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Alveolar soft part sarcoma(ASPS)

MedGen UID:
61652
•Concept ID:
C0206657
•
Neoplastic Process
Synonyms: Alveolar soft part sarcoma (disease); ASPS
SNOMED CT: Alveolar soft part sarcoma (88195001); Alveolar soft part sarcoma (404056007)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Gene (location): ASPSCR1 (17q25.3)
 
HPO: HP:0012218
Monarch Initiative: MONDO:0011655
OMIM®: 606243
Orphanet: ORPHA163699

Definition

Alveolar soft part sarcoma is an unusual tumor with highly characteristic histopathology and ultrastructure, controversial histogenesis, and enigmatic clinical behavior (Lieberman et al., 1989; Ordonez, 1999). The typical histology of ASPS shows well-defined nests of cells with abundant pink cytoplasm. The loss of central cohesion produces a pseudoalveolar appearance (Ladanyi et al., 2001). [from OMIM]

Clinical features

From HPO
Alveolar soft part sarcoma
MedGen UID:
61652
•Concept ID:
C0206657
•
Neoplastic Process
Alveolar soft part sarcoma is an unusual tumor with highly characteristic histopathology and ultrastructure, controversial histogenesis, and enigmatic clinical behavior (Lieberman et al., 1989; Ordonez, 1999). The typical histology of ASPS shows well-defined nests of cells with abundant pink cytoplasm. The loss of central cohesion produces a pseudoalveolar appearance (Ladanyi et al., 2001).
See: Feature record | Search on this feature

Conditions with this feature

Alveolar soft part sarcoma
MedGen UID:
61652
•Concept ID:
C0206657
•
Neoplastic Process
Alveolar soft part sarcoma is an unusual tumor with highly characteristic histopathology and ultrastructure, controversial histogenesis, and enigmatic clinical behavior (Lieberman et al., 1989; Ordonez, 1999). The typical histology of ASPS shows well-defined nests of cells with abundant pink cytoplasm. The loss of central cohesion produces a pseudoalveolar appearance (Ladanyi et al., 2001).
See: Condition Record
Alveolar soft part sarcoma

Professional guidelines

PubMed

Advances in treatment of alveolar soft part sarcoma: an updated review.
Fujiwara T, Kunisada T, Nakata E, Nishida K, Yanai H, Nakamura T, Tanaka K, Ozaki T
Jpn J Clin Oncol 2023 Nov 5;53(11):1009-1018. doi: 10.1093/jjco/hyad102. PMID: 37626447Free PMC Article
Advances in the management of alveolar soft part sarcoma.
O'Sullivan Coyne G, Naqash AR, Sankaran H, Chen AP
Curr Probl Cancer 2021 Aug;45(4):100775. Epub 2021 Jul 8 doi: 10.1016/j.currproblcancer.2021.100775. PMID: 34284873
Diagnosis, Prognosis, and Treatment of Alveolar Soft-Part Sarcoma: A Review.
Paoluzzi L, Maki RG
JAMA Oncol 2019 Feb 1;5(2):254-260. doi: 10.1001/jamaoncol.2018.4490. PMID: 30347044

Recent clinical studies

Etiology

Atezolizumab for Advanced Alveolar Soft Part Sarcoma.
Chen AP, Sharon E, O'Sullivan-Coyne G, Moore N, Foster JC, Hu JS, Van Tine BA, Conley AP, Read WL, Riedel RF, Burgess MA, Glod J, Davis EJ, Merriam P, Naqash AR, Fino KK, Miller BL, Wilsker DF, Begum A, Ferry-Galow KV, Deshpande HA, Schwartz GK, Ladle BH, Okuno SH, Beck JC, Chen JL, Takebe N, Fogli LK, Rosenberger CL, Parchment RE, Doroshow JH
N Engl J Med 2023 Sep 7;389(10):911-921. doi: 10.1056/NEJMoa2303383. PMID: 37672694Free PMC Article
Soft-tissue sarcoma in adolescents and young adults.
Kunisada T, Nakata E, Fujiwara T, Hosono A, Takihira S, Kondo H, Ozaki T
Int J Clin Oncol 2023 Jan;28(1):1-11. Epub 2022 Jan 27 doi: 10.1007/s10147-022-02119-7. PMID: 35084598
Durvalumab plus tremelimumab in advanced or metastatic soft tissue and bone sarcomas: a single-centre phase 2 trial.
Somaiah N, Conley AP, Parra ER, Lin H, Amini B, Solis Soto L, Salazar R, Barreto C, Chen H, Gite S, Haymaker C, Nassif EF, Bernatchez C, Mitra A, Livingston JA, Ravi V, Araujo DM, Benjamin R, Patel S, Zarzour MA, Sabir S, Lazar AJ, Wang WL, Daw NC, Zhou X, Roland CL, Cooper ZA, Rodriguez-Canales J, Futreal A, Soria JC, Wistuba II, Hwu P
Lancet Oncol 2022 Sep;23(9):1156-1166. Epub 2022 Aug 4 doi: 10.1016/S1470-2045(22)00392-8. PMID: 35934010
Axitinib plus pembrolizumab in patients with advanced sarcomas including alveolar soft-part sarcoma: a single-centre, single-arm, phase 2 trial.
Wilky BA, Trucco MM, Subhawong TK, Florou V, Park W, Kwon D, Wieder ED, Kolonias D, Rosenberg AE, Kerr DA, Sfakianaki E, Foley M, Merchan JR, Komanduri KV, Trent JC
Lancet Oncol 2019 Jun;20(6):837-848. Epub 2019 May 8 doi: 10.1016/S1470-2045(19)30153-6. PMID: 31078463
Sarcoma.
Skubitz KM, D'Adamo DR
Mayo Clin Proc 2007 Nov;82(11):1409-32. doi: 10.4065/82.11.1409. PMID: 17976362

Diagnosis

Advances in treatment of alveolar soft part sarcoma: an updated review.
Fujiwara T, Kunisada T, Nakata E, Nishida K, Yanai H, Nakamura T, Tanaka K, Ozaki T
Jpn J Clin Oncol 2023 Nov 5;53(11):1009-1018. doi: 10.1093/jjco/hyad102. PMID: 37626447Free PMC Article
Immunotherapy and Biomarkers in Sarcoma.
Dajsakdipon T, Siripoon T, Ngamphaiboon N, Ativitavas T, Dejthevaporn T
Curr Treat Options Oncol 2022 Mar;23(3):415-438. Epub 2022 Mar 9 doi: 10.1007/s11864-022-00944-6. PMID: 35262852
Options for treating different soft tissue sarcoma subtypes.
Ray-Coquard I, Serre D, Reichardt P, Martín-Broto J, Bauer S
Future Oncol 2018 May;14(10s):25-49. doi: 10.2217/fon-2018-0076. PMID: 29768052
Alveolar Soft Part Sarcoma.
Jaber OI, Kirby PA
Arch Pathol Lab Med 2015 Nov;139(11):1459-62. doi: 10.5858/arpa.2014-0385-RS. PMID: 26516944
Sarcoma.
Skubitz KM, D'Adamo DR
Mayo Clin Proc 2007 Nov;82(11):1409-32. doi: 10.4065/82.11.1409. PMID: 17976362

Therapy

Atezolizumab for Advanced Alveolar Soft Part Sarcoma.
Chen AP, Sharon E, O'Sullivan-Coyne G, Moore N, Foster JC, Hu JS, Van Tine BA, Conley AP, Read WL, Riedel RF, Burgess MA, Glod J, Davis EJ, Merriam P, Naqash AR, Fino KK, Miller BL, Wilsker DF, Begum A, Ferry-Galow KV, Deshpande HA, Schwartz GK, Ladle BH, Okuno SH, Beck JC, Chen JL, Takebe N, Fogli LK, Rosenberger CL, Parchment RE, Doroshow JH
N Engl J Med 2023 Sep 7;389(10):911-921. doi: 10.1056/NEJMoa2303383. PMID: 37672694Free PMC Article
Durvalumab plus tremelimumab in advanced or metastatic soft tissue and bone sarcomas: a single-centre phase 2 trial.
Somaiah N, Conley AP, Parra ER, Lin H, Amini B, Solis Soto L, Salazar R, Barreto C, Chen H, Gite S, Haymaker C, Nassif EF, Bernatchez C, Mitra A, Livingston JA, Ravi V, Araujo DM, Benjamin R, Patel S, Zarzour MA, Sabir S, Lazar AJ, Wang WL, Daw NC, Zhou X, Roland CL, Cooper ZA, Rodriguez-Canales J, Futreal A, Soria JC, Wistuba II, Hwu P
Lancet Oncol 2022 Sep;23(9):1156-1166. Epub 2022 Aug 4 doi: 10.1016/S1470-2045(22)00392-8. PMID: 35934010
Axitinib plus pembrolizumab in patients with advanced sarcomas including alveolar soft-part sarcoma: a single-centre, single-arm, phase 2 trial.
Wilky BA, Trucco MM, Subhawong TK, Florou V, Park W, Kwon D, Wieder ED, Kolonias D, Rosenberg AE, Kerr DA, Sfakianaki E, Foley M, Merchan JR, Komanduri KV, Trent JC
Lancet Oncol 2019 Jun;20(6):837-848. Epub 2019 May 8 doi: 10.1016/S1470-2045(19)30153-6. PMID: 31078463
Options for treating different soft tissue sarcoma subtypes.
Ray-Coquard I, Serre D, Reichardt P, Martín-Broto J, Bauer S
Future Oncol 2018 May;14(10s):25-49. doi: 10.2217/fon-2018-0076. PMID: 29768052
Sarcoma.
Skubitz KM, D'Adamo DR
Mayo Clin Proc 2007 Nov;82(11):1409-32. doi: 10.4065/82.11.1409. PMID: 17976362

Prognosis

Atezolizumab for Advanced Alveolar Soft Part Sarcoma.
Chen AP, Sharon E, O'Sullivan-Coyne G, Moore N, Foster JC, Hu JS, Van Tine BA, Conley AP, Read WL, Riedel RF, Burgess MA, Glod J, Davis EJ, Merriam P, Naqash AR, Fino KK, Miller BL, Wilsker DF, Begum A, Ferry-Galow KV, Deshpande HA, Schwartz GK, Ladle BH, Okuno SH, Beck JC, Chen JL, Takebe N, Fogli LK, Rosenberger CL, Parchment RE, Doroshow JH
N Engl J Med 2023 Sep 7;389(10):911-921. doi: 10.1056/NEJMoa2303383. PMID: 37672694Free PMC Article
Soft-tissue sarcoma in adolescents and young adults.
Kunisada T, Nakata E, Fujiwara T, Hosono A, Takihira S, Kondo H, Ozaki T
Int J Clin Oncol 2023 Jan;28(1):1-11. Epub 2022 Jan 27 doi: 10.1007/s10147-022-02119-7. PMID: 35084598
Axitinib plus pembrolizumab in patients with advanced sarcomas including alveolar soft-part sarcoma: a single-centre, single-arm, phase 2 trial.
Wilky BA, Trucco MM, Subhawong TK, Florou V, Park W, Kwon D, Wieder ED, Kolonias D, Rosenberg AE, Kerr DA, Sfakianaki E, Foley M, Merchan JR, Komanduri KV, Trent JC
Lancet Oncol 2019 Jun;20(6):837-848. Epub 2019 May 8 doi: 10.1016/S1470-2045(19)30153-6. PMID: 31078463
Options for treating different soft tissue sarcoma subtypes.
Ray-Coquard I, Serre D, Reichardt P, Martín-Broto J, Bauer S
Future Oncol 2018 May;14(10s):25-49. doi: 10.2217/fon-2018-0076. PMID: 29768052
Sarcoma.
Skubitz KM, D'Adamo DR
Mayo Clin Proc 2007 Nov;82(11):1409-32. doi: 10.4065/82.11.1409. PMID: 17976362

Clinical prediction guides

Atezolizumab for Advanced Alveolar Soft Part Sarcoma.
Chen AP, Sharon E, O'Sullivan-Coyne G, Moore N, Foster JC, Hu JS, Van Tine BA, Conley AP, Read WL, Riedel RF, Burgess MA, Glod J, Davis EJ, Merriam P, Naqash AR, Fino KK, Miller BL, Wilsker DF, Begum A, Ferry-Galow KV, Deshpande HA, Schwartz GK, Ladle BH, Okuno SH, Beck JC, Chen JL, Takebe N, Fogli LK, Rosenberger CL, Parchment RE, Doroshow JH
N Engl J Med 2023 Sep 7;389(10):911-921. doi: 10.1056/NEJMoa2303383. PMID: 37672694Free PMC Article
Immunotherapy in Sarcoma: Where Do Things Stand?
Moreno Tellez C, Leyfman Y, D'Angelo SP, Wilky BA, Dufresne A
Surg Oncol Clin N Am 2022 Jul;31(3):381-397. doi: 10.1016/j.soc.2022.03.004. PMID: 35715140
Immunotherapy and Biomarkers in Sarcoma.
Dajsakdipon T, Siripoon T, Ngamphaiboon N, Ativitavas T, Dejthevaporn T
Curr Treat Options Oncol 2022 Mar;23(3):415-438. Epub 2022 Mar 9 doi: 10.1007/s11864-022-00944-6. PMID: 35262852
Immunotherapy for sarcomas.
Nakata E, Fujiwara T, Kunisada T, Ito T, Takihira S, Ozaki T
Jpn J Clin Oncol 2021 Apr 1;51(4):523-537. doi: 10.1093/jjco/hyab005. PMID: 33611603
Diagnosis, Prognosis, and Treatment of Alveolar Soft-Part Sarcoma: A Review.
Paoluzzi L, Maki RG
JAMA Oncol 2019 Feb 1;5(2):254-260. doi: 10.1001/jamaoncol.2018.4490. PMID: 30347044

Recent systematic reviews

Comparative Combinatorial Implications and Theranostics of Immunotherapy in the Impediment of Alveolar Soft Part Sarcoma.
Yang Y, Beeraka NM, Liu J, Zuo X, Wang X, Li T, Fan R
Curr Pharm Des 2022;28(41):3404-3412. doi: 10.2174/1381612828666220921151750. PMID: 36154597
Immune checkpoint inhibitors in treatment of soft-tissue sarcoma: A systematic review and meta-analysis.
Saerens M, Brusselaers N, Rottey S, Decruyenaere A, Creytens D, Lapeire L
Eur J Cancer 2021 Jul;152:165-182. Epub 2021 Jun 6 doi: 10.1016/j.ejca.2021.04.034. PMID: 34107450

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