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Childhood Rhabdomyosarcoma with Mixed Embryonal and Alveolar Features

MedGen UID:
124634
•Concept ID:
C0279615
•
Neoplastic Process
Synonyms: Childhood Mixed Alveolar Rhabdomyosarcoma; Childhood Mixed Cell Type Rhabdomyosarcoma; Childhood Mixed Type Rhabdomyosarcoma; Mixed Childhood Rhabdomyosarcoma; Pediatric Mixed Cell Type Rhabdomyosarcoma; Pediatric Mixed Rhabdomyosarcoma; Pediatric Mixed Type Rhabdomyosarcoma

Definition

A mixed rhabdomyosarcoma that occurs in children. The neoplasm is composed of embryonic and alveolar components. It is characterized by the presence of spindle cells with myoblastic differentiation and a myxoid and a fibrous stroma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChildhood Rhabdomyosarcoma with Mixed Embryonal and Alveolar Features

Recent clinical studies

Etiology

Paratesticular alveolar rhabdomyosarcomas do not harbor typical translocations: a distinct entity with favorable prognosis?
Dantonello TM, Vokuhl C, Scheer M, Sparber-Sauer M, Stegmaier S, Seitz G, Scheithauer H, Faber J, Veit-Friedrich I, Kaatsch P, Bielack SS, Klingebiel T, Koscielniak E; Cooperative Weichteilsarkom Studiengruppe (CWS)
Virchows Arch 2018 Mar;472(3):441-449. Epub 2018 Feb 21 doi: 10.1007/s00428-018-2311-7. PMID: 29468306
Expression of WT1 in pediatric small cell tumors: report of two cases with a possible mesothelial origin.
Thorner P, Squire J, Plavsic N, Jong R, Greenberg M, Zielenska M
Pediatr Dev Pathol 1999 Jan-Feb;2(1):33-41. doi: 10.1007/s100249900087. PMID: 9841704

Diagnosis

Paratesticular alveolar rhabdomyosarcomas do not harbor typical translocations: a distinct entity with favorable prognosis?
Dantonello TM, Vokuhl C, Scheer M, Sparber-Sauer M, Stegmaier S, Seitz G, Scheithauer H, Faber J, Veit-Friedrich I, Kaatsch P, Bielack SS, Klingebiel T, Koscielniak E; Cooperative Weichteilsarkom Studiengruppe (CWS)
Virchows Arch 2018 Mar;472(3):441-449. Epub 2018 Feb 21 doi: 10.1007/s00428-018-2311-7. PMID: 29468306

Prognosis

Paratesticular alveolar rhabdomyosarcomas do not harbor typical translocations: a distinct entity with favorable prognosis?
Dantonello TM, Vokuhl C, Scheer M, Sparber-Sauer M, Stegmaier S, Seitz G, Scheithauer H, Faber J, Veit-Friedrich I, Kaatsch P, Bielack SS, Klingebiel T, Koscielniak E; Cooperative Weichteilsarkom Studiengruppe (CWS)
Virchows Arch 2018 Mar;472(3):441-449. Epub 2018 Feb 21 doi: 10.1007/s00428-018-2311-7. PMID: 29468306

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