Systemic treatment of advanced clear cell sarcoma: results from a retrospective international series from the World Sarcoma Network.
Date
2022-06-01ICR Author
Author
Smrke, A
Frezza, AM
Giani, C
Somaiah, N
Brahmi, M
Czarnecka, AM
Rutkowski, P
Van der Graaf, W
Baldi, GG
Connolly, E
Duffaud, F
Huang, PH
Gelderblom, H
Bhadri, V
Grimison, P
Mahar, A
Stacchiotti, S
Jones, RL
Type
Journal Article
Metadata
Show full item recordAbstract
BACKGROUND: Clear cell sarcoma (CCS) is a translocated aggressive malignancy with a high incidence of metastases and poor prognosis. There are few studies describing the activity of systemic therapy in CCS. We report a multi-institutional retrospective study of the outcomes of patients with advanced CCS treated with systemic therapy within the World Sarcoma Network (WSN). MATERIALS AND METHODS: Patients with molecularly confirmed locally advanced or metastatic CCS treated with systemic therapy from June 1985 to May 2021 were included. Baseline demographic and treatment information, including response by Response Evaluation Criteria in Solid Tumours (RECIST) 1.1, was retrospectively collected by local investigators. Descriptive statistics were carried out. RESULTS: Fifty-five patients from 10 institutions were included. At diagnosis, the median age was 30 (15-73) years and 24% (n = 13/55) had metastatic disease. The median age at diagnosis was 30 (15-73) years. Most primary tumours were at aponeurosis (n = 9/55, 16%) or non-aponeurosis limb sites (n = 17/55, 31%). The most common fusion was EWSR1-ATF1 (n = 24/55, 44%). The median number of systemic therapies was 1 (range 1-7). The best response rate was seen for patients treated with sunitinib (30%, n = 3/10), with a median progression-free survival of 4 [95% confidence interval (CI) 1-7] months. The median overall survival for patients with advanced/metastatic disease was 15 months (95% CI 3-27 months). CONCLUSIONS: Soft tissue sarcoma-type systemic therapies have limited benefit in advanced CCS and response rate was poor. International, multicentre prospective translational studies are required to identify new treatments for this ultra-rare subtype, and access to early clinical trial enrolment remains key for patients with CCS.
Collections
Subject
CCS
EWSR1–ATF1
chemotherapy
clear cell sarcoma
soft tissue sarcoma
sunitinib
systemic therapy
Adolescent
Adult
Aged
Humans
Middle Aged
Prospective Studies
Retrospective Studies
Sarcoma, Clear Cell
Soft Tissue Neoplasms
Sunitinib
Young Adult
Research team
Mol and Systems Oncology
Language
eng
Date accepted
2022-05-19
License start date
2022-06-01
Citation
ESMO Open, 2022, 7 (3), pp. 100522 -
Publisher
ELSEVIER
Except where otherwise noted, this item's license is described
as
https://creativecommons.org/licenses/by-nc-nd/4.0/
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