We retrospectively reviewed 30 patients with
a diffuse-type giant-cell tumour (Dt-GCT) (previously known as pigmented
villonodular synovitis) around the knee in order to assess the influence
of the type of surgery on the functional outcome and quality of
life (QOL). Between 1980 and 2001, 15 of these tumours had been
treated primarily at our tertiary referral centre and 15 had been
referred from elsewhere with recurrent lesions. The mean follow-up was 64 months (24 to 393). Functional outcome
and QOL were assessed with range of movement and the Knee injury
and Osteoarthritis Outcome Score (KOOS), the Musculoskeletal Tumour
Society (MSTS) score, the Toronto Extremity Salvage Score (TESS)
and the SF-36 questionnaire. There was recurrence in four of 14
patients treated initially by open synovectomy. Local control was
achieved after a second operation in 13 of 14 (93%). Recurrence
occurred in 15 of 16 patients treated initially by arthroscopic
synovectomy. These patients underwent a mean of 1.8 arthroscopies
(one to eight) before open synovectomy. This achieved local control
in 8 of 15 (53%) after the first synovectomy and in 12 of 15 (80%)
after two. The functional outcome and QOL of patients who had undergone
primary arthroscopic synovectomy and its attendant subsequent surgical
procedures were compared with those who had had a primary open synovectomy
using the following measures: range of movement (114º Those who had undergone open synovectomy needed fewer subsequent
operations. Most patients who had been referred with a recurrence
had undergone an initial arthroscopic synovectomy followed by multiple
further synovectomies. At the final follow-up of eight years (2
to 32), these patients had impaired function and QOL compared with
those who had undergone open synovectomy initially. We conclude that the natural history of Dt-GCT in patients who
are treated by arthroscopic synovectomy has an unfavourable outcome,
and that primary open synovectomy should be undertaken to prevent
recurrence or residual disease. Cite this article:
We have analysed the pattern of symptoms in patients presenting with synovial sarcoma to identify factors which led to long delays in diagnosis. In 35 children, the early symptoms and the results of clinical and radiological investigation were reviewed, along with the presumed diagnoses. The duration of symptoms was separated into patient delay and doctor delay. Only half of the patients had one or more of the four clinical findings suggestive of sarcoma according to the guidance of the National Institute for Clinical Excellence at the onset of symptoms. Of the 33 children for whom data were available, 16 (48.5%) presented with a painless mass and in ten (30.3%) no mass was identified. Seven (21.2%) had an unexplained joint contracture. Many had been extensively investigated unsuccessfully. The mean duration of symptoms was 98 weeks (2 to 364), the mean patient delay was 43 weeks (0 to 156) and the mean doctor delay was 50 weeks (0 to 362). The mean number of doctors seen before referral was three (1 to 6) and for 15 patients the diagnosis was obtained after unplanned excision. Tumours around the knee and elbow were associated with a longer duration of symptoms and longer doctor delay compared with those at other sites. Delays did not improve significantly over the period of our study of 21 years, and we were unable to show that delay in diagnosis led to a worse prognosis. Our findings highlight the variety of symptoms associated with synovial sarcoma and encourage greater awareness of this tumour as a potential diagnosis in childhood.