Introduction. Fibromatosis is a disorder characterised by a spectrum of biological behaviour from relative indolence to aggressive local infiltration. With aimed to describe the pre and post-operative functional status of these patients managed with surgery and analyse the effect of radiotherapy on functional outcome. Methods. 43 patients were analysed in the upper and lower limb fibromatosis database in which functional data was available pre-op and at a minimum of two years post-op. Any plantar, palmer, chest or abdominal lesion was excluded as were hormonal or chemotherapy treated patients. Results. 20 men and 23 women aged from 18 to 71 with a median age of 44 were analyzed. 36 patients had no prior resective operative intervention. 7 patients had undergone an attempted resection procedure at another unit of which 7 had locally recurred. 40 of the lesions were classified as deep and 3 superficial. 17 were located in the lower limb and 26 upper in the limb. XRT was used in 2 in the post-operative period, 35 in the pre-operative period and 6 patients did not receive XRT. 16 operations produced a margin negative resection and 27 were positive. Follow-up ranged from 24 to 168 months. In the upper limb the median MSTS score was 86.6 (range 50-100) and the TESS was 93 (range 56-100). In the lower limb the median MSTS was 90.8 (range 71-100) and the TESS 89.2 (range 58-100). There were no significant predictors for pre and post-op TESS patient reported outcomes. The MSTS post-op score was significantly associated with the pre-op score (p=0.01). Conclusions. The best predictor of the post-op MSTS value is the pre-op score in the resection of fibromatosis. Radiation does not have a significant effect on the functional outcome of patients treated surgically for fibromatosis at a minimum of two years follow-up

Fibromatosis represent a highly heterogeneous group of tumours in growth pattern, location and management. Our aim was to describe the demographics of the patient population who had undergone surgical resection and to identify predictors of local recurrence. Any lesion that was infiltrating the chest or abdominal cavity was excluded. Patients were also not included if they had a plantar or palmar lesions or had received hormonal or chemotherapy. 67 men and 88 women aged from 16 to 77 with a median age of 39 were analyzed. 121 patients had no prior resective operative intervention. 34 patients had undergone an attempted resection procedure at another unit of which 30 had locally recurred. 3 were located in the abdominal wall, 5 chest wall, 15 paraspinal, 56 lower and 76 upper limb. 40 patients did not receive XRT, 18 in the post-operative period and 97 in the pre-operative period. 67 operations produced margin negative resection, 85 were positive and 3 in which the margin status was unknown. Follow-up ranged from 1 day post op to 23.3 years. 23 patients had a local recurrence. Following subsequent re-resections, the total number of patients who were alive with evidence of disease was 16. 6 pts had deceased. 149 were alive with no evidence of disease. No factors were found to be statistically significant for predicting local recurrence, including the use of radiation (0.06) and margin status (0.81). Although radiation, given either pre or post-operatively did trend towards preventing local recurrence (HR 0.40; 95% CI 0.15 to 1.06; p = 0.06). Conclusions. The retention of critical structures whilst resecting fibromatosis continues to be an appropriate management strategy, as local recurrence rates seem to be independent of margin status. Although not statistically significant, the use of XRT did tend towards reducing local recurrence

In patients with a tumour affecting the distal ulna it is difficult to preserve the function of the wrist following extensive local resection. We report the outcome of 12 patients (nine female, three male) who underwent excision of the distal ulna without local soft-tissue reconstruction. In six patients, an aggressive benign tumour was present and six had a malignant tumour. At a mean follow-up of 64 months (15 to 132) the mean Musculoskeletal Tumour score was 64% (40% to 93%) and the mean DASH score was 35 (10 to 80). The radiological appearances were satisfactory in most patients. Local recurrence occurred in one patient with benign disease and two with malignant disease. The functional outcome was thus satisfactory at a mean follow-up in excess of five years, with a relatively low rate of complications. The authors conclude that complex reconstructive soft-tissue procedures may not be needed in these patients.

Cite this article: Bone Joint J 2014;96-B:1392–5.

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.