Dedifferentiated liposarcoma (DLPS) is one of the most frequent sarcomas of the retroperitoneum and represents most undifferentiated sarcomas of the internal trunk.
In about 5% cases, the dedifferentiated component is an heterologous sarcoma such as leiomyosarcoma or rhabdomyosarcoma.
Variants
dedifferentiated liposarcoma with rhabdomyoblastic differentiation (16012847)
Cytogenetics
Cytogenetic anomalies similar to those seen in atypical lipomatous tumor/well-differentiated liposarcoma (ALT/WDLS)
Differential diagnosis
Dedifferentiated liposarcoma (DDL), occurring in up to 10% of well differentiated liposarcoma cases, has similar histologic features to that of undifferentiated high-grade pleomorphic sarcoma (UHGPS); the former develops in a background of atypical lipomatous tumors/well differentiated liposarcoma, whereas the latter shows no specific line of differentiation. The retroperitoneum and thigh represent the most common anatomic locations for both the sarcomas. (19574885)
References
Overlapping Features Between Dedifferentiated Liposarcoma and Undifferentiated High-Grade Pleomorphic Sarcoma. Chung L, Lau SK, Jiang Z, Loera S, Bedel V, Ji J, Weiss LM, Chu PG. Am J Surg Pathol. 2009 Jul 1. PMID: 19574885
Binh MB, Guillou L, Hostein I, Chateau MC, Collin F, Aurias A, Binh BN, Stoeckle E, Coindre JM. Dedifferentiated Liposarcomas With Divergent Myosarcomatous Differentiation Developed in the Internal Trunk: A Study of 27 Cases and Comparison to Conventional Dedifferentiated Liposarcomas and Leiomyosarcomas. Am J Surg Pathol. 2007 Oct;31(10):1557-1566. PMID: 17895758
Shimada S, Ishizawa T, Ishizawa K, Kamada K, Hirose T. Dedifferentiated liposarcoma with rhabdomyoblastic differentiation. Virchows Arch. 2005 Nov;447(5):835-41. PMID: 16012847
Coindre JM, Hostein I, Maire G, Derre J, Guillou L, Leroux A, Ghnassia JP, Collin F, Pedeutour F, Aurias A. Inflammatory malignant fibrous histiocytomas and dedifferentiated liposarcomas: histological review, genomic profile, and MDM2 and CDK4 status favour a single entity. J Pathol. 2004 Jul;203(3):822-30. PMID: 15221942
Coindre JM, Mariani O, Chibon F, Mairal A, De Saint Aubain Somerhausen N, Favre-Guillevin E, Bui NB, Stoeckle E, Hostein I, Aurias A. Most malignant fibrous histiocytomas developed in the retroperitoneum are dedifferentiated liposarcomas: a review of 25 cases initially diagnosed as malignant fibrous histiocytoma. Mod Pathol. 2003 Mar;16(3):256-62. PMID: 12640106