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Rhabdomyosarcoma with mixed embryonal and alveolar features

MedGen UID:
313444
Concept ID:
C1709053
Neoplastic Process
Synonyms: Mixed Alveolar Rhabdomyosarcoma; mixed alveolar rhabdomyosarcoma; mixed embryonal rhabdomyosarcoma and alveolar rhabdomyosarcoma; Mixed Type Alveolar Rhabdomyosarcoma; mixed type alveolar rhabdomyosarcoma; mixed type rhabdomyosarcoma; Rhabdomyosarcoma with Mixed Embryonal and Alveolar Features; rhabdomyosarcoma with mixed embryonal and alveolar features
 
Monarch Initiative: MONDO:0002863

Definition

A rhabdomyosarcoma composed of embryonic and alveolar components. It is characterized by the presence of spindle cells with myoblastic differentiation, a myxoid stroma, and fibrous septa. These tumors were previously considered variants of alveolar rhabdomyosarcoma. The lack of PAX3-FOXO1 fusions in most of these tumors suggests that are biologically and clinically related to embryonal rhabdomyosarcoma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRhabdomyosarcoma with mixed embryonal and alveolar features

Recent clinical studies

Etiology

Liu JH, Qi M, Huang WH, Sha Y, Zhang F
BMC Med Imaging 2023 Jul 28;23(1):98. doi: 10.1186/s12880-023-01062-x. PMID: 37507673Free PMC Article
Bandino JP, Purvis CG, Shaffer BR, Gad A, Elston DM
Am J Dermatopathol 2018 Nov;40(11):815-818. doi: 10.1097/DAD.0000000000001232. PMID: 30045067
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
La Starza R, Nofrini V, Pierini T, Pierini V, Zin A, Bisogno G, Cerri C, Caniglia M, Sidoni A, Ludwig K, Mecucci C
Pediatr Blood Cancer 2015 Dec;62(12):2238-41. Epub 2015 Jul 14 doi: 10.1002/pbc.25664. PMID: 26179572
Chiles MC, Parham DM, Qualman SJ, Teot LA, Bridge JA, Ullrich F, Barr FG, Meyer WH; Soft Tissue Sarcoma Committee of the Children's Oncology Group
Pediatr Dev Pathol 2004 Nov-Dec;7(6):583-94. Epub 2004 Nov 17 doi: 10.1007/s10024-004-5058-x. PMID: 15630526

Diagnosis

Liu JH, Qi M, Huang WH, Sha Y, Zhang F
BMC Med Imaging 2023 Jul 28;23(1):98. doi: 10.1186/s12880-023-01062-x. PMID: 37507673Free PMC Article
Bandino JP, Purvis CG, Shaffer BR, Gad A, Elston DM
Am J Dermatopathol 2018 Nov;40(11):815-818. doi: 10.1097/DAD.0000000000001232. PMID: 30045067
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
Kunze E, Theuring F, Krüger G
Pathol Res Pract 1994 Apr;190(4):311-32. doi: 10.1016/S0344-0338(11)80404-8. PMID: 8078801
Kodet R, Fajstavr J, Kabelka Z, Koutecky J, Eckschlager T, Newton WA Jr
Cancer 1991 Jun 1;67(11):2907-13. doi: 10.1002/1097-0142(19910601)67:11<2907::aid-cncr2820671133>3.0.co;2-b. PMID: 2025857

Therapy

Bandino JP, Purvis CG, Shaffer BR, Gad A, Elston DM
Am J Dermatopathol 2018 Nov;40(11):815-818. doi: 10.1097/DAD.0000000000001232. PMID: 30045067

Prognosis

Liu JH, Qi M, Huang WH, Sha Y, Zhang F
BMC Med Imaging 2023 Jul 28;23(1):98. doi: 10.1186/s12880-023-01062-x. PMID: 37507673Free PMC Article
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
La Starza R, Nofrini V, Pierini T, Pierini V, Zin A, Bisogno G, Cerri C, Caniglia M, Sidoni A, Ludwig K, Mecucci C
Pediatr Blood Cancer 2015 Dec;62(12):2238-41. Epub 2015 Jul 14 doi: 10.1002/pbc.25664. PMID: 26179572

Clinical prediction guides

Bandino JP, Purvis CG, Shaffer BR, Gad A, Elston DM
Am J Dermatopathol 2018 Nov;40(11):815-818. doi: 10.1097/DAD.0000000000001232. PMID: 30045067
Kunze E, Theuring F, Krüger G
Pathol Res Pract 1994 Apr;190(4):311-32. doi: 10.1016/S0344-0338(11)80404-8. PMID: 8078801

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