Desmoid tumours are a rare fibroblastic proliferation of monoclonal origin, arising in deep soft-tissues. Histologically, they are characterized by locally aggressive behaviour and an inability to metastasize, and clinically by a heterogeneous and unpredictable course. Desmoid tumours can occur in any anatomical site, but commonly arise in the limbs. Despite their benign nature, they can be extremely disabling and sometimes life-threatening, causing severe pain and functional limitations. Their surgical management is complex and challenging, due to uncertainties surrounding the biological and clinical behaviour, rarity, and limited available literature. Resection has been the first-line approach for patients with a desmoid tumour but, during the last few decades, a shift towards a more conservative approach has occurred, with an initial ‘wait and see’ policy. Many medical and regional forms of treatment are also available for the management of this condition, and others have recently emerged with promising results. However, many areas of controversy remain, and further studies and global collaboration are needed to obtain prospective and randomized data, in order to develop an appropriate shared stepwise approach.

Cite this article: Bone Joint J 2023;105-B(7):729–734.

Aims

Severe spinal deformity in growing patients often requires surgical management. We describe the incidence of spinal deformity surgery in a National Health Service.

Recent evidence suggests that both the accepted mechanism of blast-mediated traumatic amputation (TA) (shockwave then blast wind exposure) and the link with fatal shockwave exposure merit review. Searching UK military prospectively gathered trauma registry data and post mortem CT (PM-CT) records identified casualties from August 2008 to August 2010 with blast-mediated TAs. TA level and associated injuries were recorded. Data on pre-debridement osseous and soft tissue injuries were only consistently available for fatalities through PM-CT imaging. 146 Cases (75 survivors and 71 fatalities) with 271 TAs (130 in survivors and 141 in fatalities) were identified. Through-joint TA rate in fatalities was 34/141 (24.1%). PM-CT analysis demonstrated only 9/34 through joint TAs with contiguous fractures in the immediately proximal long bone/limb girdle. 18/34 had no fracture, and 7/34 had a non-contiguous fracture. The previously reported link between TA and blast lung injury was not present, calling into question the significance of shockwaves in generating blast-mediated TAs. Furthermore, contemporary blast injury theory cannot account for the high prevalence of through joint TAs (previously published rate 1.3%). The proportion of through joint TAs with no associated fracture or a non-contiguous fracture (74%) is supportive of pure flail as a mechanism for blast-mediated TA

We compared case-mix and outcome variables in 1310 patients who sustained an acute fracture at the age of 80 years or over. A group of 318 very elderly patients (≥ 90 years) was compared with a group of 992 elderly patients (80 to 89 years), all of whom presented to a single trauma unit between July 2007 and June 2008. The very elderly group represented only 0.6% of the overall population, but accounted for 4.1% of all fractures and 9.3% of all orthopaedic trauma admissions. Patients in this group were more likely to require hospital admission (odds ratio 1.4), less likely to return to independent living (odds ratio 3.1), and to have a significantly longer hospital stay (ten days, p = 0.01).

The 30- and 120-day unadjusted mortality was greater in the very elderly group. The 120-day mortality associated with non-hip fractures of the lower limb was equal to that of proximal femoral fractures, and was significantly increased with a delay to surgery > 48 hours for both age groups (p = 0.04). This suggests that the principle of early surgery and mobilisation of elderly patients with hip fractures should be extended to include all those in this vulnerable age group.

Background: The literature on description and management of advanced fungating soft tissue tumours (FSST) is limited because of the rarity of cases. Recent advances in diagnostic resources and an increased awareness of the disease has made early recognition easier. Manchester Royal Infirmary is a Regional Sarcoma Centre in the North West of England. We describe our experiences in managing patients with FSST of the extremities.

Patients and Methods: Between 1997 and 2007, 18 patients presented with FSST of the extremities (13 involving the lower limb, and 5 involving the upper limb), and 1 patient with a sarcoma involving the scapular region (limb girdle). The cohort included 14 males and 5 females with a mean average age of 68.5 ± 13.7 years. Follow-up ranged from a minimum of 6 months to 10 years from the initial referral.

Results: The histological diagnosis was sarcoma in 15 patients, subclassified into spindle cell sarcoma (4), fibrous histiocytoma (2), pleomorphic sarcoma (3), liposarcoma (2), leiomyosarcoma (2), fibrosarcoma (1) and round cell sarcoma (1). In the remaining 3 patients immunohistochemistry studies confirmed a metastatic squamous cell sarcoma, a metastatic malignant melanoma and a metastasis from a poorly differentiated upper gastrointestinal malignancy. Lung metastases were present at the time of referral in 6 patients and developed later during follow-up in 4 patients.

For patients where curative surgery was an option, primary wide local excision (15 patients) or primary amputation (2 patients) was performed. The remaining 2 patients presented with unresectable disease due to the location and localised spread; an embolisation was performed for palliation in both cases. Revision surgery was needed in 9 patients for either a positive resection margin confirmed by histology, or a recurrence; these included 3 secondary amputations. A histologically proven recurrence occurred in 6 patients after an average of 15.8 (4 to 41) months. Local adjuvant radiotherapy was administered to 7 patients and a combination of radio–and chemotherapy was used in 2 patients for metastases. Mortality was 53% (9 patients) by the end of 36 months follow-up period.

Conclusion: Fungation in soft tissue tumours is a rare phenomenon and often a sign of locally advanced disease with a high grade nature. Patients present with either systemic spread, or have a tendency to develop metastases despite good local disease control. Primary wide local excision is difficult with a high chance of a positive margin; hence primary amputation may be better for local clearance. Tumour recurrence and revision surgery, however, is common. We report a mortality rate of > 50% at the end of 3 years from presentation to treatment.

Purpose: The literature on management of advanced soft tissue tumours is limited because of the rarity of cases following increased awareness and improved diagnostic resources.

Method: Our experience of managing 18 patients with fungating soft tissue tumours of the extremities and one patient with a sarcoma involving the scapular region (limb girdle) is presented. There were 14 males and 5 females. Average age was 70.6 yrs ranging between 37 – 98 years. 13 tumours involved lower limb and 6 the upper limb.

Results: The follow-up ranged from a minimum of 6 months to 10 years from the initial referral. The histological diagnosis was Sarcoma in 15 patients (Spindle cell sarcoma in 4, Fibrous Histiocytoma in 2, Pleomorphic sarcoma in 3, liposarcoma in 2, leiomyosarcoma in 2, Fibrosarcoma in 1 and 1 Round cell sarcoma). In the remaining 3 patients immunohistochemistry studies confirmed a Metastatic Squamous cell Sarcoma, a Metastatic Malignant Melanoma and a Metastases from a poorly differentiated upper GI malignancy each. Primary wide local excision was performed in 15 patients and primary amputation was performed in two patients. In 2 patients when tumour was unresectable due to the location and local spread, an embolisation was performed in both for palliation. Lung Metastases were present at the time of referral in 6 patients and developed later during follow-up in 4 patients. A histologically proven recurrence occurred in 6 patients after an average of 15.83 (4 to 41) months. Revision surgery was needed in 9 patients for either a positive margin on histology or a recurrence, including 3 secondary amputations. Local adjuvant Radiotherapy was given for 7 patients and a combination of radio and chemotherapy was used in 2 patients for metastases. Mortality was 53 % (9 patients) by the end of 32 months of follow-up.

Conclusion: Fungation in soft tissue tumours is rare and often a sign of locally advanced disease and a high grade nature, patients either have systemic spread by the time or develop later inspite of good local disease control. Primary wide local excision in such patients is difficult and has a high chance of a positive margin hence primary amputation may be better for local clearance. Recurrence of tumour and revision surgery is common and the mortality was > 50% at the end of 3 years from presentation to treatment in our series.

This study reports the experience of one treatment centre with routine surveillance MRI following excision of musculoskeletal sarcoma. The case notes, MRI and histology reports for 57 patients were reviewed. The primary outcome was local tumour recurrence detected on either surveillance MRI in asymptomatic patients, or interval MRI in patients with clinical concern. A total of 47 patients had a diagnosis of soft-tissue sarcoma and ten of a primary bone tumour. A total of 13 patients (22%) had local recurrence. Nine were identified on a surveillance scan, and four by interval scans. The cost of surveillance is estimated to be £4414 per recurrence detected if low-grade tumours with clear resection margins are excluded. Surveillance scanning has a role in the early detection of local recurrence of bone and soft-tissue sarcoma.