scholarly journals EWS-ATF-1 chimeric protein in soft tissue clear cell sarcoma associates with CREB-binding protein and interferes with p53-mediated trans-activation function

Oncogene ◽  
2001 ◽  
Vol 20 (46) ◽  
pp. 6653-6659 ◽  
Author(s):  
Yasuo Fujimura ◽  
Habibur Siddique ◽  
Leo Lee ◽  
Veena N Rao ◽  
E Shyam P Reddy
2018 ◽  
Vol 1 (Supplement) ◽  
pp. 58
Author(s):  
V. Georgeanu ◽  
T. Atasiei ◽  
D. Gartonea ◽  
B. Shazam ◽  
G. Goleşteanu ◽  
...  

Abstract Introduction. Soft Tissue Sarcomas (STS) is a group of rare malignant tumors with mesenchymal tissue origin. At present, over 50 histopathological types with typical chromosome changes are described. Treatment is multidisciplinary, centered on the surgical approach. Method. Between 2014 and 2016, 12 STS cases were treated in our clinic: 3 liposarcomas, 2 synovial sarcomas, 1 angiosarcoma, 2 rhabdomyosarcomas, 2 myxofibrosarcomas, 1 fibroblast sarcoma and 1 clear cell sarcoma. With the exception of angiosarcoma that benefited only from chemotherapy, the other cases were surgically approached, followed by radiotherapy in 7 cases, and chemotherapy in one case. Results. Patient follow-up was for a minimum of 2 years. In 3 cases (fibroblast sarcoma, clear-cell sarcoma, synovial sarcoma), a local recurrence occurred between 3 and 9 months. In 2 cases (fibroblastic sarcoma, synovial sarcoma), pulmonary metastases occurred between 6 and 18 months and the patients died at 8 months and 2 years respectively. The rest of the cases were clinically and imagistically assessed (local MRI, lung CT) at 6 months for at least 2 years and showed no signs of local recurrence or metastasis. Conclusions. The evolution of STS depends on the histological type and to the stage of diagnosis (local extension, degree of differentiation and presence of metastases). Therapeutic approach should be centered on an accurate resection, within the limits of oncological safety even with re-resection. Postoperative radiotherapy is used in most of the cases, and chemotherapy is reserved for special cases with unfavorable local evolution or metastasis.


2019 ◽  
Author(s):  
Alexandra G Lopez-Aguiar ◽  
Kenneth Cardona

Lymphotropic sarcomas encompass a rare subset of soft tissue sarcoma histotypes known for their distinct ability to metastasize to locoregional lymph nodes. Epithelioid and clear cell sarcomas are the predominant lymphotropic subtypes and the focus of this review. The utility of lymph node assessment, whether clinical, radiographic, and/or surgical, in patients with these sarcomas is presented. The role of sentinel lymph node biopsy as well as completion lymphadenectomy in patients with these lymphotropic sarcoma histotypes is discussed. Surgery remains the only potential curative option for these tumors, and advancement in histology-specific chemotherapeutic regimens and biologic therapy options is necessary.  This review contains 6 tables and 50 references. Key words: clear cell sarcoma, completion lymphadenectomy, epithelioid sarcoma, EWS-ATF1, limb-sparing surgery, lymph node metastasis, sentinel lymph node biopsy, soft tissue sarcoma


2010 ◽  
Vol 63 (5) ◽  
pp. 416-423 ◽  
Author(s):  
Kemal Kosemehmetoglu ◽  
Andrew L Folpe

Clear cell sarcoma (CCS) is a rare, distinctive soft tissue neoplasm, typically occurring in the distal extremities of young adult patients. Although CCS shows melanocytic differentiation, it is now clear that it is clinicopathologically and genetically distinct from conventional malignant melanoma. The ‘osteoclast-rich tumour of the gastrointestinal tract with features resembling clear cell sarcoma of soft parts’ is an extraordinarily rare gastrointestinal neoplasm that shares some features of CCS, but differs from it in other ways. The historical, histopathological, ultrastructural, immunohistochemical and genetic aspects of these two tumours are reviewed in this article.


2016 ◽  
Vol 10 (1) ◽  
Author(s):  
Muhammad Abdullah

Clear cell sarcoma is a rare malignant neoplasm usually presenting as soft tissue mass in foot of young adult. Ultrastructural studies reveal neuroectodermal origin with melanocytic differentiation. Thus it is considered as malignant melanoma of soft parts. Though the prognosis is apparently better as compared to the conventional melanoma, local recurrences and metastases are frequently seen. Large tumor size and extent of necrosis are factors for poor prognosis. A case of clear cell sarcoma with classical presentation is reported.


2021 ◽  
Vol 9 (3) ◽  
pp. 292-294
Author(s):  
  Kenza Kandri Rody ◽  
Soumia Faras ◽  
Najat Elaattoul ◽  
Imane Boujguenna ◽  
Hanane Rais ◽  
...  

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