Activity of anthracycline- and ifosfamide-based chemotherapy in a series of patients affected by advanced myxofibrosarcoma

Abstract

Background: We report on the activity of anthracycline-based and high-dose prolonged-infusion ifosfamide chemotherapy in a retrospective series of patients affected by advanced myxofibrosarcoma treated at Istituto Nazionale Tumori in Milan, Italy, and within the Italian Rare Cancer Network (RTR).

Methods: Advanced myxofibrosarcoma patients treated with anthracycline + ifosfamide and high-dose prolonged-infusion ifosfamide as a single agent from November 2001 to December 2016 were retrospectively reviewed. All pathological diagnosis were centrally reviewed by at least two expert pathologists. Response was evaluated by RECIST, and survival functions were computed.

Results: Among 34 advanced myxofibrosarcoma patients, 13 were treated with front-line anthracycline + ifosfamide chemotherapy (male/female = 6/7, median age 54 years, range 33-72). Overall best response was: 4 partial responses, 3 stable diseases and 6 progressive diseases, with a median progression-free survival of 4 months. Twenty-eight patients received second/further line high-dose prolonged-infusion ifosfamide (male/female = 17/11, median age 55 years, range 27-75 years). We observed 10 partial responses, 4 stable diseases and 14 progressive diseases, with a median progression-free survival of 4 months. Median overall survival was 12 months.

Conclusions: This retrospective analysis suggests that the combination of anthracyclines and ifosfamide is active in myxofibrosarcoma. In patients already treated with a combination of anthracyclines and ifosfamide, high-dose prolonged-infusion ifosfamide showed activity as well.

Keywords: Chemotherapy; High-dose prolonged-infusion ifosfamide; Myxofibrosarcoma; Soft tissue sarcoma.

Fig. 1

Survival functions for patients treated with front line anthracycline + ifosfamide chemotherapy (13 patients). a PFS (median 4 months). b OS (median 12 months)

Fig. 2

Survival functions for patients treated with continuous infusion HDIFX (28 patients). a PFS (median 4 months). b OS (median 12 months)

Fig. 3

On haematoxylin and eosin staining, a pathologic partial response in a patient treated with six cycles of high-dose ifosfamide. a A thoracic wall metastasis from a myxofibrosarcoma arising from the thoracic wall at baseline. b In high power and c the pathologic partial response after 6 months of treatment with high-dose ifosfamide with areas of necrosis included both vascular and avascular pattern and vital areas