Primary vulval melanoma and genital lichen sclerosus.

Background: Lichen sclerosus (LS) is a chronic, inflammatory skin disease with a predilection for the genitalia. Although, the association between squamous cell cancer and genital LS is well established, a link with genital melanoma has not been thoroughly explored. However, we have recently published a case series of penile melanoma where 9/11 (82%) of patients seen over a 10 year period with penile melanoma were retrospectively found to have histological and/or clinical evidence of genital LS on review. Objectives: The aim of this study was to illuminate further the relationship between vulval melanoma and genital LS by reviewing all the cases managed by our hospital and undertaking a literature review. Methods: We identified all the cases with a diagnosis of vulval melanoma over a 16-year period (2006-2022) where histology was available. The clinical notes were retrospectively reviewed, and the histological features of all cases were reassessed by two independent mutually 'blinded' histopathologists. We also performed a literature review of genital LS in patients with vulval melanoma. Results: A total of 11 patients with vulval melanoma were identified for the review. Histopathological review found evidence of genital LS in seven of them (64%). Genital LS was not documented in any of the original histology reports. Clinical notes and letters were available in nine cases. The literature review identified 12 relevant studies with a total of 18 patients. Twelve cases concerned adult women, and six concerned female children. Conclusion: The presence of genital LS in as high as 64% of our vulval melanoma cases might indicate a causative relationship between genital LS and vulval melanoma. The pathogenesis of vulval melanoma remains largely unknown. Although ultraviolet radiation is an important pathogenic factor for cutaneous melanoma, it cannot be a factor in vulval melanoma. While possible mechanisms behind this association remain unclear, it is possible that chronic inflammation from genital LS leads to melanocytic distress and increased mutagenesis.

Primary penile melanoma and genital lichen sclerosus.

Background: There is a well-established association between squamous cell cancer and genital lichen sclerosus (GLSc). Although there have been several reported cases of vulval melanoma (MM) associated with LSc, particularly in the paediatric population, fewer cases of male genital (M) GLSc and penile (Pe)MM have been published. Objectives: The aim of this study was to explore further the relationship between PeMM and MGLSc by reviewing all the cases managed by our multidisciplinary service over a finite period. Methods: All patients known to our tertiary urology and male genital dermatology service with a diagnosis of PeMM and where histology was available for review were identified over an 11-year period (2011-2022). The histology was reviewed by two independent, mutually 'blinded' histopathologists. Photographs and clinical notes, where available, were retrospectively reviewed by two independent dermatologists for signs or symptoms of LSc. Results: Eleven patients with PeMM were identified for review. Histopathological examination found evidence of LSc in nine patients, and review of clinical photos corroborated the presence of LSc in three. Overall, features of LSc were present in nine out of eleven cases (82%). Conclusion: The presence of LSc in 9 out of 11 cases of PeMM is suggestive of a causative relationship between LSc and PeMM. This may be due to chronic melanocytic distress created by chronic inflammation secondary to LSc.

Genital lichen sclerosus and melanoma; a systematic review.

Background: Lichen sclerosus (LSc) is a chronic, inflammatory, destructive skin disease with a predilection for the genitalia (GLSc). An association with vulval (Vu) and penile (Pe) squamous carcinoma (SCC) is now well established but melanoma (MM) has only rarely been reported complicating GLSc. Methods: We have performed a systematic literature review of GLSc in patients with genital melanoma (GMM). Only articles that mentioned both GMM and LSc affecting either the penis or vulva were included. Results: Twelve studies with a total of 20 patients were included. Our review shows that an association of GLSc with GMM has been more frequently reported in women and female children than men viz, 17 cases compared with three. It is notable that five of the cases (27.8%) concerned female children aged under twelve. Discussion: These data suggest a rare association between GLSc and GMM. If proven, there arise intriguing questions about pathogenesis and consequences for counselling of patients and follow-up.

Negative impact of the pandemic on hospital admissions, morbidity and early mortality for acute cirrhosis decompensation.

INTRODUCTION: The global pandemic has diverted resources away from management of chronic diseases, including cirrhosis. While there is increasing knowledge on COVID-19 infection in liver cirrhosis, little is described on the impact of the pandemic on decompensated cirrhosis admissions and outcomes, which was the aim of this study. METHODS: A single-centre, retrospective study, evaluated decompensated cirrhosis admissions to a tertiary London hepatology and transplantation centre, from October 2018 to February 2021. Patients were included if they had an admission with cirrhosis decompensation defined as new-onset jaundice or ascites, infection, encephalopathy, portal hypertensive bleeding or renal dysfunction. RESULTS: The average number of admissions stayed constant between the pre-COVID-19 (October 2018-February 2020) and COVID-19 periods (March 2020-February 2021). Patients transferred in from secondary centres had consistently higher severity scores during the COVID-19 period (UK Model for End-Stage Liver Disease 58 vs 54; p=0.007, Model for End-Stage Liver Disease-Sodium 22 vs 18; p=0.006, EF-CLIF Acute Decompensation (AD) score 55.0 vs 51.0; p=0.055). Of those admitted to the intensive care without acute-on-chronic liver failure, there was a significant increase in AD scores during the COVID-19 period (58 vs 48, p=0.009). In addition, there was a trend towards increased hospital readmission rates during the COVID-19 period (29.5% vs 21.5%, p=0.067). When censored at 30 days, early mortality postdischarge was significantly higher during the COVID-19 period (p<0.001) with a median time to death of 35 days compared with 62 days pre-COVID-19. DISCUSSION: This study provides a unique perspective on the impact that the global pandemic had on decompensated cirrhosis admissions. The findings of increased early mortality and readmissions, and higher AD scores on ICU admission, highlight the need to maintain resourcing for high-level hepatology care and follow-up, in spite of other disease pressures.

A systematic review and meta-analysis of transthoracic echocardiogram vs. cardiac magnetic resonance imaging for the detection of left ventricular thrombus.

Transthoracic echocardiography (TTE) is the most commonly used imaging modality to diagnose left ventricular thrombus (LVT), however, cardiac magnetic resonance (CMR) remains the gold standard investigation. A comparison of the diagnostic performance between two modalities is needed to inform guidelines on a diagnostic approach towards LVT. We performed a systematic review and meta-analysis to investigate the diagnostic performance of three methods of TTE (non-contrast, contrast, and apical wall motion scoring) for the detection of LVT compared to CMR as a reference test. Studies comprising 2113 patients investigated for LVT using both TTE and CMR were included in the meta-analysis. For non-contrast TTE, pooled sensitivity and specificity were 47% [95% confidence interval (CI): 32-62%], and 98% (95% CI: 96-99%), respectively. In contrast, TTE pooled sensitivity and specificity values were 58% (95% CI: 46-69%), and 98% (95% CI: 96-99%), respectively. Apical wall motion scoring on non-contrast TTE yielded a sensitivity of 100% [95% CI: 93-100%] and a specificity of 54% (95% CI: 42-65%). The area under the curve (AUC) values from our summary receiver operating characteristic curve (SROC) for non-contrast and contrast TTE were 0.87 and 0.86 respectively, with apical wall motion studies having the highest AUC of 0.93. Despite high specificity, routine contrast and non-contrast TTE are likely to miss a significant number of LVT, making it a suboptimal screening tool. The addition of apical wall motion scoring provides a promising method to reliably identify patients requiring further investigations for LVT, whilst excluding others from unnecessary testing.

The formation of left ventricular thrombus (LVT), a blood clot in the left pumping chamber of the heart, can lead to serious complications such as a stroke. Whilst cardiac magnetic resonance (CMR) is the best imaging tool to detect these clots, the most used tool is a transthoracic echocardiogram (TTE), which visualizes the heart by placing an ultrasound on the chest. This is due to the affordability and widespread availability of TTE. Thus, it is important to know how TTE compares to CMR when it comes to detecting LVT. This study pools the results of previous research to compare the diagnostic performance of three different methods of TTE compared to CMR for detecting LVT. Non-contrast TTE.Contrast TTE: The addition of an enhancing dye is thought to improve imaging.Apical wall motion scoring: Evaluating the movement of the heart's walls using TTE. Our results show that current methods of TTE may miss half of the patients with LVT and that the use of contrast did not provide significant improvement. Interestingly, the use of apical wall motion scoring was able to accurately detect all the patients with LVT. This shows promise as a future tool to reliably exclude patients from unnecessary testing, whilst identifying those who need further investigations.