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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.
 
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).

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).

Professional guidelines

PubMed

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
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
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

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
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
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
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
Skubitz KM, D'Adamo DR
Mayo Clin Proc 2007 Nov;82(11):1409-32. doi: 10.4065/82.11.1409. PMID: 17976362

Diagnosis

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
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
Jaber OI, Kirby PA
Arch Pathol Lab Med 2015 Nov;139(11):1459-62. doi: 10.5858/arpa.2014-0385-RS. PMID: 26516944
Skubitz KM, D'Adamo DR
Mayo Clin Proc 2007 Nov;82(11):1409-32. doi: 10.4065/82.11.1409. PMID: 17976362
Zarrin-Khameh N, Kaye KS
Arch Pathol Lab Med 2007 Mar;131(3):488-91. doi: 10.5858/2007-131-488-ASPS. PMID: 17516754

Therapy

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
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
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
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
Skubitz KM, D'Adamo DR
Mayo Clin Proc 2007 Nov;82(11):1409-32. doi: 10.4065/82.11.1409. PMID: 17976362

Prognosis

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
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
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
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
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

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
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
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
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
Paoluzzi L, Maki RG
JAMA Oncol 2019 Feb 1;5(2):254-260. doi: 10.1001/jamaoncol.2018.4490. PMID: 30347044

Recent systematic reviews

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
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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