Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2022 Apr 1;128(7):1439-1448.
doi: 10.1002/cncr.34083. Epub 2022 Jan 13.

Weekly cisplatin with or without imatinib in advanced chordoma: A retrospective case-series analysis from the Italian Rare Cancers Network

Giacomo G Baldi  1 , Salvatore Lo Vullo  2 , Giovanni Grignani  3 , Bruno Vincenzi  4 , Giuseppe Badalamenti  5 , Marinella Mastore  6 , Ciriaco Buonomenna  7 , Carlo Morosi  7 , Marta Barisella  8 , Anna Maria Frezza  9 , Salvatore Provenzano  9 , Noemi Simeone  9 , Fernanda Picozzi  10   11 , Luigi Mariani  2 , Paolo G Casali  9   12 , Silvia Stacchiotti  9
Affiliations
Free article

Weekly cisplatin with or without imatinib in advanced chordoma: A retrospective case-series analysis from the Italian Rare Cancers Network

Giacomo G Baldi et al. Cancer. .
Free article

Abstract

Background: To report on a retrospective case-series analysis of weekly cisplatin (wCDDP) as a single agent or combined with imatinib (wCDDP/I) in patients with advanced chordoma treated within the Italian Rare Cancer Network.

Methods: Adult patients with a diagnosis of advanced, brachyury-positive chordoma, treated from April 2007 to October 2020 with wCDDP or wCDDP/I were retrospectively identified. Imatinib was withheld at the same time as wCDDP. Response according to Response Evaluation Criteria in Solid Tumors, overall survival (OS), and progression-free survival (PFS) were analyzed.

Results: Thirty-three consecutive patients were identified (wCDDP as front-line n = 8 [24.2%]; wCDDP as a further line n = 25 [75.8%]; prior imatinib n = 25 [75.8%]; evidence of progression before starting wCDDP n = 33). Of 32 patients evaluable for response (wCDDP, n = 22 [68.8%]; wCDDP/I, n = 10 [31.3%]), best response was stable disease (SD) in 27 patients (84.3%) and progression in 5 patients (15.6%). At a median follow-up of 54 months, the median OS (m-OS) was 30.3 months (interquartile range [IQR], 18.1-56.6), the m-PFS was 8.0 months (IQR, 5.1-17.0), the 6-month PFS rate was 65.2%, and the 12-month PFS rate was 30.3%. Of 22 patients who received wCDDP, the best response was SD in 18 patients (81.8%) and progression in 4 patients (18.2%), and the m-PFS was 8.0 months (IQR, 5.1-17.0 months). Of 10 patients who received treatment with wCDDP/I, the best response was SD in 9 patients (90%) and progression in 1 patient (10%), and the m-PFS was 9.3 months (IQR, 4.9-26.5 months).

Conclusions: This series suggests that wCDDP, both as a single agent and combined with imatinib, has antitumor activity in chordoma. Although no dimensional responses were observed, 65% and 30% of previously progressive patients were progression-free at 6 and 12 months, respectively. A prospective study is warranted.

Keywords: bone sarcoma; chemotherapy; chordoma; cisplatin; imatinib; systemic treatment.

PubMed Disclaimer

References

    1. Choi KS, Cohn MJ, Harfe BD. Identification of nucleus pulposus precursor cells and notochordal remnants in the mouse: implications for disk degeneration and chordoma formation. Dev Dyn. 2008;237:3953-3958.
    1. Flanagan AM, Yamaguchi T. Chordoma. In: Fletcher CDM, Bridge JA, Pancras CW, Mertens F, eds. World Health Organization Classification of Tumours of Soft Tissue and Bone. Pathology and Genetics. IARC Press; 2013:328-329.
    1. Smoll NR, Gautschi OP, Radovanovic I, Schaller K, Weber DC. Incidence and relative survival of chordomas: the standardized mortality ratio and the impact of chordomas on a population. Cancer. 2013;119:2029-2037.
    1. Heffelfinger MJ, Dahlin DC, MacCarty CS, Beabout JW. Chordomas and cartilaginous tumors at the skull base. Cancer. 1973;32:410-420.
    1. de Pinieux G, Karanian M, Le Loarer F, et al. Nationwide incidence of sarcomas and connective tissue tumors of intermediate malignancy over four years using an expert pathology review network. PLoS One. 2021;16:e0246958.

LinkOut - more resources