Clear cell sarcoma

Czarnecka, Anna M.; Sobczuk, Paweł; Zdzienicki, Marcin; Spałek, Mateusz; Dudzisz-Śledź, Monika; Rutkowski, Piotr

doi:10.5603/ocp.2018.0049

Cited by 4 publications

(3 citation statements)

References 68 publications

(161 reference statements)

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“…The errors that accompanied this patient were inappropriate chemotherapy, as CCS is chemo resistant [26,27] and the patient could have undergone sentinel node biopsy if the diagnosis was clear earlier [28,29].…”

Section: Discussionmentioning

confidence: 99%

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A rare case of clear cell sarcoma of the foot with a cascade of pathological misdiagnosis – the importance of expert sarcoma pathology

Anand¹,

Gupta²,

Barwad³

et al. 2022

ecancer

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Sarcoma pathology discrepancy is well known owing to the extremely heterogenous and rare nature of this tumour. Through this case, we want to highlight the difficulty that a patient has to undergo in a case of misdiagnosis. A 20-year-old male presented with swelling in the right foot for 4 months, which was initially diagnosed as alveolar rhabdomyosarcoma, subsequently as synovial sarcoma and finally as Ewing's sarcoma (based upon positive Ewing Sarcoma Breakpoint Region 1 (EWSR1) by fluorescence in situ hybridisation and he underwent neoadjuvant chemotherapy and surgical excision with grafting before he presented to our institute, where the pathologists reviewed the biopsy slides, which were positive for HMB45 and negative for Melan-A suggestive of clear cell sarcoma. The next-generation sequencing suggested EWSR1-ATF1 fusion, which again reinforced the diagnosis. This case throws light on the importance of expert pathology and interpreting molecular results in the right context.

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Section: Discussionmentioning

confidence: 99%

“…The sensitivity to chemotherapy and immunotherapy in this subset has rarely been demonstrated [27,30]. However, early diagnosis, surgery, sentinel node biopsy and radiation when required are the current treatment of choice for localised CCS [27].…”

Section: Discussionmentioning

confidence: 99%

A rare case of clear cell sarcoma of the foot with a cascade of pathological misdiagnosis – the importance of expert sarcoma pathology

Anand¹,

Gupta²,

Barwad³

et al. 2022

ecancer

View full text Add to dashboard Cite

show abstract

“…However, the most effective but toxic chemotherapy regimens used in STS may be not suitable for elderly patients or those with significant comorbidities [ 5 ]. Moreover, low-grade STS and selected histopathological STS subtypes are considered chemoresistant [ 6 , 7 , 8 , 9 , 10 , 11 , 12 ]. Another approach could be the use of high-dose definitive RT.…”

Section: Introductionmentioning

confidence: 99%

The Feasibility Study of Hypofractionated Radiotherapy with Regional Hyperthermia in Soft Tissue Sarcomas

Spałek

Borkowska

Telejko

et al. 2021

Cancers

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Introduction: Management of marginally resectable or unresectable soft tissue sarcomas (STS) in patients who are not candidates for neoadjuvant chemotherapy due to chemoresistant pathology or contraindications remains a challenge. Therefore, in these indications, we aimed to investigate a feasibility of 10x 3.25 Gy radiotherapy combined with regional hyperthermia (HT) that could be followed by surgery or 4x4 Gy radiotherapy with HT. Materials and methods: We recruited patients with locally advanced marginally resectable or unresectable STS who (1) presented chemoresistant STS subtype, or (2) progressed after neoadjuvant chemotherapy, or (3) were unfit for chemotherapy. The primary endpoint was the feasibility of the proposed regimen. Results: Thirty patients were enrolled. All patients received the first part of the treatment, namely radiotherapy with HT. Among them, 14 received the second part of radiotherapy with HT whereas 13 patients underwent surgery. Three patients did not complete the treatment protocol. The feasibility criteria were fulfilled in 90% of patients. Two patients developed distant metastases. One patient died due to distant progression. One patient developed rapid local recurrence after surgery. Conclusions: Hypofractionated radiotherapy with HT is a feasible treatment for marginally resectable or unresectable STS in patients who are not candidates for chemotherapy. Results of this clinical trial support the further validation of RT and HT combinations in STS.

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The current management of clear cell sarcoma

Ikuta

Nishida

Imagama

et al. 2023

Japanese Journal of Clinical Oncology

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Clear cell sarcoma (CCS) is a rare melanocytic soft tissue sarcoma with a high propensity for lymphatic metastasis and poor prognosis. It is characterized by the translocation of t (12;22), resulting in the rearrangement of the EWSR1 gene and overexpression of MET. Despite improvements in the diagnosis and treatment of soft tissue sarcomas, the management of CCSs remains challenging owing to their rarity, unique biological behaviour and limited understanding of their molecular pathogenesis. The standard treatment for localized CCSs is surgical excision with negative margins. However, there is an ongoing debate regarding the role of adjuvant chemotherapy, radiotherapy and lymphadenectomy in the management of this disease. CCSs are usually resistant to conventional chemotherapy. Targeted therapies, such as sunitinib and MET inhibitors, may provide promising results. Immunotherapy, particularly immune checkpoint inhibitors, is currently under investigation as a potential treatment option for CCSs. Further research is needed to better understand the biology of CCSs and develop effective therapeutic strategies. The purpose of this review is to provide a comprehensive overview of current knowledge and advances in the diagnosis and treatment of CCSs.

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Clear cell sarcoma

Cited by 4 publications

References 68 publications

A rare case of clear cell sarcoma of the foot with a cascade of pathological misdiagnosis – the importance of expert sarcoma pathology

A rare case of clear cell sarcoma of the foot with a cascade of pathological misdiagnosis – the importance of expert sarcoma pathology

The Feasibility Study of Hypofractionated Radiotherapy with Regional Hyperthermia in Soft Tissue Sarcomas

The current management of clear cell sarcoma

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