Biology and management of clear cell sarcoma: state of the art and future perspectives

Cornillie, Jasmien; Cann, Thomas Van; Woźniak, Agnieszka; Hompes, Daphne; Schöffski, Patrick

doi:10.1080/14737140.2016.1197122

Cited by 40 publications

(49 citation statements)

References 52 publications

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“…A phase I trial of an autologous GM-CSF-secreting tumor cell vaccine in advanced CCS patients demonstrated induction of T cell-mediated delayed-type hypersensitivity reactions to irradiated, autologous tumor cells [2]. Anecdotal reports in CCS patients have included a complete response to perilesional interferon-alpha, stable disease for 6–24 months in 2 patients following ipilimumab, and a partial response to pembrolizumab in a 6-year old [5]. Single modality radiotherapy would likely have limited efficacy in bulky, unresectable CCS [16].…”

Section: Discussionmentioning

confidence: 99%

“…CCS shares striking histological and immunohistochemical similarities with cutaneous melanoma, often containing melanin pigment and expressing melanocyte differentiation antigens including S-100, HMB-45, and Melan-A [5]. Like melanoma and in contrast to most sarcomas, CCS is thought to derive from neural crest, spreads to regional lymph nodes in up to 50% of patients, and is frequently associated with in transit metastases [1, 2, 5]. CCS carries a high risk of hematogeneous dissemination with 5-year overall survival of 50–60% for localized disease and poor response to chemotherapy [1, 5].…”

Section: Introductionmentioning

confidence: 99%

“…Like melanoma and in contrast to most sarcomas, CCS is thought to derive from neural crest, spreads to regional lymph nodes in up to 50% of patients, and is frequently associated with in transit metastases [1, 2, 5]. CCS carries a high risk of hematogeneous dissemination with 5-year overall survival of 50–60% for localized disease and poor response to chemotherapy [1, 5]. Thus, modern authors have classified CCS as a melanoma subtype [4].…”

Section: Introductionmentioning

confidence: 99%

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Complete response of mediastinal clear cell sarcoma to pembrolizumab with radiotherapy

2017

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BackgroundClear cell sarcoma (CCS) is a rare, aggressive soft tissue sarcoma thought to derive from neural crest and characterized by a 12;22 translocation. The resulting fusion protein directly activates expression of the melanocyte master transcription factor and drives the same down-stream pathways in CCS and melanoma leading to significant clinical parallels between these malignancies. Striking success of immune checkpoint blockade in melanoma has promoted interest in immunotherapy of CCS.Case presentationWe report the first complete clinical response of a bulky chest wall recurrence of mediastinal CCS in a young woman to anti-PD1 checkpoint blockade with pembrolizumab combined with standard fractionation radiotherapy to enhance regional control and potentially boost the systemic immune response. The treatment was well tolerated with grade 2 skin toxicity within the range expected with radiation alone. Significant reduction in tumor bulk occurred after only 2 radiation fractions and complete response was achieved at 50 Gray.ConclusionThe complete clinical response observed in our patient suggests synergy between concurrent radiotherapy and PD1 blockade in CCS. This case and the striking parallels between CCS and melanoma indicate the need for prospective trials of immune checkpoint blockade combined with radiotherapy in this rare malignancy.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Complete response of mediastinal clear cell sarcoma to pembrolizumab with radiotherapy

2017

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“…Clear cell sarcoma (CCS) is a rare type comprising approximately 1% of all soft tissue sarcomas [1]. It was first described by Enzinger in 1965 [2], and for many years in the literature it was referred as to clear cell sarcoma of tendons and aponeuroses or malignant melanoma of soft tissue.…”

Section: Epidemiologymentioning

confidence: 99%

“…The grading of CCS, like other soft tissue sarcoma (STS), is based on the Fédération Française des Centres de Lutte Contre le Cancer (FNCLCC) system, which takes into account the degree of cell differentiation, necrosis, and mitotic activity. The CCS is characterized by a low mitotic index and a rare occurrence of necrosis, which is why most cases are classified as 1 or 2 malignancy [1,15].…”

Section: Biology Genetics and Histopathologymentioning

confidence: 99%

Clear cell sarcoma

et al. 2019

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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.

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Multiple metastases of clear cell sarcoma at diagnosis presented in bone marrow aspirate: A case report

Jin

Shang

et al. 2022

Diagnostic Cytopathology

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Clear cell sarcoma (CCS) is a rare and aggressive soft‐tissue sarcoma that arises most commonly in adolescents and young adults of both sexes. CCS presents a diagnostic challenge due to its morphological and immunohistochemical similarity to malignant melanoma. We present a rare and severe case of CCS simultaneously with multiple bone and lymph node metastases at the time of initial diagnosis in a previously healthy 15‐year‐old Chinese man. Detailed information on clinical manifestations, laboratory profiles, histopathological findings and poor outcome were described. The cytomorphology of bone marrow aspirate in CCS in Wright–Giemsa staining smear was first depicted in this case. The diagnostic difficulties of the rare case was also discussed.

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Biology and management of clear cell sarcoma: state of the art and future perspectives

Cited by 40 publications

References 52 publications

Complete response of mediastinal clear cell sarcoma to pembrolizumab with radiotherapy

Complete response of mediastinal clear cell sarcoma to pembrolizumab with radiotherapy

Clear cell sarcoma

Multiple metastases of clear cell sarcoma at diagnosis presented in bone marrow aspirate: A case report

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