Abstract
Is it a sarcoma or is it a melanoma? Clear cell sarcoma (CCS), also called clear cell sarcoma of tendons and aponeuroses [1, 2], has features of both. CCS represents perhaps 1 % of all sarcomas. Since it starts in soft tissue and usually does not affect skin [3], CCS is anatomically distinct from melanoma. Patients are typically younger, between 15 and 45 years of age, and women are more commonly affected than men. The foot and ankle are common primary locations for this rare sarcoma. Age and site distribution for adult patients are shown in Figs. 18.1 and 18.2.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Enzinger FM. Clear-cell sarcoma of tendons and aponeuroses. An analysis of 21 cases. Cancer. 1965;18:1163–74.
Chung EB, Enzinger FM. Malignant melanoma of soft parts. A reassessment of clear cell Âsarcoma. Am J Surg Pathol. 1983;7(5):405–13.
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.
Bridge JA, Borek DA, Neff JR, et al. Chromosomal abnormalities in clear cell sarcoma. Implications for histogenesis. Am J Clin Pathol. 1990;93(1):26–31.
Antonescu CR, Nafa K, Segal NH, et al. EWS-CREB1: a recurrent variant fusion in clear cell sarcoma–association with gastrointestinal location and absence of melanocytic differentiation. Clin Cancer Res. 2006;12(18):5356–62.
Antonescu CR, Dal Cin P, Nafa K, et al. EWSR1-CREB1 is the predominant gene fusion in angiomatoid fibrous histiocytoma. Genes Chromosomes Cancer. 2007;46(12):1051–60.
Rossi S, Szuhai K, Ijszenga M, et al. EWSR1-CREB1 and EWSR1-ATF1 fusion genes in angiomatoid fibrous histiocytoma. Clin Cancer Res. 2007;13(24):7322–8.
Clark MA, Johnson MB, Thway K, et al. Clear cell sarcoma (melanoma of soft parts): The Royal Marsden hospital experience. Eur J Surg Oncol. 2008;34(7):800–4.
Daigeler A, Kuhnen C, Moritz R, et al. Lymph node metastases in soft tissue sarcomas: a single center analysis of 1,597 patients. Langenbecks Arch Surg. 2009;394(2):321–9.
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.
Jones RL, Constantinidou A, Thway K, et al. Chemotherapy in clear cell sarcoma. Med Oncol. 2011;28(3):859–63.
Goldberg J, Demetri GD, Choy E, et al. Preliminary results from a phase II study of ARQ197 in patients with microphthalmia transcription factor family-associated tumors. J Clin Oncol. 2009;27(15S):Abstr 10502.
Stacchiotti S, Grosso F, Negri T, et al. Tumor response to sunitinib malate observed in clear-cell sarcoma. Ann Oncol. 2010;21(5):1130–1.
Toretsky JA, Kalebic T, Blakesley V, et al. The insulin-like growth factor-I receptor is required for EWS/FLI-1 transformation of fibroblasts. J Biol Chem. 1997;272(49):30822–7.
Blazer 3rd DG, Lazar AJ, Xing Y, et al. Clinical outcomes of molecularly confirmed clear cell sarcoma from a single institution and in comparison with data from the surveillance, epidemiology, and end results registry. Cancer. 2009;115(13):2971–9.
Ipach I, Mittag F, Kopp HG, et al. Clear-cell sarcoma of the soft tissue – a rare diagnosis with a fatal outcome. Eur J Cancer Care (Engl). 2012;21(3):412–20.
Author information
Authors and Affiliations
Rights and permissions
Copyright information
© 2013 Springer Science+Business Media New York
About this chapter
Cite this chapter
Brennan, M.F., Antonescu, C.R., Maki, R.G. (2013). Clear Cell Sarcoma/Melanoma of Soft Parts. In: Management of Soft Tissue Sarcoma. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-5004-7_18
Download citation
DOI: https://doi.org/10.1007/978-1-4614-5004-7_18
Published:
Publisher Name: Springer, New York, NY
Print ISBN: 978-1-4614-5003-0
Online ISBN: 978-1-4614-5004-7
eBook Packages: MedicineMedicine (R0)