Purpose of the study. Fibromatosis is a benign, but locally aggressive tumour. We had a series of patients who had a high rate of recurrence though they had a wide surgical excision. The question raised was whether there are newer treatment modalities with a higher success rate. We did a retrospective study and review of the literature in order to see if there was anything new that can help us reduce recurrences. Materials and Methods. A retrospective study of all patients who presented with histologically confirmed fibromatosis at an orthopaedic practice in the past 19 years was conducted. Age of the patient at first presentation; sex; tumour site; surgery performed; histological results; first line of treatment and recurrence rate were reviewed. Patients were also contacted telephonically in order to know if they had any recurrence that was managed by another orthopaedic surgeon. Results. We evaluated 17 patients of which 8 were males and 9 females. The mean age was 25.87 years (range 2–52 years). All of the primary sites were extra abdominal. Median follow up was 3.9 years (0–9) with a mean recurrence rate of 2.3 times. All the patients were treated with a wide marginal surgical excision without adjuvant therapy. Conclusion. Fibromatosis has a high recurrence rate with our current treatment modalities. Complete surgical excision does not guarantee a good outcome. A wide variety of treatment modalities are available. Non-surgical treatment includes: hormonal therapies; NSAIDs; chemotherapy; and radiotherapy. Wide surgical excision is the mainstay of treatment but a multidisciplinary approach is necessary in order to optimize the efficacy of our treatment. Level of evidence: Level III. NO DISCLOSURES

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

Plantar fibromatosis is a relatively rare disease compared to its counterpart in the hand. Though it is considered to be a part of Dupuytrens diathesis it has been less exhaustively studied to enable evidence based management strategies.

We followed up all patients presenting with plantar fibromatosis to our institute between 1980 and 2006, identifying 41 patients. 6 patients were lost to followup. Thirty-five patients with 60 involved feet were included in the study. There were 22 males and 13 females, all white Caucasians. The median age at presentation was 45 (19–63 years), and the median follow up was 10 years (2–25 years)

Twenty-one of our patients had palmar Dupuytren’s disease, six had knuckle pads, four had Peyronie’s disease, four had other superficial fibomatoses and two keloids. Six were diabetic, four had epilepsy of whom two took valproate and one phenobarbitone. Eight patients had a family history of fibromatoses.

The most common presentation was a painful lump (20); 13 patients had a painless lump (13) and two had only pain. All patients reported a proliferative phase of enlarging nodule size, often with pain, which lasted 1–4 years (median 2 years). Thereafter most patients reported improvement in symptoms (size of lump and pain) as well as function. As we came to recognise this, we treated most patients with symptomatic measures and observation only. At review, 17 patients considered their symptoms were improving, 14 were stable and only four had noticed deterioration. Seven patients, mostly early in the series, were treated by wide excision; six had recurrence at review although only one was symptomatic.

Plantar fibromatosis is a benign condition which stabilises and may improve after an initial proliferative phase lasting about two years Most patients require no intervention.

Introduction: Aggressive fibromatosis is a benign but locally aggressive process. It arises from musculo-aponeurotic tissues, and invades locally without respect for tissue planes, surrounding vessels and nerves, which makes treatment of local recurrences difficult.

Aims: Our aim is to review our experience in the management of aggressive fibromatosis, focussing on the cases of multiple recurrences, as well as to evaluate the need for disabling surgery.

Material and methods: We present the series of 33 patients (15 male and 18 female) diagnosed of aggressive fibromatosis treated between 1993 and 2003; the follow-up period was no less than two years. The locations were shoulder girdle (8), lower extremity (8), upper extremity (6), gluteus (5), paravertebral (4) and thorax (2). There were 6 cases with 3 or more episodes of local recurrences; in these cases, depending on location and size, and considering high surgical morbidity, associations of radiotherapy, chemotherapy and hormone therapy were given, avoiding disabling surgery.

Results: With an average follow-up of 32 months (25 to 50), there were no deaths and, in the 6 cases of multiple recurrences, there were no amputations. The control MRI demonstrated stability of the process in 5 out of 6 cases, and minimum growth without clinical correlation in the other one. In the other 27 cases, there were 14.8% surgical treated local recurrences, with no need for amputation.

Conclusion: We consider that cases of multiple recurrences of aggressive fibromatosis benefit from adjuvant treatment (radiotherapy, chemotherapy and hormone therapy), avoiding disabling surgery, which is unnecessary following our criteria. This requires strict clinical and radiological control.

Desmoid tumour is an histological benign tumour. Nevertheless, peri-scapular relapses can decrease the function and intra thoracic progression threaten life. To prevent these complications, damaging treatment (radiotherapy, amputation) are sometimes proposed. To precise the optimal indications of treatment, we reviewed our cases.

Patients: from 1984 to 2008, we treated 11 patients with peri-scapula fibromatosis (mean age 42 (13–58)). Only 4 patients were seen at first hand, 7 for relapses (3 of them after radiotherapy).

Treatment was adapted to each patient, in function of age, history of illness, and risks of spontaneous evolution. En bloc extratumoral resection was performed each time, when it didn’t expose to heavy functional risk (8). The other patients were treated by contaminated resection, but never invaliding. 4 patients received pre or/and post-operative chemotherapy. 1 received Interferon alpha, and 7 tamoxifen.

Results: with a median follow-up 15 years 3 months, 7 patients suffered of recurrence. No patient died from disease (thoracic complications) or therapeutic complication9 patients are in complete remission and 2 in stable disease. Following radiotherapy, local relapses (7 cases) and repeated surgery, functional sequellaes are numerous: 2 circumflex nerve palsies, 3 articular stiffness. Major functional sequellaes came from radiotherapy (limb discrepancy, lung and thorax deformity, skin and muscle atrophy.

Conclusion: in this non predictable illness, therapeutic indications should individually balance risks of spontaneous evolution and of complications of treatment. Besides surgery, needed in fast all cases, but often insufficient, it must be considered the value of interferon, tamoxifen and/or chemotherapy. The most important concept is the necessity to treatment avoiding late sequellaes and particularly radiotherapy or mutilating surgery.

Objective: This study was performed to review the current treatment and outcome of extra abdominal fibromatosis in our hospital, supplemented by a current review of the literature.

Method: A retrospective study of 72 patients with fibromatosis seen at the Royal National Orthopaedic Hospital (RNOH) between 1980 and 2009 was performed. Patients were identified using the databases at the peripheral nerves injury (PNI) unit and the histopathology department. Medical and radiological records were reviewed.

Results: There were 72 patients treated at the Sarcoma and PNI units. 40 patients were primary referrals, and 32 more had operations at the referring hospital. An operation was not carried out in 5 patients. 48 patients were treated by operation alone and this was supplemented by adjuvant therapy in 19 patients. Recurrence was seen in 24 (50.0%) of the operation alone group and 10 (52.6%) in the operation and adjuvant therapy group. The rate of recurrence was lower with complete excision. However, complete excision was impossible in some cases because of extension into the chest or spinal canal, or involvement with the axial vessels and lumbosacral or brachial plexus.

Conclusion: We suggest that operative excision should seek to preserve function and that supplementary adjuvant therapy may reduce the risk of recurrence, although excision margin appears to be the most important factor. The aggressive, infiltrative behaviour of deep fibromatoses and the associated genetic mutations identified, clearly distinguish them from the superficial fibromatoses and makes their treatment more difficult and dangerous, especially where vital structures are involved. We agree with the recent recommendation that these lesions should be treated in regional soft tissue sarcoma units.

The stiffness of the shoulder can result of many illness. Nevertheless, we observed a severe stiffness of the scapulo thoracic space only in fibromatosis. To precise the real diagnostic value of this symptom, we examined patients with different diseases of shoulder (tumoral and non tumoral).

The passive mobility of the shoulder of 11 patients with peri-scapular fibromatosis was compared to the mobility of those in 50 patients with non tumoral diseases of shoulder (arthritis and rotator cuff pathology), 50 peri-scapular soft tissues tumours, and 100 patients with primitive or secondary malignancies of humerus or scapula.

Results: in 10 of 11 patients with peri-scapular fibromatosis, the passive mobility of the scapulo thoracic spacewas severely impaired (less than 20°). In non tumoral pathology of shoulder, the passive mobility of the shoulder is frequently impaired but the stiffness hangs only on scapulo humeral articulation. In metastases, sarcoma and soft tissue tumour (except fibromatosis) the passive mobility of the scapulo-humeral joint is usually preserved and the mobility of the scapulothoracic space is always normal even in very huge tumours.

After treatment of fibromatosis, 9/11 patients are in complete remission and the mobility of their scapulo thoracic space restored. 2 patients are in stable disease and one suffers of a residual stiffness of the scapulothoracic space.

We conclude that the frozen scapulo thoracic is a specific symptom of peri scapulo thoracic fibromatosis. The restoration of the mobility of the scapulo thoracic after cure of the desmoid tumour confirms its specific role and represents a good marker of the tumoral evolution.

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 report the case of an 82-year-old man who underwent fasciectomy for a severe Dupuytren’s contracture, during which an ossified lesion was encountered within the contracture and surrounding the neurovascular bundle. The abnormal tissue was removed with difficulty and heterotopic ossification was confirmed histologically. We believe this is the first report of heterotopic ossification in Dupuytren’s disease.

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.