Myxofibrosarcoma of the extremity and trunk: a multidisciplinary approach leads to good local rates of LOCAL control.

AIMS: Myxofibrosarcomas (MFSs) are malignant soft-tissue sarcomas characteristically presenting as painless slowly growing masses in the extremities. Locally infiltrative growth means that the risk of local recurrence is high. We reviewed our experience to make recommendations about resection strategies and the role of the multidisciplinary team in the management of these tumours. PATIENTS AND METHODS: Patients with a primary or recurrent MFS who were treated surgically in our unit between 1997 and 2012 were included in the study. Clinical records and imaging were reviewed. A total of 50 patients with a median age of 68.4 years (interquartile range 61.6 to 81.8) were included. There were 35 men; 49 underwent surgery in our unit. RESULTS: The lower limb was the most common site (32/50, 64%). The mean size of the tumours was 8.95 cm (1.5 to 27.0); 26 (52%) were French Fédération Nationale des Centres de Lutte Contre le Cancer grade III. A total of 21 (43%) had positive margins after the initial excision; 11 underwent further excision. Histology showed microscopic spread of up to 29 mm beyond macroscopic tumour. Local recurrence occurred in seven patients (14%) at a mean of 21 months (3 to 33) and 15 (30%) developed metastases at a mean of 17 months (3 to 30) post-operatively. CONCLUSION: High rates of positive margins and the need for further excision makes this tumour particularly suited to management by multidisciplinary surgical teams. Microscopic tumour can be present up to 29 mm from the macroscopic tumour in fascially-based tumours. Cite this article: Bone Joint J 2016;98-B:1682-8.

Surgical outcome and patterns of recurrence for retroperitoneal sarcoma at a single centre.

INTRODUCTION: Retroperitoneal sarcoma is a surgically managed condition that can recur locally following macroscopically complete resection. Owing to the low incidence of the condition, advances in treatment are reported infrequently but complete compartmental resection and adjuvant or neoadjuvant radiotherapy are areas under investigation. Given the practical difficulty of randomised trials, observational data can highlight advantages from progressive treatment approaches. METHODS: A retrospective database of consecutive retroperitoneal sarcoma resections performed at a single referral centre between March 1997 and March 2013 was interrogated. Histological, radiological and clinical data were collected. Univariate and multivariate analyses for disease free and overall survival were performed to establish independent predictors of disease recurrence and patient survival. RESULTS: A total of 79 patients underwent 90 resections (63 primary). The mean five-year overall and disease free survival rates were 55.3% and 24.8% respectively. Higher patient age, high tumour grade, presence of extraretroperitoneal disease and invasive tumour phenotype were found to significantly predict survival following multivariate analysis. Half (50%) of the tumours displayed invasive behaviour on histopathology and 42% of locoregional recurrence was intraperitoneal. CONCLUSIONS: Retroperitoneal sarcoma is commonly an infiltrative tumour and often recurs outside of the retroperitoneum. These features limit the therapeutic impact of interventions that focus on gaining local control such as complete compartmental resection and radiotherapy. It seems likely that future advances in the management of this cancer will involve new systemic agents to treat this frequently systemic disease.

Lipoma or liposarcoma? A cautionary case report.

MERRF syndrome (myoclonic epilepsy with ragged-red fibres) is a mitochondrial encephalomyopathy characterised by a mixed seizure disorder and myoclonus. The condition is associated with multiple large cervical lipomas that often require surgical excision. Comprehensive clinical examination combined with pre-operative radiographic imaging is vital in such cases to help differentiate benign fatty masses from potential liposarcomas. We describe a case in which a lipoma-like liposarcoma was identified following excision of what was expected, on clinical and radiological grounds, to be a lipoma. This case highlights the potential for sarcomatous changes in suspicious lipomas, and the key role that imaging plays in differentiating benign from malignant.

Imatinib in gastrointestinal stromal tumour: Northern Cancer Network experience.

UNLABELLED: Imatinib treatment in metastatic or inoperable gastrointestinal stromal tumours (GIST) has shifted the paradigm of treatment of this disease. Successful clinical trials of imatinib led to rapid regulatory approval and, in England and Wales, National Institute for Health and Clinical Excellence (NICE) guidance on use of this technology. NICE recommend detailed audit of their guidelines in clinical practice. This audit reflects that guidance and was designed to document the use of imatinib in routine clinical practice. METHODS: We conducted a retrospective audit of patients with GIST treated with imatinib from 1 February 2002 to 31 March 2007. Information gathered included patient demographics, disease characteristics and details of treatment administered, treatment response, toxicities and follow-up data. The primary objective was to record disease control rate (DCR), defined as a lack of progression on computed tomography at three months. Secondary end points of this audit were progression-free and overall survival. These were compared with published clinical trial results. RESULTS: Thirty-six consecutive patients with a diagnosis of GIST treated with imatinib were identified. Median age of patients was 70.1 years. At the time of analysis, patients have been followed up for a median of 41.6 months. In total, patients were treated for a median of 15.8 months. Treatment was generally well tolerated with a small percentage of patients experiencing grade 3/4 toxicities. Disease control was observed in 30 patients (DCR, 83.3%, 95% CI 67.2-93.6, intention to treat analysis). The median progression free survival (PFS) in this cohort was 23.7 months (95% CI 12.9-34.4); while the median overall survival was 39.7 months (95% CI 22.8-56.5). CONCLUSION: Our data demonstrated that the treatment of unselected GIST patients within the NICE guidance compares favourably to previously published data of randomized registration studies of imatinib. Of note, the median age of this cohort is some ten years older than that reported in the trials. Imatinib was well tolerated with acceptable treatment-related adverse events.

A rare cause of spinal cord compression: imaging appearances of gout of the cervical spine.

Gout is a metabolic disorder typically affecting the peripheral joints, more commonly in males. Spinal involvement is uncommon and is usually associated with hyperuricemia. We present the imaging findings of a case of spinal gout in a female patient with no previous history of hyperuricaemia, involving multiple spinal segments.

Congenital multifocal inflammatory pseudotumor: a case report.

Inflammatory pseudotumor is an uncommon benign tumor found in various sites throughout the body. The etiology is unclear with evidence to support both neoplastic and infective processes. The authors present a case with several unique features. It represents the first reported case detected antenatally. The tumor was found separately within the abdomen and thorax. The thoracic component of the tumor was managed without resection or ablative treatment but showed spontaneous regression.