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Askin tumor

MedGen UID:
209112
•Concept ID:
C0877849
•
Neoplastic Process
Synonym: Askin's tumor (PNET of the chest wall) (type)
SNOMED CT: Askin's tumor (128783001); Askin tumor (128783001)
 
Monarch Initiative: MONDO:0006094
OMIM®: 612219

Definition

A primitive neuroectodermal tumor (small round blue cell tumor) of the thorax which can involve the periosteum, thoracic wall and/or pleura though it spares the lung parenchyma. [from NCI]

Professional guidelines

PubMed

Outcomes after interdisciplinary management of 7 patients with Askin tumor.
Basharkhah A, Pansy J, Urban C, Höllwarth ME
Pediatr Surg Int 2013 May;29(5):431-6. Epub 2013 Feb 10 doi: 10.1007/s00383-013-3272-1. PMID: 23397588
Ewing's sarcoma: standard and experimental treatment options.
Subbiah V, Anderson P, Lazar AJ, Burdett E, Raymond K, Ludwig JA
Curr Treat Options Oncol 2009 Apr;10(1-2):126-40. Epub 2009 Jun 17 doi: 10.1007/s11864-009-0104-6. PMID: 19533369
Current treatment for Ewing's sarcoma.
Thacker MM, Temple HT, Scully SP
Expert Rev Anticancer Ther 2005 Apr;5(2):319-31. doi: 10.1586/14737140.5.2.319. PMID: 15877528

Recent clinical studies

Etiology

Bad to the Bone: Emerging Approaches to Aggressive Bone Sarcomas.
Wood GE, Graves LA, Rubin EM, Reed DR, Riedel RF, Strauss SJ
Am Soc Clin Oncol Educ Book 2023 May;43:e390306. doi: 10.1200/EDBK_390306. PMID: 37220319
Survival after high-dose chemotherapy for refractory and recurrent Ewing sarcoma.
Windsor R, Hamilton A, McTiernan A, Dileo P, Michelagnoli M, Seddon B, Strauss SJ, Whelan J
Eur J Cancer 2022 Jul;170:131-139. Epub 2022 May 26 doi: 10.1016/j.ejca.2022.04.007. PMID: 35635934
Antitumor Activity of Lurbinectedin, a Selective Inhibitor of Oncogene Transcription, in Patients with Relapsed Ewing Sarcoma: Results of a Basket Phase II Study.
Subbiah V, Braña I, Longhi A, Boni V, Delord JP, Awada A, Boudou-Rouquette P, Sarantopoulos J, Shapiro GI, Elias A, Ratan R, Fernandez C, Kahatt C, Cullell-Young M, Siguero M, Zeaiter A, Chawla SP
Clin Cancer Res 2022 Jul 1;28(13):2762-2770. doi: 10.1158/1078-0432.CCR-22-0696. PMID: 35486638Free PMC Article
Askin tumor: CT and FDG-PET/CT imaging findings and follow-up.
Xia T, Guan Y, Chen Y, Li J
Medicine (Baltimore) 2014 Jul;93(6):e42. doi: 10.1097/MD.0000000000000042. PMID: 25058144Free PMC Article
Emerging chemotherapeutic strategies and the role of treatment stratification in Ewing sarcoma.
Seddon BM, Whelan JS
Paediatr Drugs 2008;10(2):93-105. doi: 10.2165/00148581-200810020-00004. PMID: 18345719

Diagnosis

Rapidly fatal Askin's tumor: a case report and literature review.
Laine M, Ghorfi IA, Lambatten D, Kettani F, Abid A
Pan Afr Med J 2014;18:104. Epub 2014 May 30 doi: 10.11604/pamj.2014.18.104.4549. PMID: 25404964Free PMC Article
Askin tumor: CT and FDG-PET/CT imaging findings and follow-up.
Xia T, Guan Y, Chen Y, Li J
Medicine (Baltimore) 2014 Jul;93(6):e42. doi: 10.1097/MD.0000000000000042. PMID: 25058144Free PMC Article
Congenital Askin tumor with favorable outcome: case report and review of the literature.
Crocoli A, Bagolan P, Boldrini R, Natali GL, De Ioris MA, Morini F
J Pediatr Surg 2012 Jul;47(7):1440-4. doi: 10.1016/j.jpedsurg.2012.02.028. PMID: 22813811
Ewing tumor: tumor biology and clinical applications.
de Alava E, Pardo J
Int J Surg Pathol 2001 Jan;9(1):7-17. doi: 10.1177/106689690100900104. PMID: 11469351
Evidence for neural origin and PAS-positive variants of the malignant small cell tumor of thoracopulmonary region ("Askin tumor").
Linnoila RI, Tsokos M, Triche TJ, Marangos PJ, Chandra RS
Am J Surg Pathol 1986 Feb;10(2):124-33. doi: 10.1097/00000478-198602000-00005. PMID: 3953935

Therapy

Phase 2 trial of palbociclib and ganitumab in patients with relapsed Ewing sarcoma.
Shulman DS, Merriam P, Choy E, Guenther LM, Cavanaugh KL, Kao PC, Posner A, Bhushan K, Fairchild G, Barker E, Klega K, Stegmaier K, Crompton BD, London WB, DuBois SG
Cancer Med 2023 Jul;12(14):15207-15216. Epub 2023 Jun 12 doi: 10.1002/cam4.6208. PMID: 37306107Free PMC Article
Bad to the Bone: Emerging Approaches to Aggressive Bone Sarcomas.
Wood GE, Graves LA, Rubin EM, Reed DR, Riedel RF, Strauss SJ
Am Soc Clin Oncol Educ Book 2023 May;43:e390306. doi: 10.1200/EDBK_390306. PMID: 37220319
Pilot Study of Recurrent Ewing's Sarcoma Management with Vigil/Temozolomide/Irinotecan and Assessment of Circulating Tumor (ct) DNA.
Anderson P, Ghisoli M, Crompton BD, Klega KS, Wexler LH, Slotkin EK, Stanbery L, Manning L, Wallraven G, Manley M, Horvath S, Bognar E, Nemunaitis J
Clin Cancer Res 2023 May 1;29(9):1689-1697. doi: 10.1158/1078-0432.CCR-22-2292. PMID: 36780200Free PMC Article
Survival after high-dose chemotherapy for refractory and recurrent Ewing sarcoma.
Windsor R, Hamilton A, McTiernan A, Dileo P, Michelagnoli M, Seddon B, Strauss SJ, Whelan J
Eur J Cancer 2022 Jul;170:131-139. Epub 2022 May 26 doi: 10.1016/j.ejca.2022.04.007. PMID: 35635934
Antitumor Activity of Lurbinectedin, a Selective Inhibitor of Oncogene Transcription, in Patients with Relapsed Ewing Sarcoma: Results of a Basket Phase II Study.
Subbiah V, Braña I, Longhi A, Boni V, Delord JP, Awada A, Boudou-Rouquette P, Sarantopoulos J, Shapiro GI, Elias A, Ratan R, Fernandez C, Kahatt C, Cullell-Young M, Siguero M, Zeaiter A, Chawla SP
Clin Cancer Res 2022 Jul 1;28(13):2762-2770. doi: 10.1158/1078-0432.CCR-22-0696. PMID: 35486638Free PMC Article

Prognosis

Survival after high-dose chemotherapy for refractory and recurrent Ewing sarcoma.
Windsor R, Hamilton A, McTiernan A, Dileo P, Michelagnoli M, Seddon B, Strauss SJ, Whelan J
Eur J Cancer 2022 Jul;170:131-139. Epub 2022 May 26 doi: 10.1016/j.ejca.2022.04.007. PMID: 35635934
Askin tumor: CT and FDG-PET/CT imaging findings and follow-up.
Xia T, Guan Y, Chen Y, Li J
Medicine (Baltimore) 2014 Jul;93(6):e42. doi: 10.1097/MD.0000000000000042. PMID: 25058144Free PMC Article
Late surgery for spinal deformities in children previously treated for neural tumors.
Barrena S, Miguel M, de la Torre CA, Ramírez M, Díaz M, Martinez L, Pérez-Grueso FS, Lassaletta L, Tovar JA
Eur J Pediatr Surg 2011 Jan;21(1):54-7. Epub 2011 Feb 16 doi: 10.1055/s-0030-1267968. PMID: 21328191
Emerging chemotherapeutic strategies and the role of treatment stratification in Ewing sarcoma.
Seddon BM, Whelan JS
Paediatr Drugs 2008;10(2):93-105. doi: 10.2165/00148581-200810020-00004. PMID: 18345719
Malignant small-cell tumor of the thoracopulmonary region: 'Askin tumor'.
Bourque MD, Di Lorenzo M, Collin PP, Russo P, Laberge JM, Moir C
J Pediatr Surg 1989 Oct;24(10):1079-83. doi: 10.1016/s0022-3468(89)80221-0. PMID: 2553909

Clinical prediction guides

Pilot Study of Recurrent Ewing's Sarcoma Management with Vigil/Temozolomide/Irinotecan and Assessment of Circulating Tumor (ct) DNA.
Anderson P, Ghisoli M, Crompton BD, Klega KS, Wexler LH, Slotkin EK, Stanbery L, Manning L, Wallraven G, Manley M, Horvath S, Bognar E, Nemunaitis J
Clin Cancer Res 2023 May 1;29(9):1689-1697. doi: 10.1158/1078-0432.CCR-22-2292. PMID: 36780200Free PMC Article
Survival after high-dose chemotherapy for refractory and recurrent Ewing sarcoma.
Windsor R, Hamilton A, McTiernan A, Dileo P, Michelagnoli M, Seddon B, Strauss SJ, Whelan J
Eur J Cancer 2022 Jul;170:131-139. Epub 2022 May 26 doi: 10.1016/j.ejca.2022.04.007. PMID: 35635934
Suicide gene therapy of sarcoma cell lines using recombinant adeno-associated virus 2 vectors.
Veldwijk MR, Berlinghoff S, Laufs S, Hengge UR, Zeller WJ, Wenz F, Fruehauf S
Cancer Gene Ther 2004 Aug;11(8):577-84. doi: 10.1038/sj.cgt.7700718. PMID: 15280909
Ten-day schedule oral etoposide therapy in advanced childhood malignancies.
Schiavetti A, Varrasso G, Maurizi P, Cappelli C, Clerico A, Properzi E, Castello MA
J Pediatr Hematol Oncol 2000 Mar-Apr;22(2):119-24. doi: 10.1097/00043426-200003000-00008. PMID: 10779024
Evidence for neural origin and PAS-positive variants of the malignant small cell tumor of thoracopulmonary region ("Askin tumor").
Linnoila RI, Tsokos M, Triche TJ, Marangos PJ, Chandra RS
Am J Surg Pathol 1986 Feb;10(2):124-33. doi: 10.1097/00000478-198602000-00005. PMID: 3953935

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