue tumors. II. Recurrent reciprocal translocation t(12;16)(q13;p11) in myxoid liposarcomas. Cancer Genet Cytogenet 23:291- ,1986.

Ugras S, Brill E, Jacobsen A, Hafner M, Socci ND, Decarolis PL, Khanin R, O'Connor R, Mihailovic A, Taylor BS, Sheridan R, Gimble JM, Viale A, Crago A, Antonescu CR, Sander C, Tuschl T, Singer S. Small RNA sequencing and functional characterization reveals MicroRNA-143 tumor suppressor activity in liposarcoma. Cancer Res. 2011 Sep 1;71(17):5659-69. Pubmed

Wang L, Wang L, Song R, Shen Y, Sun Y, Gu Y, Shu Y, Xu Q. Targeting sarcoplasmic/endoplasmic reticulum Ca2+-ATPase 2 by curcumin induces ER stress-associated apoptosis for treating human liposarcoma. Mol Cancer Ther. 2011 Mar;10(3):461-71.

Wang X, Asmann YW, Erickson-Johnson MR, Oliveira JL, Zhang H, Moura RD, Lazar AJ, Lev D, Bill K, Lloyd RV, Yaszemski MJ, Maran A, Oliveira AM. High-resolution genomic mapping reveals consistent amplification of the fibroblast growth factor receptor substrate 2 gene in well-differentiated and dedifferentiated liposarcoma. Genes Chromosomes Cancer. 2011 Nov;50(11):849-58. doi: 10.1002/gcc.20906. Pubmed

Weiss SW, Rao VK: Well-differentiated liposarcoma (atypical lipoma) of deep soft tissue of the extremities, retroperitoneum, and miscellaneous sites: a follow-up study of 92 cases with analysis of the incidence of 'dedifferentiation'. Am J Surg Pathol 16:1051-8,1992.
Yoshikawa H, Ueda T, Mori S, Araki N, Myoui A, Uchida A, Fukuda H: Dedifferentiated liposarcoma of the subcutis. Am J Surg Pathol 20:1525-30,1996.

Peer Review

OrthopaedicsOne Peer Review Workflow is an innovative platform that allows the process of peer review to occur right within an OrthopaedicsOne article in an open, transparent and flexible manner. Learn more

Instructions for Authors

Read our Instructions for Authors to learn about contributing or editing articles on OrthopaedicsOne.

Content Partner

Learn about becoming an OrthopaedicsOne Content Partner.

Academic Resources

Resources on Liposarcoma from Pubget.

Error rendering macro 'rss' : The RSS macro is retrieving an HTML page.
Related Content

Resources on Liposarcoma and related topics in OrthopaedicsOne spaces.

Page: Liposarcoma (OrthopaedicsOne Articles)
Page: Myositis ossificans (OrthopaedicsOne Articles)
Page: Glomus tumor (OrthopaedicsOne Articles)
Page: Pigmented villonodular synovitis (PVNS) (OrthopaedicsOne Articles)
Page: Synovial sarcoma (OrthopaedicsOne Articles)
Page: Giant cell tumor of tendon sheath (OrthopaedicsOne Articles)
Page: Fibromatosis (OrthopaedicsOne Articles)
Page: Dermatofibrosarcoma protuberans (DFSP) (OrthopaedicsOne Articles)
Page: Angiosarcoma (OrthopaedicsOne Articles)
Page: Desmoplastic fibroma (OrthopaedicsOne Articles)
Showing first 10 of 152 results