title: Chemotherapy in clear cell sarcoma.
abstract: Clear cell sarcoma is a rare translocation-related sarcoma. There have been few studies documenting the response rate and progression-free survival in clear cell sarcoma patients treated with palliative chemotherapy. The prospectively maintained databases of two referral centres were searched to identify clear cell sarcoma patients treated with chemotherapy. Twenty-four patients were treated with palliative first-line chemotherapy with a median age of 30 years at diagnosis. There were 18 men and 6 women. One (4%) achieved a partial response and 9 (38%) had stable disease. Fourteen patients (58%) progressed on therapy. The median progression-free survival was 11 weeks (95% CI, 3–20 weeks). The median overall survival from commencing first-line chemotherapy was 39 weeks (95% CI, 34–45 weeks). Second-line chemotherapy was administered to 12 patients, 11 (92%) of these progressed and one (8%) had stable disease. Of the 5 patients treated with third-line chemotherapy, 4 (80%) progressed and one (20%) had stable disease. One patient received fourth-line chemotherapy and maintained stable disease for 4 months. Conventional chemotherapy has minimal activity in clear cell sarcoma as documented by the response rate of 4% and median progression-free survival of 11 weeks in this retrospective series. These data provide a reference for response and outcome in the assessment of novel agents in this histological subtype.
authors.affiliation: Royal Marsden Hospital, London, UK. ROBIN.JONES@doctors.org.uk
authors.forename: Robin L
authors.initials: RL
authors.lastname: Jones
authors.forename: Anastasia
authors.initials: A
authors.lastname: Constantinidou
authors.forename: Khin
authors.initials: K
authors.lastname: Thway
authors.forename: Sue
authors.initials: S
authors.lastname: Ashley
authors.forename: Michelle
authors.initials: M
authors.lastname: Scurr
authors.forename: Omar
authors.initials: O
authors.lastname: Al-Muderis
authors.forename: Cyril
authors.initials: C
authors.lastname: Fisher
authors.forename: Cristina R
authors.initials: CR
authors.lastname: Antonescu
authors.forename: David R
authors.initials: DR
authors.lastname: D'Adamo
authors.forename: Mary Louise
authors.initials: ML
authors.lastname: Keohan
authors.forename: Robert G
authors.initials: RG
authors.lastname: Maki
authors.forename: Ian R
authors.initials: IR
authors.lastname: Judson
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D012189:Retrospective Studies; D018227:Sarcoma, Clear Cell / Q000188:drug therapy* / Q000401:mortality*
references.citation: Am J Surg Pathol. 1983 Jul;7(5):405-13
references.pmid: 6614306
references.citation: Cancer. 2007 Jan 1;109(1):109-16
references.pmid: 17133413
references.citation: Br J Radiol. 1979 Mar;52(615):238-41
references.pmid: 86372
references.citation: Am J Surg Pathol. 2008 Mar;32(3):452-60
references.pmid: 18300804
references.citation: Eur J Cancer. 1991;27(3):254-6
references.pmid: 1827307
references.citation: Am J Surg Pathol. 1992 Dec;16(12):1197-204
references.pmid: 1463095
references.citation: Mol Cancer Ther. 2008 Jun;7(6):1751-61
references.pmid: 18566246
references.citation: J Clin Oncol. 2003 May 1;21(9):1775-81
references.pmid: 12721254
references.citation: Nat Genet. 1993 Aug;4(4):341-5
references.pmid: 8401579
references.citation: Cancer. 1999 Sep 15;86(6):969-75
references.pmid: 10491522
references.citation: Cancer. 1965 Sep;18:1163-74
references.pmid: 14332545
references.citation: Cancer. 1990 Jan 15;65(2):367-74
references.pmid: 2295060
references.citation: Cancer. 2002 Jun 15;94(12):3269-76
references.pmid: 12115360
references.citation: Eur J Surg Oncol. 2008 Jul;34(7):800-4
references.pmid: 18042498
references.citation: Cancer. 1984 Oct 1;54(7):1412-7
references.pmid: 6467162
references.citation: J Surg Oncol. 2001 May;77(1):16-20
references.pmid: 11344475
references.citation: Cancer. 1983 Oct 15;52(8):1482-8
references.pmid: 6616410
references.citation: Genes Chromosomes Cancer. 1992 Mar;4(2):122-7
references.pmid: 1373311
references.citation: Pigment Cell Melanoma Res. 2008 Aug;21(4):457-63
references.pmid: 18627530
references.citation: Tumori. 1999 Nov-Dec;85(6):512-4
references.pmid: 10774576