Liposarcoma myxoid

The Wilms tumor-1 (WTl) gene is located on the short arm of chromosome 11 (llpl3). It encodes a protein that is a critical determinant of urogenital development and is expressed in >80% of nephroblastomas. With regard to osseous and soft tissue tumors, WTl protein is not a specific marker and is potentially present in angiosarcoma, malignant peripheral nerve sheath tumor, synovial sarcoma, osteosarcoma, myxoid liposarcoma, and clear-cell sarcoma.Nevertheless, it is most often utilized (in a structured panel with other immunodeter-minants) as an adjunctive indicator of endothelial differentiation in diagnostic practice. ... 

Aggressive angiomyxoma (AAM) is a peculiar neoplasm of the pelvic and perineal soft tissues that most commonly affects women. It is composed of loosely arranged, bland spindled to stellate cells embedded in a myxoid matrix punctuated by numerous venule- and capillary-sized blood vessels reminiscent of myxoid liposarcoma (Fig. 4.8). The vascular pattern of AAM lacks the arborizing appearance that is seen in some overtly malignant myxoid tumors. Some morphologic and immunophenotypic features of aggressive AAM also are shared in part by intramuscular myxoma. 

Gibas Z, Miettinen M, Limon J, et al. Cytogenetic and immunohistochemical profile of myxoid liposarcoma. Am J Clin Pathol. 1995 103 20-26. 

Myxoid liposarcoma Myxoid liposarcoma Acute myeloid leukemia Acute myeloid leukemia Ewing s sarcoma Ewing s sarcoma Ewing s sarcoma Clear cell sarcoma... 

The t(12 16)(ql3 pll) translocation is the most common translocation, described in more than 90% of myxoid and round cell liposarcoma, mainly those arising within the thigh soft tissue. Myxoid liposarcoma arising elsewhere seems to be not related to this specific genetic abnormality. The t(12 16)(ql3 pll) translocation is caused by the fusion of the TLS gene and the CHOP gene. 

Some studies show that there is no significant association between the fusion variant and the tumor behavior but other studies reported that the poorly differentiated round cell type of myxoid liposarcoma is mainly associated with the fusion transcript type 1. 

Combined morphologic and karyotypic study of 28 myxoid Liposarcoma. Am J Surg Pathol, 20, 1047-1055. 

Hosaka, T, Nakashima, Y, Kusuzaki, K. et al. (2002) A novel type of EWS-CHOP fusion transcript gene in two cases of myxoid liposarcoma./ Mol Diagn, 4, 164-171. 

Myxoid liposarcoma with adipocytic maturation detection ofTLS/CHOP fusion transcript. Diag Mol Pathol, 13, 92-96. 

Matsui, Y, Ueda, T, Kubo, T. et al. (2006) A novel type of EWS-CHOP fusion gene in myxoid liposarcoma. Biochem Biophys Res Commun, 22, 437-444. 

Fomi C, Minuzzo M, Virdis E, TambOTini E, Simone M, Tavecchio M, Erba E, Grosso F, Gronchi A, Amari P, Casali P, D lcald M, Pilotti S, Mantovani R. Trabectedin (ET-743) promotes diifraentiation in myxoid liposarcoma tumtns. Mol Cancer Ther. 2009 6 449-57. 

Stephenson CA (1990) Sonographic diagnosis of tenosynovitis of the posterior tibial tendon. J Clin Ultrasound 18 114-116 Sung MS, Kang HS, Suh JS et al (2000) Myxoid liposarcoma appearance at MR imaging with histologic correlation. RadioGraphics 20 1007-1019... 

Especially when deep-seated, sarcomas of the thigh may have reached a considerable size by the time they become clinically apparent. In fact, it is not uncommon that even large-sized tumors are found during a US examination. The US appearance of these lesions is nonspecific, although myxoid liposarcomas often have a homogeneous hyperechoic echotexture (Fig. 13.38). 

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