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Vol 14, No 6 (2018)
Review paper
Published online: 2019-03-15

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Clear cell sarcoma

Anna M. Czarnecka12, Paweł Sobczuk13, Marcin Zdzienicki1, Mateusz Spałek4, Monika Dudzisz-Śledź1, Piotr Rutkowski1
DOI: 10.5603/OCP.2018.0049
Oncol Clin Pract 2018;14(6):354-363.

Abstract

Clear cell sarcoma (CCS), also referred as to melanoma of soft tissues, is a rare malignant tumour of soft tissues. This tumour harbors the characteristic features of soft tissue sarcoma (STS) and is a slowly growing, painless tumour, which then acquires an aggressive course. CCS is characterized by a translocation t(12; 22)(q13; q12), which in addition to the diagnostic implications may be important for targeted treatment in the future. CCS occurs mainly on the limbs, most often shin (in feet and ankle area) in the tendons and aponeurosis, often at a young age. CCS is characterized by high potential to develop metastases in regional lymph nodes (about 30% of cases). In the diagnostic process one should consider performing a sentinel node biopsy with possible subsequent radical lymphadenectomy in the case of metastases detection. Treatment of localized disease is limited to radical local excision with complementary radiotherapy. Due to the resistance to classical chemotherapy and the presence of characteristic molecular abnormalities, trials of molecular targeted therapies in this group of cancers are ongoing. In clinical trials, MET inhibitors, tyrosine kinase inhibitors (TKI) — sunitinib and pazopanib were evaluated. CCS was also one of the subtypes of tumours evaluated in the CREATE clinical trial with crizotinib.

Keywords: clear cell sarcomacrizotinibsentinel node biopsysarcomatranslocation

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