Cancer Report

Cancer : Soft Tissue Tumors: Low grade fibromyxoid sarcoma
Cancer Name Soft Tissue Tumors: Low grade fibromyxoid sarcoma
Cancer Alias Hyalinizing spindle cell tumor with giant rosettes
Description Low grade fibromyxoid sarcoma is a rare, low-grade malignant soft tissue neoplasm with a potential for local recurrences as well as distant metastases. Low grade fibromyxoid sarcoma belongs to the group of fibroblastic/myofibroblastic soft tissue tumors. Two main subtypes have been recognized: classical low grade fibromyxoid sarcoma and low grade fibromyxoid sarcoma with giant collagen rosettes.
Clinics and Pathology
Disease Low grade fibromyxoid sarcoma (LGFMS)
Etiology Unknown. No known risk factors.
Epidemiology Low grade fibromyxoid sarcoma is supposed to be rare, but as it is difficult to diagnose the true incidence is unknown. Patients of any age may be affected, and the male:female ratio is 1:1.
Pathology Classical cases of LGFMS display a mixture of hypocellular, collagen-rich areas and more cellular, myxoid areas. A characteristic feature is the whorling growth pattern, often seen at the transition from hypocellular to more cellular, myxoid areas. Mitotic figures are rare. A subset of LGFMS shows focal collagen rosettes.
Treatment The only consensus treatment for low grade fibromyxoid sarcoma is surgical excision.
Prognosis When radically excised, the prognosis is usually good. However, local recurrences have been reported in approximately 10% of the cases, and distant spreading occurs in 5-10% of the cases.
Related Genes
Gene Symbol CREB3L2
Description From OMIM: "Low-grade fibromyxoid sarcoma (LGFMS) is a variant of fibrosarcoma. In 2 cases of soft tissue sarcoma that fulfilled morphologic criteria for LGFMS, Storlazzi et al. (2003) identified an FUS (137070)/CREB3L2 chimera, caused by a translocation between chromosome bands 7q33-q34 (CREB3L2) and 16p11 (FUS)."
Cytogenetics Morphological The chromosomal translocation t(7;16)(q33;p11) is a characteristic feature. A few cases contain a supernumerary ring as the sole chromosomal abnormality. Comparative genomic hybridization allowed to assess the chromosomal origin of a supernumerary ring chromosome in one case. The analysis revealed gain of material from 7p14-pter, 7q31-q33, and 16p. FISH experiments using contigs of BAC clones were performed in two cases of low grade fibromyxoid sarcoma carrying a t(7;16) abnormality. The analysis revealed that the breakpoints were located within BAC clones RP11-388M20 (AC009088) in band 16p11.2, and RP11- 29B3 (AC022173) and RP11-377B19 (AC009263) in band 7q33; all the examined clones gave split signals on the derivative chromosomes 7 and 16. The FISH results for chromosome 7 identified a breakpoint region containing a single gene (LOC155008), which is homologous to Drosophila Bbf-2, encodes a B-ZIP transcription factor and named BBF2H7 (BBF2 human homolog on chromosome 7). The data for chromosome 16 suggested FUS as the other candidate target gene
Title Low-grade fibromyxoid sarcoma and hyalinizing spindle cell tumor with giant rosettes share a common t(7;16)(q34;p11) translocation.
Authors Reid, R.; de Silva, M. V. C.; Paterson, L.; Ryan, E.; Fisher, C.
Citations Am. J. Surg. Path. 27: 1229-1236, 2003.
PubMedID 12960807
Title Low-grade fibromyxoid sarcoma and hyalinizing spindle cell tumor with giant rosettes share a common t(7;16)(q34;p11) translocation.
Authors Reid R, de Silva MVC, Paterson L, Ryan E, Fisher C.
Citations Am J Surg Pathol 2003; 27: 1229-1236.
PubMedID 12915480
Title Fusion of the FUS and BBF2H7 genes in low grade fibromyxoid sarcoma.
Authors Storlazzi CT, Mertens F, Nascimento A, Isaksson M, Wejde J, Brosjo?O, Mandahl N, Panagopoulos I.
Citations Hum Mol Genet 2003; 12:2349-2358.
PubMedID 12915480
Title Hyalinizing spindle cell tumor with giant rosettes-a soft tissue tumor with mesenchymal and neuroendocrine features. An immunohistochemical, ultrastructural, and cytogenetic analysis.
Authors Bejarano PA, Padhya TA, Smith R, Blough R, Devitt JJ, Gluckman JL.
Citations Arch Pathol Lab Med 2000;124:1179-1184
PubMedID 10923080
Title Low grade fibromyxoid sarcoma
Authors Folpe A, van den Berg E, Molenaar WM.
Citations World Health Organization Classification of Tumours.Pathology and Genetics of Soft Tissue and Bone Tumours.Editors: CDM Fletcher, KK Unni, F Mertens.IARC Press, Lyon 2002; pp: 104-105
PubMedID 12960807
Title Low grade fibromyxoid sarcoma. a further low-grade soft tissue malignancy characterized by a ring chromosome..
Authors Mezzelani A, Sozzi G, Nessling M, Riva C, Della Torre G, Testi MA, Azzarelli A, Pierotti MA, Lichter P, Pilotti S.
Citations Cancer Genet Cytogenet 2000; 122: 144-148.
PubMedID 11106828
Source and Citation
Source Atlas of Genetics and Cytogenetics in Oncology and Haematology
Citation Panagopoulos I, Mertens F, Mandahl N, Storlazzi CT . Soft Tissue Tumors: Low grade fibromyxoid sarcoma. Atlas Genet Cytogenet Oncol Haematol. December 2004 .