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Primary clear cell sarcoma of the sacrum: a case report

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Abstract

Clear cell sarcoma, first described by Enzinger in 1965, is an uncommon malignant soft tissue neoplasm that displays melanocytic differentiation. It occurs predominantly in the soft tissue of the extremities in young adults or children. Primary clear cell sarcoma of the bone is extremely rare, only seven cases have been reported to our knowledge. We present here a case of a huge primary clear cell sarcoma of the sacrum arising in a 25-year-old man. The average percentage of melanin content in the tumor was 9.25 ± 6.71%, which resulted in hypointense signal in T2-weighted and heterogeneous hyperintense signal in T1-wieghted images of magnetic resonance imaging (MRI). The patient was treated with curettage of the mass and neither further radiotherapy nor chemotherapy had been provided. The patient had local regrowth of tumor at 9 months after surgery. To our knowledge, this is the first case of CCS in the axial skeleton documented in English literature.

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References

  1. Enzinger FM. Clear-cell sarcoma of tendons and aponeuroses. An analysis of 21 cases. Cancer. 1965;18:1163–74.

    Article  PubMed  CAS  Google Scholar 

  2. Segal NH, Pavlidis P, Noble WS, et al. Classification of clear-cell sarcoma as a subtype of melanoma by genomic profiling. J Clin Oncol. 2003;21(9):1775–81.

    Article  PubMed  CAS  Google Scholar 

  3. Kawai A, Hosono A, Nakayama R, et al. Clear cell sarcoma of tendons and aponeuroses: a study of 75 patients. Cancer. 2007;109(1):109–16.

    Article  PubMed  CAS  Google Scholar 

  4. Fletcher CDM, UK, Mertens F, editors. World Health Organization Classification of Tumors. Pathology and genetics. Tumors of soft tissue and bones. IARC Press, Lyon, 2002.

  5. Panagopoulos I, Mertens F, Isaksson M, Mandahl N. Absence of mutations of the BRAF gene in malignant melanoma of soft parts (clear cell sarcoma of tendons and aponeuroses). Cancer Genet Cytogenet. 2005;156(1):74–6.

    Article  PubMed  CAS  Google Scholar 

  6. Zucman J, Delattre O, Desmaze C, et al. EWS and ATF-1 gene fusion induced by t(12;22) translocation in malignant melanoma of soft parts. Nat Genet. 1993;4(4):341–5.

    Article  PubMed  CAS  Google Scholar 

  7. Mrozek K, Karakousis CP, Perez-Mesa C, Bloomfield CD. Translocation t(12;22)(q13;q12.2-12.3) in a clear cell sarcoma of tendons and aponeuroses. Genes Chromosom Cancer. 1993;6(4):249–52.

    Article  PubMed  CAS  Google Scholar 

  8. Langezaal SM, Graadt van Roggen JF, Cleton-Jansen AM, Baelde JJ, Hogendoorn PC. Malignant melanoma is genetically distinct from clear cell sarcoma of tendons and aponeurosis (malignant melanoma of soft parts). Br J Cancer. 2001;84(4):535–8.

    Article  PubMed  CAS  Google Scholar 

  9. Hantschke M, Mentzel T, Rutten A, et al. Cutaneous clear cell sarcoma: a clinicopathologic, immunohistochemical, and molecular analysis of 12 cases emphasizing its distinction from dermal melanoma. Am J Surg Pathol. 2010;34(2):216–22.

    Article  PubMed  Google Scholar 

  10. Choi JH, Gu MJ, Kim MJ, et al. Primary clear cell sarcoma of bone. Skeletal Radiol. 2003;32(10):598–602.

    Article  PubMed  CAS  Google Scholar 

  11. Ekfors TO, Kujari H, Isomaki M. Clear cell sarcoma of tendons and aponeuroses (malignant melanoma of soft parts) in the duodenum: the first visceral case. Histopathology. 1993;22(3):255–9.

    Article  PubMed  CAS  Google Scholar 

  12. Donner LR, Trompler RA, Dobin S. Clear cell sarcoma of the ileum: the crucial role of cytogenetics for the diagnosis. Am J Surg Pathol. 1998;22(1):121–4.

    Article  PubMed  CAS  Google Scholar 

  13. Rubin BP, Fletcher JA, Renshaw AA. Clear cell sarcoma of soft parts: report of a case primary in the kidney with cytogenetic confirmation. Am J Surg Pathol. 1999;23(5):589–94.

    Article  PubMed  CAS  Google Scholar 

  14. Fukuda T, Kakihara T, Baba K, Yamaki T, Yamaguchi T, Suzuki T. Clear cell sarcoma arising in the transverse colon. Pathol Int. 2000;50(5):412–6.

    Article  PubMed  CAS  Google Scholar 

  15. Saw D, Tse CH, Chan J, Watt CY, Ng CS, Poon YF. Clear cell sarcoma of the penis. Hum Pathol. 1986;17(4):423–5.

    Article  PubMed  CAS  Google Scholar 

  16. Huang W, Zhang X, Li D, et al. Osteoclast-rich tumor of the gastrointestinal tract with features resembling those of clear cell sarcoma of soft parts. Virchows Arch. 2006;448(2):200–3.

    Article  PubMed  Google Scholar 

  17. Pauwel P, Debiec-Rychter M, Sciot R, et al. Clear cell sarcoma of the stomach. Histopathology. 2002;41(6):526–30.

    Article  Google Scholar 

  18. Taminelli L, Zaman K, Gengler C, et al. Primary clear cell sarcoma of the ileum: an uncommon and misleading site. Virchows Arch. 2005;447(4):772–7.

    Article  PubMed  Google Scholar 

  19. Zambrano E, Reyes-Mugica M, Franchi A, Rosai J. An osteoclast-rich tumor of the gastrointestinal tract with features resembling clear cell sarcoma of soft parts: reports of 6 cases of a GIST simulator. Int J Surg Pathol. 2003;11(2):75–81.

    Article  PubMed  Google Scholar 

  20. Suehara Y, Yazawa Y, Hitachi K, Terakado A. Clear cell sarcoma arising from the chest wall: a case report. J Orthop Sci. 2004;9(2):171–4.

    Article  PubMed  Google Scholar 

  21. Yokoyama R, Mukai K, Hirota T, Beppu Y, Fukuma H. Primary malignant melanoma (clear cell sarcoma) of bone: report of a case arising in the ulna. Cancer. 1996;77(12):2471–5.

    Article  PubMed  CAS  Google Scholar 

  22. Brekke MK, Northcote K, Temple WE. Clear cell sarcoma in the first metatarsal. An unusual case. J Am Podiatr Med Assoc. 1998;88(9):457–61.

    PubMed  CAS  Google Scholar 

  23. Gelczer RK, Wenger DE, Wold LE. Primary clear cell sarcoma of bone: a unique site of origin. Skeletal Radiol. 1999;28(4):240–3.

    Article  PubMed  CAS  Google Scholar 

  24. Inaoka T, Takahashi K, Tandai S, et al. Primary clear cell sarcoma (malignant melanoma) in the right radius. Skeletal Radiol. 2003;32(10):594–7.

    Article  PubMed  Google Scholar 

  25. Hersekli MA, Ozkoc G, Bircan S, et al. Primary clear cell sarcoma of rib. Skeletal Radiol. 2005;34(3):167–70.

    Article  PubMed  Google Scholar 

  26. Rocco G, de Chiara AR, Fazioli F, et al. Primary giant clear cell sarcoma (soft tissue malignant melanoma) of the sternum. Ann Thorac Surg. 2009;87(6):1927–8.

    Article  PubMed  Google Scholar 

  27. Ferris JD, Bloom PA, Goddard PR, Collins C. Quantification ofmelanin and iron content in uveal malignant melanomas and correlation with magnetic resonance image. Br J Ophthalmol. 1993;77:297–301.

    Article  PubMed  CAS  Google Scholar 

  28. Dim DC, Cooley LD, Miranda RN. Clear cell sarcoma of tendons and aponeuroses: a review. Arch Pathol Lab Med. 2007;131(1):152–6.

    PubMed  CAS  Google Scholar 

  29. Jones RL, Constantinidou A, Thway K, et al. Chemotherapy in clear cell sarcoma. Med Oncol, 2010.

  30. Hisaoka M, Ishida T, Kuo TT, et al. Clear cell sarcoma of soft tissue: a clinicopathologic, immunohistochemical, and molecular analysis of 33 cases. Am J Surg Pathol. 2008;32(3):452–60.

    Article  PubMed  Google Scholar 

  31. Chung EB. E.F. Clear-cell sarcorma of tendons and aponeuroses: further observation. Lab Invest. 1978;38:338.

    Google Scholar 

  32. Lyle PL, Amato CM, Fitzpatrick JE, Robinson WA. Gastrointestinal melanoma or clear cell sarcoma? Molecular evaluation of 7 cases previously diagnosed as malignant melanoma. Am J Surg Pathol. 2008;32(6):858–66.

    Article  PubMed  Google Scholar 

  33. Curry CV, Dishop MK, Hicks MJ, Naeem R, Reed JA, Lopez-Terrada DH. Clear cell sarcoma of soft tissue: diagnostic utility of fluorescence in situ hybridization and reverse transcriptase polymerase chain reaction. J Cutan Pathol. 2008;35(4):411–7.

    Article  PubMed  Google Scholar 

  34. Farrokh D, Fransen P, Faverly D. MR findings of a primary intramedullary malignant melanoma: case report and literature review. AJNR Am J Neuroradiol. 2001;22:1864–6.

    PubMed  CAS  Google Scholar 

  35. Sealey RC. Radicals in melanin biochemistry. Methods Enzymol. 1984;105:479–83.

    Article  Google Scholar 

  36. Gerber S, Ollivier L, Leclere J, et al. Imaging of sacral tumours. Skeletal Radiol. 2008;37(4):277–89.

    Article  PubMed  CAS  Google Scholar 

  37. Speleman F, Sciot R. Clear cell sarcoma of soft tissue. World Health Organization classification of tumours. Pathology and genetics of tumours of soft tissue and bone. Lyon: IARC Press; 2002. p. 211–2.

    Google Scholar 

  38. Lucas DR, Nascimento AG, Sim FH. Clear cell sarcoma of soft tissues. Mayo Clinic experience with 35 cases. Am J Surg Pathol. 1992;16(12):1197–204.

    Article  PubMed  CAS  Google Scholar 

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Acknowledgements

This study was supported by funding of the Science and Technology Commission of Shanghai Municipality (No. 10ZR1427800).

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Correspondence to Yuhui Shen.

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Zhang, W., Shen, Y., Wan, R. et al. Primary clear cell sarcoma of the sacrum: a case report. Skeletal Radiol 40, 633–639 (2011). https://doi.org/10.1007/s00256-010-1077-z

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  • DOI: https://doi.org/10.1007/s00256-010-1077-z

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