Dermatofibrosarcoma Protuberans in Children.

OPINION STATEMENT: Paediatric dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue malignant tumour which displays aggressive local behaviour and has low metastatic potential. The diagnosis is often delayed as DFSP is usually mistaken for other skin conditions, particularly in the early stages of disease. DFSP tends to follow an indolent course after the initial presentation with what is often described as a "rubbery lump". As the disease progresses, the lump tends to enlarge, change colour, and exhibit a more nodular consistency. In rare cases, DFSP can present as an ulcerated exophytic lesion or a depressed area of skin, making diagnosis even more challenging. A high index of suspicion is warranted for early diagnosis, and referral to a specialist unit with expertise in both oncologic resection and reconstruction. DFSP tumours arise from the dermis and grow with finger-like projections. Therefore, in cosmetically sensitive or functionally important locations, an excision and analysis technique that assesses all excision margins is the gold standard of care. Slow Mohs technique performed with en bloc excision is a well-tolerated option for oncologic resection of the tumour. Mohs technique can also be considered but can be challenging in children for reasons explained below. As an alternative, depending on the anatomical location, tumours can be excised with a wide local excision. While an excision technique that incorporates the deep fascia with a 3-cm peripheral margin is acceptable in adults, planning of the excision margin in children should involve consideration of preoperative imaging with MRI, site of the tumour, age, and physical built of the child. Patients should be offered all treatment options considering the local outcomes, available expertise, and cost. A multidisciplinary approach and good communication between team members is crucial. Close collaboration with a pathologist who is familiar with sectioning technique that allows margin control is of paramount importance. Soft tissue reconstruction should be performed immediately after oncologic clearance, although a staged approach may be required. Adjuvant radiotherapy should be avoided in children due to the long-term risk of secondary malignancies and potential for growth disruption.

Rapid redesign and effect on clinical workload of a supra-regional burns and plastic surgery service during the COVID-19 pandemic.

INTRODUCTION: In March 2020, South Wales experienced the most significant COVID-19 outbreak in the UK outside of London. We share our experience of the rapid redesign and subsequent change in activity in one of the busiest supra-regional burns and plastic surgery services in the UK. METHODS: A time-matched retrospective service evaluation was completed for a 7-week "COVID-19" study period and the equivalent weeks in 2018 and 2019. The primary aim of this study was to evaluate plastic surgery theatre use and the impact of service redesign. Comparison between study periods was tested for statistical significance using two-tailed t-tests. RESULTS: Operation numbers reduced by 64% and total operating time by 70%. General anaesthetic cases reduced from 41% to 7% (p<0.0001), and surgery was mainly carried out in ringfenced daycase theatres. Emergency surgery decreased by 84% and elective surgery by 46%. Cancer surgery as a proportion of total elective operating increased from 51% to 96% (p<0.0001). The absolute number of cancer-related surgeries undertaken was maintained despite the pandemic. CONCLUSION: Rapid development of COVID-19 SOPs minimised inpatient admissions. There was a significant decrease in operating while maintaining emergency and cancer surgery. Our ringfenced local anaesthetic Plastic Surgery Treatment Centre was essential in delivering a service. COVID-19 acted as a catalyst for service innovations and the uptake of activities such as telemedicine, virtual MDTs, and online webinars. Our experiences support the need for a core burns and plastic service during a pandemic, and show that the service can be effectively redesigned at speed.

Preoperative versus Post-operative Radiotherapy for Extremity Soft tissue Sarcoma: a Systematic Review and Meta-analysis of Long-term Survival.

BACKGROUND: The role of perioperative radiotherapy in the management of resectable extremity soft tissue sarcoma (ESTS) is widely recognised for local tumour control, wound complications (WC) and long-term function. However, debate continues regarding its implications on long-term survival. This study aimed to determine whether the timing of perioperative radiotherapy affects long-term survival outcomes in adults with ESTS. METHODS: A systematic literature search of MEDLINE, EMBASE, Web of Science and Cochrane was performed. The primary outcome measure was the pooled hazard ratio (HR) at 95% confidence intervals. Secondary outcomes and subgroup analyses were presented as cumulative odds ratios (OR). A random-effects, generic inverse variance method and sensitivity analysis were performed to minimise heterogeneity. RESULTS: Six studies (n = 4192 patients) were identified. Time-to-event analysis demonstrated a statistically significant advantage in post-operative radiotherapy for overall survival (HR 1.15 and p = 0.05). Combined HRs for disease-free (1.25 and p = 0.22) and disease-specific (1.06 and p = 0.43) survival also favoured post-operative radiotherapy but did not achieve statistical significance. Post-operative radiotherapy was shown to confer an overall (OR 1.19 and p = 0.01), disease-free (OR 1.19 and p = 0.01) and disease-specific (OR 1.19 and p = 0.01) survival advantage on subgroup analysis. This survival benefit was best observed at three years in the disease-free survival comparison (OR 1.55 and p = 0.003). Preoperative radiotherapy was associated with more WC (OR 2.74 and p<0.00001). CONCLUSIONS: Pooled analysis of published literature suggests that post-operative radiotherapy confers a significant long-term survival advantage with fewer WC. Further large multicentre randomised controlled trials with long-term follow-up are required to determine the optimal perioperative radiotherapy regime in adult ESTS.

Seeing White: Management of TIVA during autologous breast reconstruction.

Total IntraVenous Anaesthesia is frequently the anaesthetic of choice for enhanced recovery after surgery pathways during breast reconstruction free flap surgery. This relies upon the continuous intravenous infusion of propofol. We describe our experience of two patients where augmentation of a venously congested DIEP flap with a cephalic vein transposition procedure, risked interruption of the intravenous delivery of anaesthesia to the patient. We also share our steps taken to mitigate this risk going forward.

Reduced S100 Protein Expression in Malignant Ossifying Fibromyxoid Tumors: A Case Report.

Ossifying fibromyxoid tumor of soft parts (OFMT) is a rare mesenchymal neoplasm of uncertain lineage. OMFT normally has a benign clinical course, and malignant variants are considered unusual. Criteria defining malignancy have not yet been clearly identified and universally accepted, and there is diagnostic uncertainty between pathologists as to how best to recognize a malignant variant. We present the case of a 68-year-old male patient who, following initial diagnosis of typical OFMT in the left scapular region, presented to the sarcoma service 9 years later with a short history of a solid lesion in the right calf. Biopsy confirmed metastatic OFMT and further imaging identified three other radiologically similar but distant lesions, which were later resected. The histology of the initial biopsy was reviewed, and the original observations were found to be accurate and due to current diagnostic criteria, the specimen was reported as typical. We propose that this case report contributes to a growing body of literature suggesting that negative S100 expression may be a useful feature in identifying and characterizing malignant OFMT.

Use of a Fascia Lata Autograft to Reconstruct a Large Triceps Tendon Defect After En-Bloc Resection of a Soft-Tissue Sarcoma: A Case Report.

CASE: In this report, we present the case of a patient undergoing en-bloc resection of a large triceps soft-tissue sarcoma, requiring reconstruction of the triceps tendon including its distal insertion. We describe a surgical technique using fascia lata (FL) autograft to reconstruct the patient's extensor mechanism with long-term follow-up and functional results. CONCLUSIONS: FL autograft is a viable option for reconstruction of large tendinous defects. It is simple and straightforward to harvest, inexpensive when compared with alternatives, with many potential applications.

Long-Term Outcomes of Smile Reconstruction in Möbius Syndrome.

BACKGROUND: Gracilis muscle transplant is the standard of care for smile reconstruction in children with Möbius syndrome. The authors describe the long-term clinical efficacy, durability, and psychosocial impact of this approach at a mean follow-up of 20 years. METHODS: Patients with Möbius syndrome who underwent gracilis muscle transfer between 1985 and 2005 were included in the study. The authors assessed midfacial movements using photographs and Scaled Measurement of Improvement in Lip Excursion measure, administered the patient-reported Facial Clinimetric Evaluation Scale, and used semistructured interviews. RESULTS: The mean age at surgery was 13.2 ±10.6 years and the mean follow-up was 20.4 ± 4.4 years postoperatively. Twenty-two gracilis muscle transplants were performed, all innervated by the motor nerve to the masseter. Photographic analysis using Scaled Measurement of Improvement in Lip Excursion software demonstrated that movement of the muscle was unchanged 20 years after surgery. Smile symmetry was stable long term. The overall mean Facial Clinimetric Evaluation score of the cohort was 62.3 ± 12.0. Facial comfort (86.4 ± 21.2) and social function (69.9 ± 18.5) subdomains were the highest. Although the facial movement subdomain score was 35.6 ± 12.4, the score related to smiling alone was 84.1 ± 20.2, indicating satisfaction with the midfacial reconstruction. Interview responses indicated high satisfaction with surgery; fulfilled expectations of social acceptance; improved communication; enhanced self-confidence; and a sense of increased facial symmetry, spontaneity, and smile excursion. CONCLUSION: Segmental gracilis muscle transplantation provides long-lasting, durable improvements in objective and patient-reported measures of facial function.

Motivation Measures in Sport: A Critical Review and Bibliometric Analysis.

Motivation is widely-researched, in both sport psychology and other fields. As rigorous measurement is essential to understanding this latent construct, a critical appraisal of measurement instruments is needed. Thus, the purpose of this review was to evaluate the six most highly cited motivation measures in sport. Peer-reviewed articles published prior to August 2016 were searched to identify the six most highly cited motivation questionnaires in sport: Sport Motivation Scale (SMS), Intrinsic Motivation Inventory (IMI), Situational Motivational Scale (SIMS), Perceptions of Success Questionnaire (POSQ), Behavioural Regulation in Sport Questionnaire (BRSQ), and Task and Ego Orientation in Sport Questionnaire (TEOSQ). The questionnaires were then evaluated and discussed in four sections: Development, Reliability, Correlates, and Summary. Bibliometric data were also calculated (average weighted impact factor) and assessed (e.g., citations per year) to evaluate the impact of the use of each questionnaire. Despite some variance in their psychometric properties, conceptualization, structure, and utility, the six questionnaires are psychometrically strong instruments for quantifying motivation that are widely supported in the literature. Bibliometric analyses suggested that the IMI ranks first and the SMS ranks sixth according to the average weighted impact factors of their original publications. Consideration of each questionnaire's psychometric strengths/limitations, and conceptualization of motivation in the context of specific research questions should guide researchers in selecting the most appropriate instrument to measure motivation in sport. The average weighted impact factor of each questionnaire is a useful value to consider as well. With these points in mind, recommendations are provided.

Recurrent meningioma of the scalp after 13†years.

Meningiomas represent some of the most common types of tumour of the central nervous system. They are considered benign, with ∼1 in 1000 known to metastasise. We report a case of recurrent meningioma of the scalp 13 years following craniotomy to treat the primary. The recurrent tumour of the scalp was eventually excised 5 years later and the defect closed with an anterolateral thigh free flap in a joint procedure between the neurosurgeons and plastic surgeons. Histology of the scalp lesion confirmed a WHO grade I meningioma. Although a number of explanations for recurrent scalp meningiomas exist, the precise role of isolated genetic defects in scalp meningiomas is incomplete. The scalp meningioma in this case consisted of a complex karyotype suggestive of more aggressive disease. This case emphasises that cytogenetics may play a greater role in identifying more aggressive tumours than histology alone.