1452P - Multidisciplinary treatment outcome of Desmoid-type fibromatosis (DTF). A registry-based study from Spanish Group for Research on Sarcoma (GEIS)

Date 29 September 2014
Event ESMO 2014
Session Poster Display session
Topics Anti-Cancer Agents & Biologic Therapy
Soft Tissue Sarcomas
Surgery and/or Radiotherapy of Cancer
Presenter Javier Martinez-Trufero
Citation Annals of Oncology (2014) 25 (suppl_4): iv494-iv510. 10.1093/annonc/mdu354
Authors J. Martinez-Trufero1, R. Alvarez Alvarez2, A. Lopez Pousa3, I. Sevilla4, P. Sancho Marquez5, C. Orbegoso6, J. Cruz Jurado7, M.A. Sala Gonzalez8, M.J. Blanco Sanchez9, C. Valverde Morales10, J.A. Pérez-Fidalgo11, J.P. Berros12, J. Martinez García13, J.L. Arranz14, A. Meana15, J.A. López-Martín16, J. Rubio17, J.M. Cano18, I. Juez-Martel19, J. Martin Broto20
  • 1Medical Oncology, Hospital Miguel Servet, 50009 - Zaragoza/ES
  • 2Medical Oncology, hospital gregorio marañon, madrid/ES
  • 3Medical Oncology, Hospital de la Sta. Creu i St. Pau, ES-08025 - Barcelona/ES
  • 4Medical Oncology, Virgen de la Victoria University Hospital, Malaga/ES
  • 5Medical Oncology, Hospital Virgen del Rocio, sevilla/ES
  • 6Medical Oncology, Hospital Marqués de Valdecilla, santander/ES
  • 7Medical Oncology Dept. 6a Planca, Hospital Universitario de Canarias, 38320 - San Cristobal de la Laguna/ES
  • 8Medical Oncology, UNIVERSITARY HOSPITAL OF BASURTO, 48013 - BILBAO/ES
  • 9Medical Oncology, Hospital Dr Negrín, Las Palmas/ES
  • 10Oncologia Médica, Vall d'Hebron University HospitalInstitut d'Oncologia, ES-08035 - Barcelona/ES
  • 11Medical Oncology, H Clinico Universitario de Valencia, valencia/ES
  • 12Oncologia Medica, Hospital Universitario Central de Asturias, ES-33006 - Oviedo/ES
  • 13Medical Oncology, Hospital Virgen de la Arrixaca, Murcia/ES
  • 14Medical Oncology, Fundación Jimenez Diaz, Madrid/ES
  • 15Medical Oncology, U G U D´Alacant, alicante/ES
  • 16Medical Oncology, '12 de Octubre' University Hospital, 28041-MADRID - MADRID/ES
  • 17Oncology, ICO Girona, Girona/ES
  • 18Clinical Oncology, Hospital General Ciudad Real, 13005 - Ciudad Real/ES
  • 19Medical Oncology, Hospital de Fuenlabrada, Madrid/ES
  • 20Medical Oncology, Hospital Universitario Son Espases, ES-07010 - Palma de Mallorca/ES

Abstract

Aim

We analyzed retrospectively data about treatment outcome with existing treatments in DTF.

Methods

Descriptive analysis of data related to diagnosis and treatment from all DTF was collected in patients (p) between Sept. 1999 and Nov. 2013 in 26 hospitals of GEIS . Ethics committee approval was obtained.

Results

185 patients. Age: median 37 years (6-85) .63.2% female .Median time lapse from first symptom to diagnosis: 4 months(m). Location : trunk wall 81p (43.8%), extremities 50p (27%), retroperitoneum 10p (5.4%), Gastro-intestinal 16p (8.6%), Head/neck 6p( 3.2%), others 6p(3.2%), 2p (1.1%) missing . 20p (11%) presented a second neoplasia (throughout the whole process). Median tumor size: 8 cm(range 1-96). 3 p (1.6%) presented distant peritoneal disease. First treatment : No treatment 6p (2.7%), Surgery (S) alone 144 p ( 77.8%, 3p >1), S+radiotherapy (RT) 7p (3.7%), S+ chemotherapy (CT) 2 p (1.08%), S+hormonetherapy (HT) 3p (1.62%), S +HT+ Non steroidal antiinflammatory drug (NSAID)+Tirosin-kinasa inhibitor(TKI) 1p (0.54%), CT 5p (2.7%), RT 3p ( 1.6%),NSAID 5p (2.6%), HT 2p (1.08%), TKI 1p(0.54%), HT+NSAID 6p (2.7%). 128p (69.2%) became free of disease, in 49p (26.5%) residual disease remained . 61p (33%) progressed. Median Progression free survival (PFS) :109 m (95%CI 44.6-175.1).Multivariate analysis for PFS: extended/radical surgery(p=0.000) and tumor size<8 cms (p=0.001) predicted independently better PFS.Median follow-up: 36 m . 4p (2.1%) has died (1p tumor-related, 3p other causes), 113p (61.1%) are alive without disease, 66p (35.7%) alive with disease, 2p (1.1%) lost . Secondary treatments(ST): 61p 2nd line, 36p 3rd line, 40p further than 4th line. ST: 59 S, 29 CT, 17 RT, 31 other (11p TKI, 7p NSAID,6p HT, 2p NSAID+HT,1p NSAID+TKI, 4p unknown).

Response to CT /other treatments

CT treatment Other treatments (TKI, HT, NSAID)
p % p %
PR 12 30.7 4 7.4
SD 15 38.4 31 55.3
PD 3 7.6 10 17.8
NE 4 10.2 6 10.7
NA 5 12.8 5 8.9
Total 39 100 56 100

PR: partial response; SD: stable disease; PD: progressive disease; NE: non evaluable; NA: non available data.

Conclusions

Surgical quality and tumor size seems to play a relevant role in predicting PFS in DFT. Other sistemic treatments showed meaningful activity in progressive disease.

Disclosure

All authors have declared no conflicts of interest.