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>Journals >Cirugía y Cirujanos >Year 2013, Issue 4

Citores-Pascual MÁ, Tinoco-Carrasco C, Arenal-Vera JJ, Benito- Fernández C, Torres-Nieto MÁ, Zamora-Martínez T
Low grade fibromixoid sarcoma: a purpose of 3 cases and review of the bibliography
Cir Cir 2013; 81 (4)

Language: Español
References: 18
Page: 333-339
PDF: 470.46 Kb.

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Background: Low grade fibromyxoid sarcoma or tumor Evans is a variety of soft tissue sarcoma that represents 1% of all malignancies. More common in limbs and trunk (50%), we present 3 new cases of retroperitoneal reviewing their characteristics and performing a literature review.
Clinical case: In the retroperitoneal location highlights the poor specificity of clinical symptoms, demonstrating, according to their growth and size, as an abdominal tumor, usually painless, or by compression and / or invasion of nearby structures. In cases that present the most important finding was the presence of a palpable abdominal tumor without other symptoms despite remarkable infiltrate other organs that should be included in surgical resection.
Conclusions: We emphasize the difficulty of correct diagnosis preoperatively because preoperative studies are inconclusive and only the histological and immuno-histo-typing chemistry allow precise identification.

Key words: Low-grade fibromyxoid sarcoma, soft tissue sarcoma, retroperitoneal tumor.


  1. Panagopoulos I, Mertens F, Mandahl N, Storlazzi CT. Soft tissue Tumors: Low grade fibromyxoid sarcoma. Atlas Genet Cytogenet Oncol Haematol 2005; 9(1):52-54. Disponible en: http://documents.irevues.inist.fr/bitstream/2042/38164/1/12-2004- LowGradFibromyxSarcID5185.pdf. Published in Atlas Database: December 2004 en http://AtlasGeneticsOncology.org

  2. Billings SD, Giblen G, Fanburg-Smith JC. Superficial Low-grade Fibromyxoid Sarcoma (Evans Tumor): A Clinicopathologic Analysis of 19 Cases With a Unique Observation in the Pediatric Population. Am J Surg Pathol 2005;29(2):204-210.

  3. Wu X, Petrovic V, Torode IP, Chow CW. Low-grade fibromyxoid sarcoma: problems in the diagnosis and management of a malignant tumour with bland histological appearance. Pathology 2009;41(2):155-160.

  4. Park IJ, Kim HC, Yu CS, Kim JS, Jang SJ, Kim JC. Low-grade fibromyxoid sarcoma of the colon. Dig Liver Dis 2007;39(3):274- 277.

  5. Lee AF, Yip S, Smith AC, Hayes MM, Nielsen TO, O’Connell JX. Low-grade fibromyxoid sarcoma of the perineum with heterotopic ossification: case report and review of the literature. Hum Pathol 2011;42(11):1804-1809.

  6. Del Valle González N, Santos Largo J, Martínez-Sagarra Oceja J, Rodríguez Tesedo V. Sarcoma fibromixoide renal. Actas Urol Esp 2009;33(9):1036-1039.

  7. Rose B, Tamvakopoulos GS, Dulay K, Pollock R, Skinner J, Briggs T, et al. The clinical significance of the FUS-CREB3L2 translocation in low-grade fibromyxoid sarcoma. J Orthop Surg Res 2011;6(1):15.

  8. Folpe AL, Lane KL, Paull G, Weiss SW. Low-Grade Fibromixoid Sarcoma and Hyalinizing Spindle Cell Tumor With Giant Rosettes: A Clinicopathologic Study of 73 Cases Supporting Their Identity and Assessing the Impact of High-Grade Areas. Am J Surg Pathol 2000;24(10):1353-1360.

  9. Evans HL. Low-grade fibromyxoid sarcoma. A report of two metastasizing neoplasms having a deceptively benign appearance. Am J Clin Pathol 1987;88(5):615-619.

  10. Folpe A, Van den Berg E, Molenaar WM. Low-Grade Fibromyxoid Tumor Sarcoma. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Fletcher C.D.M., Unni K.K., Mertens F., editor. Low grade fibromyxoid sarcoma. Lyon AND IARC Press, 2002 p. 104-105.

  11. Evans HL. Low-grade fibromyxoid sarcoma. A report of 12 cases. Am J Surg Pathol 1993;17(6):595-600.

  12. Arnaoutoglou Christina C, Lykissas Marios G, MG Gelalis, Ioannis D ID, Batistatou Anna A, Goussia Anna A, et al. Low-grade fibromyxoid sarcoma: A case report and review of the literature. J Orthop Surg Res 2010; 5:49-57. Published online 2010 July 29. doi: 10.1186/1749-799X-5-49

  13. Kim SK, Jee WH, Lee AW, Chung YG. Haemorrhagic low-grade fibromyxoid sarcoma: MR findings in two young women. Br J Radiol 2011;84(1003):146-150.

  14. Vernon SE, Bejarano PA. Low-grade fibromyxoid sarcoma: A brief review. Arch Pathol Lab Med 2006;130(9):1358-1360.

  15. Laurini JA, Zhang L, Goldblum JR, Montgomery E, Folpe AL. Low-grade fibromyxoid sarcoma of the small intestine: report of 4 cases with molecular cytogenetic confirmation. Am J Surg Pathol 2011;35(7):1069-1073.

  16. Reid R, de Silva MV, Paterson L, Ryan E, Fisher C. Low-grade fibromyxoid sarcoma and hyalinizing spindle cell tumor with giant rosettes share a common t(7;16)(q34;p11) translocation. Am J Surg Pathol 2003;27(9):1229-1236.

  17. Mertens F, Fletcher CD, Antonescu CR, Coindre JM, Colecchia M, Domanski HA, et al. Clinicopathologic and molecular genetic characterization of low-grade fibromyxoid sarcoma, and cloning of a novel FUS/CREB3L1 fusion gene. Lab Invest 2005;85(3):408- 415.

  18. Doyle LA, Möller E, Dal Cin P, Fletcher CD, Mertens F, Hornick JL. MUC4 is a highly sensitive and specific marker for low-grade fibromyxoid sarcoma. Am J Surg Pathol 2011;35(5):733-741.

>Journals >Cirugía y Cirujanos >Year 2013, Issue 4

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