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The Desmoid Tumor Research Foundation

June 2018

FAP-related desmoid tumours treated with low dose chemotherapy: Results from a multicentre retrospective analysis

Author Information

B Vincenzi, S Provenzano, A Brunello, G Badalamenti, M Nannini, T Ibrahim, P Hohenberger, S Gasperoni, A Napolitano, M Silletta, M Vitellaro, A Paolo Dei Tos, D Santini, G Tonini, E Palassini

Dept of Medical Oncology, University Campus Bio-Medico of Rome, Rome, Italy; Medical Oncology Unit 2, Medical Oncology Dept, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy; Istituto Oncologico Veneto, IRCCS, Padova, Italy; Dept of Surgical, Oncological, and Oral Sciences, Section of Medical Oncology, University of Palermo, Palermo, Italy; University of Bologna, Bologna, Italy; Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori (IRST) IRCCS, Meldola, Italy; Division of Surgical Oncology and Thoracic Surgery, Mannheim University Medical Centre, University of Heidelberg, Mannheim, Germany; Azienda Ospedaliera Universitaria Careggi, Firenze, Italy; University Campus Bio Medico of Rome, ROMA, Italy; Dept of Medical Oncology, University Campus Bio-Medico, Rome, Italy; Istituto Tumori Milano, Milano, MI, IT; Dept of Pathology, General Hospital of Treviso, Treviso, Italy; Dept of Medical Oncology, Campus Bio-Medico University of Rome, Rome, Italy; Unit of Clinical Oncology, University Cattolica del Sacro Cuore, Rome, Italy; Dept of Cancer Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy.

Publication

J Clin Onc

Summary

We retrospectively reviewed data from all patients treated with low-dose methotrexate(MTX) + vinca alkaloids for FAP-associated DTs in 5 reference centres and cases included into the National rare cancer network were also reviewed and included if sufficiently informative for the study purposes. Radiological responses were assessed using both RECIST and CHOI criteria. We identified 28 patients treated with MTX + vinca alkaloids. All patients had progressive disease before chemotherapy; 17 patients and 9 patients had previously received respectively surgery and/or systemic treatments (i.e. hormone therapy, NSAIDs). Chemotherapy was administered for a median duration of 11 months. According to RECIST criteria (CHOI evaluation is ongoing) complete response, partial response, stable disease, and progressive disease were observed in 1, 17, 10, and 0 patients, respectively. The median progression-free survival (PFS) was 78 months; it was 124 months in responding patients. After chemotherapy withdrawal, MTX + vinca alkaloids rechallenge was offered to 11 patients with progressive disease. In these patients, we obtained a control rate of 100%, resulting in a median second PFS of 64 months. To the best of our knowledge, this is the largest series on the activity of low dose chemotherapy in FAP-related DTs. Our data suggest a tremendous activity of low dose chemotherapy in this very rare subset of patients.

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