Tenosynovial giant cell tumour/pigmented villonodular synovitis: Outcome of 294 patients before the era of kinase inhibitors

Emanuela Palmerini, Eric L. Staals, Robert G. Maki, Stefano Pengo, Angela Cioffi, Marco Gambarotti, Piero Picci, Primo Andrea Daolio, Antonina Parafioriti, Carol Morris, Cristina R. Antonescu, Alessandro Gronchi, Paolo Giovanni Casali, Davide M. Donati, Stefano Ferrari, Silvia Stacchiotti

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56 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)210-217
Number of pages8
JournalEuropean Journal of Cancer
Issue number2
StatePublished - Jan 2015


  • Pigmented villonodular synovitis
  • Prognostic factors
  • Tenosynovial giant cell tumour
  • Treatment
  • Tyrosine kinase inhibitors

ASJC Scopus subject areas

  • Oncology
  • Cancer Research


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