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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

The magnetic resonance characteristics of sinonasal rhabdomyosarcoma in adults: analysis of 27 cases and comparison with pathological subtypes.
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
A Comparison of the Histopathologic Growth Patterns Between Non-Merkel Cell Small Round Blue Cell Tumors and Merkel Cell Carcinoma.
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
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
Molecular Cytogenetics Detect an Unbalanced t(2;13)(q36;q14) and PAX3-FOXO1 Fusion in Rhabdomyosarcoma With Mixed Embryonal/Alveolar Features.
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
Sclerosing rhabdomyosarcomas in children and adolescents: a clinicopathologic review of 13 cases from the Intergroup Rhabdomyosarcoma Study Group and Children's Oncology Group.
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

The magnetic resonance characteristics of sinonasal rhabdomyosarcoma in adults: analysis of 27 cases and comparison with pathological subtypes.
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
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
Sclerosing rhabdomyosarcomas in children and adolescents: a clinicopathologic review of 13 cases from the Intergroup Rhabdomyosarcoma Study Group and Children's Oncology Group.
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
Primary mesenchymal tumors of the urinary bladder. A histological and immunohistochemical study of 30 cases.
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
Is fetal cellular rhabdomyoma an entity or a differentiated rhabdomyosarcoma? A study of patients with rhabdomyoma of the tongue and sarcoma of the tongue enrolled in the intergroup rhabdomyosarcoma studies I, II, and III.
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

A Comparison of the Histopathologic Growth Patterns Between Non-Merkel Cell Small Round Blue Cell Tumors and Merkel Cell Carcinoma.
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

The magnetic resonance characteristics of sinonasal rhabdomyosarcoma in adults: analysis of 27 cases and comparison with pathological subtypes.
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
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
Molecular Cytogenetics Detect an Unbalanced t(2;13)(q36;q14) and PAX3-FOXO1 Fusion in Rhabdomyosarcoma With Mixed Embryonal/Alveolar Features.
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

A Comparison of the Histopathologic Growth Patterns Between Non-Merkel Cell Small Round Blue Cell Tumors and Merkel Cell Carcinoma.
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
Primary mesenchymal tumors of the urinary bladder. A histological and immunohistochemical study of 30 cases.
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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