Gemcitabine in advanced angiosarcoma: a retrospective case series analysis from the Italian Rare Cancer Network

• Published in: Annals of oncology Vol. 23; no. 2; pp. 501 - 508
• Main Authors: Stacchiotti, S., Palassini, E., Sanfilippo, R., Vincenzi, B., Arena, M.G., Bochicchio, A.M., De Rosa, P., Nuzzo, A., Turano, S., Morosi, C., Dei Tos, A.P., Pilotti, S., Casali, P.G.
• Format: Journal Article
• Language: English
• Published: Oxford Elsevier Ltd 01-02-2012; Oxford University Press
• Subjects: Adult; Aged; Aged, 80 and over; angiosarcoma; Antineoplastic agents; Biological and medical sciences; chemotherapy; Deoxycytidine - analogs & derivatives; Deoxycytidine - therapeutic use; gemcitabine; Hemangiosarcoma - drug therapy; Humans; Medical sciences; Middle Aged; Pharmacology. Drug treatments; Retrospective Studies; sarcoma; Survival Analysis; Italy; Europe; angiosarcoma; gemcitabine; sarcoma; chemotherapy; Antineoplastic agent; Gemcitabine; Sarcoma; Cardiovascular disease; Malignant tumor; Angiosarcoma; Vascular disease; Chemotherapy; Treatment; Antimetabolic; Follow up study; Network; Pyrimidine derivatives; Advanced stage; Fluorine Organic compounds; Pyrimidine nucleoside; Cancer
• ISSN: 0923-7534; 1569-8041
• DOI: 10.1093/annonc/mdr066

Angiosarcoma is a highly aggressive soft tissue sarcoma. Responses to anthracyclines plus/minus ifosfamide, and taxanes alone or in combination with gemcitabine are well documented. Very few data are available on gemcitabine as a single agent.
We retrospectively reviewed all cases of advanced progressive angiosarcoma treated with gemcitabine as a single agent (1000 mg/m2 i.v. every week for 3 weeks every 4 weeks), at Istituto Nazionale Tumori and within the Italian Rare Cancers Network from January 2008 to November 2010.
Twenty-five patients [mean age: 52 years; radiation therapy (RT)-related: 8] received gemcitabine. Best tumor response by RECIST was as follows: complete response = 2, partial response = 14, stable disease = 2, progressive disease = 7 cases, for an overall response rate (PR + CR) of 68%. Six of eight post-RT angiosarcomas responded to treatment. Median overall survival (OS) was 17 months. Median progression-free survival (PFS) was 7 months (range 1–40 months). One patient with a locally advanced thyroid angiosarcoma became resectable after 5 months of gemcitabine, with <10% residual viable tumor cells seen on surgical specimen. Overall, gemcitabine was well tolerated.
Gemcitabine is active in both RT- and non-RT-related angiosarcoma, with dimensional and possibly long-lasting responses. A formal phase II study on gemcitabine as a single agent is warranted.