top of page
CCS Reviews
Clear cell sarcoma-A review
Publication Date:
September 6, 2018
Abstract
Clear cell sarcoma (CCS) previously known as malignant melanoma (MM) of the soft tissue, although, similar in morphology to MM, contemporary histopathologic and cytogenetic techniques have made this diagnosis obsolete, as it is now possible to distinguish between CCS and MM. CCS is often diagnosed in young adults with median age of 25 years. Overall mortality is generally poor, and the 5-year survival is between 40 and 60%. Hence, early diagnosis and radical surgery are key in the treatment of this extremely rare malignancy of the soft tissue comprising only about 1% of all sarcomas. This article present an overview of this rare malignancy.
Authors
Rami Mossad Ibrahim a,∗, Signe Steenstrup Jensen b, Jacob Juel c
Clear cell sarcoma of the gastrointestinal tract and malignant gastrointestinal neuroectodermal tumour: distinct or related entities? A review
Publication Date:
June 30, 2018
Abstract
Clear cell sarcoma is an uncommon sarcoma which rarely occurs as a primary tumour in the gastrointestinal tract (CCS-GIT). It shares common molecular genetic abnormalities with the more recently described entity, malignant gastrointestinal neuroectodermal tumour (GNET) but is distinguished by its morphological and immunohistochemical findings. The exact nosological relationship between these tumours continues to be debated. In this review, we present two cases of these rare neoplasms from our files and perform a statistical comparison of all published cases to determine if significant differences exist in their clinicopathological features and biological behaviour. Thirteen cases of CCS-GIT and 58 of GNET were included. CCS-GIT occurred more commonly in males (84.6% vs 46.6%, p = 0.01) and in an older age group (median 57 vs 33 years, p < 0.01). There was no significant difference in their location in the gastrointestinal tract, median tumour size and proportion of cases with an EWSR1-ATF1 vs EWSR1-CREB1 fusion. Median survival for CCS-GIT was 13.5 months and for GNET, 9.5 months (p = 0.78). There was no significant difference in the Kaplan-Meier survival curves for either time to first metastasis (p = 0.88) or overall survival (p = 0.18), including after controlling for tumour size using regression models. Our analysis confirms that aside from morphological variations between these tumours, they also exhibit epidemiological and clinical differences. Despite the prevalent perception that GNET is associated with a more aggressive clinical course, our findings indicate that there is no significant difference in their biological behaviour, although both clearly share a bleak prognosis. Further experience is awaited to determine optimal treatment strategies and whether CCS-GIT and GNET would differ in their response to various therapies.
Authors
Celia Green 1, Dominic V Spagnolo 1, Peter D Robbins 1, Soraya Fermoyle 2, Daniel D Wong 3
Biology and management of clear cell sarcoma: state of the art and future perspectives
Publication Date:
June 20, 2016
Abstract
Introduction: Clear cell sarcoma (CCS) is an aggressive tumor, typically developing in tendons or aponeuroses. The outcome of this orphan disease is poor, with 5-year and 10-year survival rates of localized CCS around 60-70% and 40-50%. Once the disease has metastasized, it is usually fatal due to its chemotherapy-resistant nature. Systemic treatment options are poorly standardized and the use of chemotherapy is based on weak scientific evidence.
Areas covered: In this review, we systematically discuss the current scientific evidence for the systemic treatment of CCS, including tyrosine kinase inhibitors, immunotherapy and MET inhibitors. Expert commentary: Recent insights in the biology of CCS have identified new potential therapeutic targets, which should be tested in prospective clinical trials. Whenever possible, patients with metastatic CCS should be included in clinical trials with good biological rationale. Innovative trial methodology and new regulatory mechanisms are required to provide patients with uncommon cancers with active drugs.
Authors
Jasmien Cornillie 1, Thomas van Cann 1, Agnieszka Wozniak 1, Daphne Hompes 2, Patrick Schöffski 1
Clear cell sarcoma-like tumor of the gastrointestinal tract: an evolving entity
Publication Date:
March 1, 2015
Abstract
Clear cell sarcoma-like tumor of the gastrointestinal tract (CCSLGT) is a rare malignant neoplasm that occurs in the wall of the small bowel, stomach, or large bowel, predominantly in young adults. It is an aggressive neoplasm that frequently presents with metastatic disease and has a high mortality rate. Histologically, it is usually composed of medium-sized primitive ovoid or epithelioid cells with pale or clear cytoplasm that are arranged in sheets or in papillary or alveolar architectures. Clear cell sarcoma-like tumor of the gastrointestinal tract is positive for S100 protein, invariably negative for melanocyte-specific markers and is often also positive for neuroendocrine markers. The etiology of CCSLGT is unknown, but many studies have shown associations with EWSR1-CREB1 gene fusions and, less frequently, with EWSR1-ATF1 fusions. Here, we discuss the current status of CCSLGT, including histologic, immunophenotypic, and molecular findings.
Authors
Jayson Wang 1, Khin Thway
Chemotherapy in clear cell sarcoma
Publication Date:
April 14, 2010
Abstract
Clear cell sarcoma is a rare translocation-related sarcoma. There have been few studies documenting the response rate and progression-free survival in clear cell sarcoma patients treated with palliative chemotherapy. The prospectively maintained databases of two referral centres were searched to identify clear cell sarcoma patients treated with chemotherapy. Twenty-four patients were treated with palliative first-line chemotherapy with a median age of 30 years at diagnosis. There were 18 men and 6 women. One (4%) achieved a partial response and 9 (38%) had stable disease. Fourteen patients (58%) progressed on therapy. The median progression-free survival was 11 weeks (95% CI, 3–20 weeks). The median overall survival from commencing first-line chemotherapy was 39 weeks (95% CI, 34–45 weeks). Second-line chemotherapy was administered to 12 patients, 11 (92%) of these progressed and one (8%) had stable disease. Of the 5 patients treated with third-line chemotherapy, 4 (80%) progressed and one (20%) had stable disease. One patient received fourth-line chemotherapy and maintained stable disease for 4 months. Conventional chemotherapy has minimal activity in clear cell sarcoma as documented by the response rate of 4% and median progression-free survival of 11 weeks in this retrospective series. These data provide a reference for response and outcome in the assessment of novel agents in this histological subtype.
Authors
Robin L. Jones • Anastasia Constantinidou • Khin Thway • Sue Ashley • Michelle Scurr • Omar Al-Muderis • Cyril Fisher • Cristina R. Antonescu • David R. D’Adamo • Mary Louise Keohan • Robert G. Maki • Ian R. Judson
Get updates on CCSF happenings
bottom of page