Abstract
Dedifferentiated areas of dedifferentiated liposarcoma (DDL) usually show malignant fibrous histiocytoma (MFH)- or fibrosarcoma-like features and lack any histologic signs of specific differentiation. However, some reports have demonstrated specific differentiation in these areas, with histologic features resembling those of rhabdomyosarcoma, leiomyosarcoma, and osteosarcoma. We report here a pathologic and genetic analysis of three cases of DDLs with rhabdomyosarcomatous areas. MFH- or fibrosarcoma-like areas of one primary DDL and two recurrent DDLs contained various amounts of rhabdomyoblasts, which were immunoreactive for desmin, myoglobin, muscle actin (HHF-35), and myogenin. An ultrastructural examination demonstrated rhabdomyoblasts with abundant cytoplasm containing thin and thick filaments and Z-bands. By real-time PCR, amplification of mdm2 and cdk4 was confirmed in both well-differentiated and dedifferentiated areas with rhabdomyoblasts of all cases. To our knowledge, only seven cases of DDLs with rhabdomyosarcomatous components have been reported, and furthermore, the genetic profiles of the rhabdomyosarcomatous components in DDLs have not been investigated. This study demonstrates that DDLs with rhabdomyosarcomatous areas have genetic alterations that are common to well-differentiated/dedifferentiated liposarcomas.
Similar content being viewed by others
References
Dei Tos AP, Doglioni C, Piccinin S, Maestro R, Mentzel T, Barbareschi M et al (1997) Molecular abnormalities of the p53 pathway in dedifferentiated liposarcoma. J Pathol 181:8–13
Dei Tos AP, Doglioni C, Piccinin S, Sciot R, Furlanetto A, Boiocchi M et al (2000) Coordinated expression and amplification of the MDM2, CDK4, and HMGI-C genes in atypical lipomatous tumours [comment]. J Pathol 190:531–536
Evans HL (1979) Liposarcoma: a study of 55 cases with reassessment of its classification. Am J Surg Pathol 3:507–523
Evans HL, Khurana KK, Kemp BL, Ayala AG (1994) Heterologous elements in the dedifferentiated component of dedifferentiated liposarcoma. Am J Surg Pathol 18:1150–1157
Henrichs WH, Chu YC, Goldblum JR, Weiss SW (1997) Dedifferentiated liposarcoma. A clinicopathological analysis of 155 cases with a proposal for an expanded definition of dedifferentiation. Am J Surg Pathol 21:271–281
Hostein I, Pelmus M, Aurias A, Pedeutour F, Mathoulin-Pelissier S, Coindre JM (2004) Evaluation of MDM2 and CDK4 amplification by real-time PCR on paraffin-embedded material: a potential tool for the diagnosis of atypical lipomatous tumours/well differentiated liposarcomas. J Pathol 202:95–102
McCormick D, Metzel T, Beham A, Fletcher C (1994) Dedifferentiated liposarcoma: clinicopathologic analysis of 32 cases suggesting a better prognostic group among pleomorphic sarcomas. Am J Surg Pathol 18:1213–1223
Newman PL, Fletcher CD (1991) Malignant mesenchymoma. Clinicopathologic analysis of a series with evidence of low-grade behaviour. Am J Surg Pathol 15:607–614
Pedeutour F, Forus A, Coindre JM, Berner JM, Nicolo G, Michiels JF et al (1999) Structure of the supernumerary ring and giant rod chromosomes in adipose tissue tumors. Genes Chromosomes Cancer 24:30–41
Pedeutour F, Suijkerbuijk RF, Forus A, Van Gaal J, Van de Klundert W, Coindre JM et al (1994) Complex composition and co-amplification of SAS and MDM2 in ring and giant rod marker chromosomes in well-differentiated liposarcoma. Genes Chromosomes Cancer 10:85–94
Pilotti S, Della Torre G, Lavarino C, Di Palma S, Sozzi G, Minoletti F et al (1997) Distinct mdm2/p53 expression patterns in liposarcoma subgroups: implications for different pathogenetic mechanisms. J Pathol 181:14–24
Pilotti S, Della Torre G, Mezzelani A, Tamborini E, Azzarelli A, Sozzi G et al (2000) The expression of MDM2/CDK4 gene product in the differential diagnosis of well differentiated liposarcoma and large deep-seated lipoma. Br J Cancer 82:1271–1275
Rosai J, Akerman M, Dal Cin P, DeWever I, Fletcher CD, Mandahl N et al (1996) Combined morphologic and karyotypic study of 59 atypical lipomatous tumors. Evaluation of their relationship and differential diagnosis with other adipose tissue tumors (a report of the CHAMP Study Group). Am J Surg Pathol 20:1182–1189
Salzano RP, Tomkiewicz Z, Africano WA (1991) Dedifferentiated liposarcoma with features of rhabdomyosarcoma. Conn Med 55:200–202
Schneider-Stock R, Walter H, Radig K, Rys J, Bosse A, Kuhnen C et al (1998) MDM2 amplification and loss of heterozygosity at Rb and p53 genes: no simultaneous alterations in the oncogenesis of liposarcomas. J Cancer Res Clin Oncol 124:532–540
Stout A (1948) Mesenchymoma, the mixed tumor of mesenchymal derivatives. Ann Surg 127:278–290
Tallini G, Erlandson R, Brennan M, Woodruff J (1993) Divergent myosarcomatous differentiation in retroperitoneal liposarcoma. Am J Surg Pathol 17:546–556
Tos APD, Pedeutour F (2002) Dedifferentiated liposarcoma. Pathology and genetics of tumours of soft tissue and bone. IARC Press, Lyon
Weiss SWW, Goldblum JR (2001) Enzinger and Weiss's soft tissue tumors, 4th edn. Mosby, St. Louis
Yoo J, Park SY, Kang SJ, Shim SI, Kim BK (2002) Altered expression of G1 regulatory proteins in human soft tissue sarcomas. Arch Pathol Lab Med 126:567–573
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Shimada, S., Ishizawa, T., Ishizawa, K. et al. Dedifferentiated liposarcoma with rhabdomyoblastic differentiation. Virchows Arch 447, 835–841 (2005). https://doi.org/10.1007/s00428-005-0025-0
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00428-005-0025-0