Clear cell sarcoma-A review

Ibrahim, Rami Mossad; Jensen, Signe Steenstrup; Juel, Jacob

doi:10.1016/j.jor.2018.08.039

Cited by 33 publications

(16 citation statements)

References 30 publications

(50 reference statements)

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“…For example, Chung and Enzinger ( 4 ) reported that 50 out of 115 patients had died from metastatic tumors in 1983. Despite progress in the different treatments available, the prognosis of patients with CCS is still poor, as 30% of patients have metastases at the time of diagnosis ( 5 ). Lymphatic metastasis is rare in other types of malignant soft tissue tumors; however, is commonly detected in CCS ( 4 , 6 ).…”

Section: Introductionmentioning

confidence: 99%

An obstructive role of NK cells on metastatic growth of clear‑cell sarcoma cells in a xenoplant murine model

et al. 2020

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Clear cell sarcoma (CCS) affects the deep soft tissues in young adults and is known to have high rates of metastasis, including lymphatic metastasis. In our previous study an xenoplant model of CCS was established, which exhibited local tumor growth, lymphatic metastasis, and distant metastasis in SCID-Beige mice. In the current study, the role of NK cells during metastasis in the same xenoplant murine model was investigated. Injection of murine or human NK cells significantly suppressed the metastasis of HS-MM CCS cells in SCID-Beige mice. Notably, reverse transcription-quantitative PCR analysis demonstrated that injection of NK cells did not alter the mRNA expression levels of ERSR1-ATF1 , which is specifically transcribed in CCS, in the buffy coat of circulating blood cells of HS-MM-xenoplanted SCID-Beige mice. BALB/c nude mice xenoplanted with HS-MM cells exhibited local growth without evident metastasis, whereas inoculation with the anti-asialo-GM1 antibody, which has previously been found to abolish NK-cell activity, resulted in metastasis of HS-MM cells in BALB/c nude mice. The injection of the anti-CD96 antibody, which increases the cytotoxicity of NK cells, significantly suppressed the metastasis of HS-MM cells in SCID-Beige mice. These results indicated that NK cells impaired the metastatic tumor microenvironments in the present mice xenoplant model.

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Section: Introductionmentioning

confidence: 99%

An obstructive role of NK cells on metastatic growth of clear‑cell sarcoma cells in a xenoplant murine model

et al. 2020

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“…Common cancer types used for comparisons are shown in lavender: Lung (L), Breast (B), Colon (C), and Prostate (P). Gray zone lymphoma (#7) -lymph node; GIST (#12) -Stomach; 13 Follicular lymphoma (#13) -lymph node; Primary mediastinal lymphoma (#19) -between the 2 lungs in the mediastinum [4,[12][13][14][15][16][17].…”

Section: Somatic and Pathogenic Germline Variants Detected In The Rarmentioning

confidence: 99%

Rare Tumor-Normal Matched Whole Exome Sequencing Identifies Novel Genomic Pathogenic Germline and Somatic Aberrations

Sprissler

Perkins

Johnstone

et al. 2020

Cancers

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Whole exome sequencing (WES) of matched tumor-normal pairs in rare tumors has the potential to identify genome-wide mutations and copy number alterations (CNAs). We evaluated 27 rare cancer patients with tumor-normal matching by WES and tumor-only next generation sequencing (NGS) as a comparator. Our goal was to: 1) identify known and novel variants and CNAs in rare cancers with comparison to common cancers; 2) examine differences between germline and somatic variants and how that functionally impacts rare tumors; 3) detect and characterize alleles in biologically relevant genes-pathways that may be of clinical importance but not represented in classical cancer genes. We identified 3343 germline single nucleotide variants (SNVs) and small indel variants—1670 in oncogenes and 1673 in tumor suppressor genes—generating an average of 124 germline variants/case. The number of somatic SNVs and small indels detected in all cases was 523:306 in oncogenes and 217 in tumor suppressor genes. Of the germline variants, six were identified to be pathogenic or likely pathogenic. In the 27 analyzed rare cancer cases, CNAs are variable depending on tumor type, germline pathogenic variants are more common. Cell fate pathway mutations (e.g., Hippo, Notch, Wnt) dominate pathogenesis and double hit (mutation + CNV) represent ~18% cases.

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“…Clear cell sarcoma (CCS) is an extremely rare sarcoma, accounting for approximately 1% of all soft tissue sarcomas (STSs), with approximately 400 new cases in China each year. 1 , 2 CCS was originally named soft tissue melanoma because of its immunohistochemical, ultrastructural, and genomic predisposition to melanocyte differentiation. 3 , 4 This designation is now obsolete because pathologists can now accurately identify clear cell sarcomas.…”

Section: Introductionmentioning

confidence: 99%

“… 3 , 4 This designation is now obsolete because pathologists can now accurately identify clear cell sarcomas. 1 , 5 CCS may originate anywhere in the body but more often in the extremities. The initial symptom is usually a slowly increasing painless mass with lymph node metastasis in the early to middle stages.…”

Section: Introductionmentioning

confidence: 99%

Clinical Experience with Apatinib and Camrelizumab in Advance Clear Cell Sarcoma: A Retrospective Study

Wang¹,

Gao²,

Yang³

et al. 2021

CMAR

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Purpose Advanced clear cell sarcoma (CCS) is a rare subtype of sarcoma with few effective treatments. Evidence shows that apatinib is efficacious and safe for CCS. This study aimed to assess the safety and efficacy of apatinib and/or camrelizumab (a PD-1 inhibitor) in treating advanced CCS. Methods We retrospectively reviewed 12 patients with advanced CCS who received apatinib and/or camrelizumab therapy between November 2018 and July 2021. Standard descriptive statistics were employed for continuous variables and categorical variables (number and percentage). Results Of the 12 CCS patients, 3 had a partial response (PR), and 4 had stable disease (SD). Among the 5 patients treated with apatinib monotherapy, 1 PR and 2 SD were found, and the addition or replacement of camrelizumab after progressive disease (PD) did not work. In the 4 patients who received apatinib plus camrelizumab combination therapy, 1 PR and 1 SD were found. All 3 patients who received camrelizumab first had PD, and 1 PR and 1 SD were found after adding apatinib. Grade 3 or 4 adverse events were significantly more common in the apatinib plus camrelizumab combination therapy than in the apatinib or camrelizumab monotherapy, and these included increased aspartate aminotransferase and increased alanine aminotransferase levels. Conclusion Apatinib has promising effectiveness for CCS. Camrelizumab efficacy for the treatment of clear cell sarcoma is inconclusive. The efficacy of apatinib and PD-1 inhibitors in CCS need to be further investigated in prospective clinical trials.

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Clear cell sarcoma-A review

Cited by 33 publications

References 30 publications

An obstructive role of NK cells on metastatic growth of clear‑cell sarcoma cells in a xenoplant murine model

An obstructive role of NK cells on metastatic growth of clear‑cell sarcoma cells in a xenoplant murine model

Rare Tumor-Normal Matched Whole Exome Sequencing Identifies Novel Genomic Pathogenic Germline and Somatic Aberrations

Clinical Experience with Apatinib and Camrelizumab in Advance Clear Cell Sarcoma: A Retrospective Study

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