title: Tenosynovial giant cell tumour/pigmented villonodular synovitis: outcome of 294 patients before the era of kinase inhibitors.
abstract: BACKGROUND
  Tenosynovial giant cell tumour/pigmented villonodular synovitis (TGCT/PVNS) is a benign neoplasm of synovium and tendon sheath. We conducted a retrospective pooled analysis in three major referral centers.
  
  
  METHODS
  Patients treated between 1998 and 2008 were examined. Only patients presenting with primary disease or first relapse were included. 5-year local failure free survival (5-year-LFFS) was analysed.
  
  
  RESULTS
  294 patients were included: 254 with new diagnosis and 40 in 1st local recurrence (171 F/123 M; median age: 36 years; tumour size ⩽2 cm in 27% of patients, >2 to ⩽5 cm in 41%, and >5 cm in 32%). A diffuse pattern was reported in 69%, localised in 31%. No metastases were documented. Local failure (LF) was reported in 28% of patients: 36% in diffuse pattern, 14% in localised (p = 0.002); median time to LF: 16 months. With a median follow-up of 4.4 years, 5-year-LFFS was 66%, with multiple (up to five) local recurrences in 40% of relapsed patients. Size <2 cm, macroscopically complete resection, female gender and new diagnosis were associated with a better local control. After multivariate analysis, a previous relapse was independently associated with local failure.
  
  
  CONCLUSIONS
  This study underlines the propensity of TGCT/PVNS to multiple local recurrences. In absence of clinical factors, biological studies are needed to identify prognostic factors of local failure. After a first local recurrence, surgery does not seem to have a curative potential. In these high risk patients, studies addressing the role of target therapies are needed.
authors.affiliation: Chemotherapy, Musculoskeletal Oncology Department, Istituto Ortopedico Rizzoli, Bologna, Italy.
authors.forename: Emanuela
authors.initials: E
authors.lastname: Palmerini
authors.affiliation: Orthopaedic Surgery, Musculoskeletal Oncology Department, Istituto Ortopedico Rizzoli, Bologna, Italy. Electronic address: eric.staals@ior.it.
authors.forename: Eric L
authors.initials: EL
authors.lastname: Staals
authors.affiliation: Departments of Medicine, Pediatrics, and Orthopaedics, Mount Sinai School of Medicine, NY, USA.
authors.forename: Robert G
authors.initials: RG
authors.lastname: Maki
authors.affiliation: Sarcoma Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Pengo
authors.affiliation: Department of Medicine, Memorial Sloan-Kettering Cancer Center, NY, USA.
authors.forename: Angela
authors.initials: A
authors.lastname: Cioffi
authors.affiliation: Surgical Pathology, Musculoskeletal Oncology Department, Istituto Ortopedico Rizzoli, Bologna, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Gambarotti
authors.affiliation: Laboratory of Experimental Oncology, Musculoskeletal Oncology Department, Istituto Ortopedico Rizzoli, Bologna, Italy.
authors.forename: Piero
authors.initials: P
authors.lastname: Picci
authors.affiliation: Istituto Ortopedico Gaetano Pini, Milano, Italy.
authors.forename: Primo Andrea
authors.initials: PA
authors.lastname: Daolio
authors.affiliation: Istituto Ortopedico Gaetano Pini, Milano, Italy.
authors.forename: Antonina
authors.initials: A
authors.lastname: Parafioriti
authors.affiliation: Department of Medicine, Memorial Sloan-Kettering Cancer Center, NY, USA.
authors.forename: Carol
authors.initials: C
authors.lastname: Morris
authors.affiliation: Department of Medicine, Memorial Sloan-Kettering Cancer Center, NY, USA.
authors.forename: Cristina R
authors.initials: CR
authors.lastname: Antonescu
authors.affiliation: Sarcoma Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Gronchi
authors.affiliation: Sarcoma Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
authors.forename: Paolo Giovanni
authors.initials: PG
authors.lastname: Casali
authors.affiliation: Orthopaedic Surgery, Musculoskeletal Oncology Department, Istituto Ortopedico Rizzoli, Bologna, Italy.
authors.forename: Davide M
authors.initials: DM
authors.lastname: Donati
authors.affiliation: Chemotherapy, Musculoskeletal Oncology Department, Istituto Ortopedico Rizzoli, Bologna, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Ferrari
authors.affiliation: Sarcoma Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
authors.forename: Silvia
authors.initials: S
authors.lastname: Stacchiotti
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D005870:Giant Cell Tumors / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009364:Neoplasm Recurrence, Local; D017063:Outcome Assessment, Health Care / Q000379:methods / Q000706:statistics & numerical data; D011379:Prognosis; D012189:Retrospective Studies; D000074402:Synovectomy; D013583:Synovial Membrane / Q000473:pathology*; D013586:Synovitis, Pigmented Villonodular / Q000473:pathology* / Q000601:surgery; D013710:Tendons / Q000473:pathology* / Q000601:surgery; D013997:Time Factors; D055815:Young Adult
keywords: Pigmented villonodular synovitis; Prognostic factors; Tenosynovial giant cell tumour; Treatment; Tyrosine kinase inhibitors