Pediatric Tumors: Rhabdomyosarcoma

2017 ◽  
Author(s):  
John Groundland ◽  
Sara Shaw ◽  
R L Randall

Rhabdomyosarcoma (RMS) is a group of soft tissue sarcomas that share a common feature of primitive skeletal muscle differentiation. Three main subtypes have been characterized: embryonal, alveolar, and pleomorphic. Presentation and prognosis of RMS are highly variable, depending on anatomic location, subtype, and risk stratification. Currently, treatment centers on systemic control with cytotoxic chemotherapy and local control with surgery and/or therapeutic radiation. This review contains 3 figures, 10 tables and 50 references  Key words: alveolar rhabdomyosarcoma, embryonal rhabdomyosarcoma, pediatric soft tissue sarcoma, pleomorphic rhabdomyosarcoma, rhabdomyosarcona, t(2;13), t(1;13)

2017 ◽  
Author(s):  
John Groundland ◽  
Sara Shaw ◽  
R L Randall

Rhabdomyosarcoma (RMS) is a group of soft tissue sarcomas that share a common feature of primitive skeletal muscle differentiation. Three main subtypes have been characterized: embryonal, alveolar, and pleomorphic. Presentation and prognosis of RMS are highly variable, depending on anatomic location, subtype, and risk stratification. Currently, treatment centers on systemic control with cytotoxic chemotherapy and local control with surgery and/or therapeutic radiation. This review contains 3 figures, 10 tables and 50 references  Key words: alveolar rhabdomyosarcoma, embryonal rhabdomyosarcoma, pediatric soft tissue sarcoma, pleomorphic rhabdomyosarcoma, rhabdomyosarcona, t(2;13), t(1;13)


2015 ◽  
Vol 2015 ◽  
pp. 1-5
Author(s):  
Dana Balitzer ◽  
Timothy H. McCalmont ◽  
Andrew E. Horvai

We describe a 16-year-old boy with rhabdomyosarcoma, consistent with embryonal subtype, of the lower extremity who received systemic neoadjuvant chemotherapy and subsequent excision. Microscopic sections of the postchemotherapy excision demonstrated diffuse, prominent, and immature adipocyte-like differentiation, in addition to skeletal muscle differentiation. Adipocyte-like differentiation was confirmed by a combination of positive Oil Red O and adipophilin immunohistochemical staining. To our knowledge, this represents the first report of an unusual phenomenon of differentiation of a soft tissue rhabdomyosarcoma into adipocyte-like cells after chemotherapy.


2017 ◽  
Author(s):  
John Groundland ◽  
Sara Shaw

Extraskeletal Ewing sarcoma is a rare clinical entity under the umbrella of the Ewing sarcoma family of tumors. The pathogenesis of the tumor has yet to be fully described, but a gene rearrangement and the resultant fusion protein characterize the molecular basis of the disease. Current therapy centers on cytotoxic chemotherapy and local control, either through surgical resection or therapeutic radiation. Survival data specific to extraskeletal Ewing sarcoma are limited due to the rare nature and varied presentation of the disease but parallel those of skeletally based Ewing sarcoma. This review contains 14 figures, 5 tables and 50 references.  Key words: CD 99, Ewing sarcoma, EWS-FLI1, extraskeletal Ewing sarcoma, pediatric soft tissue sarcoma, t(11;22) 


1995 ◽  
Vol 35 (4) ◽  
pp. 278-282 ◽  
Author(s):  
Masaki Chigira ◽  
H. Watanabe ◽  
Akira Hasegawa ◽  
Tetsuya Shinozaki ◽  
Satoru Arita

1995 ◽  
Vol 35 (4) ◽  
pp. 278-282 ◽  
Author(s):  
Hideomi Watanabe ◽  
Akira Hasegawa ◽  
Tetsuya Shinozaki ◽  
Satoru Arita ◽  
Masaki Chigira

2017 ◽  
Author(s):  
John Groundland ◽  
Sara Shaw

Extraskeletal Ewing sarcoma is a rare clinical entity under the umbrella of the Ewing sarcoma family of tumors. The pathogenesis of the tumor has yet to be fully described, but a gene rearrangement and the resultant fusion protein characterize the molecular basis of the disease. Current therapy centers on cytotoxic chemotherapy and local control, either through surgical resection or therapeutic radiation. Survival data specific to extraskeletal Ewing sarcoma are limited due to the rare nature and varied presentation of the disease but parallel those of skeletally based Ewing sarcoma. This review contains 14 figures, 5 tables and 50 references.  Key words: CD 99, Ewing sarcoma, EWS-FLI1, extraskeletal Ewing sarcoma, pediatric soft tissue sarcoma, t(11;22) 


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