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Lung carcinoma management secondary to chronic lymphocytic leukemia could be quite challenging. We report a case of a 60-year-old male with several co-morbidities, who presented with shortness of breath and persistent cough. A chest imaging showed a right pleural effusion and complete white-out of the right chest cavity. A computed tomography scan revealed consolidation of the right upper lobe with a 6-cm lesion in hilum with complete occlusion of right lobe bronchus. The patient underwent a video-assisted thoracoscopic surgery, drainage of pleural effusion and pleural and lung biopsy. Talc pleurodesis as well as a flexible bronchoscopy of the endobronchial lesion was performed. Histopathological examination showed a small B-cell lymphoma of the right pleura and an invasive non-small cell carcinoma of the right lung. Dual neoplasms are challenging in terms of diagnosing, and they usually require a multidisciplinary team for the right treatment strategy, including surgery and chemotherapy.

Objectives: Cystic neutrophilic granulomatous mastitis (CNGM) is a rare cause of destructive inflammatory breast disease which has been linked to the presence of lipophilic Corynebacteria which often show broad resistance to antibiotics. Antibiotic therapy is frequently utilised as an adjunct to surgical management however there is a lack of reported data on optimal antimicrobial strategies and recovery is often prolonged. Methods: We developed a management strategy in a tertiary infectious diseases unit to identify potential Corynebacteria-related CNGM pathology and took a targeted antibiotic-led approach to treatment. Clinical, laboratory, imaging, and therapeutic data were collected retrospectively from patients seen in our clinic between 2016-2020. Results: Thirteen patients were identified who met criteria for the diagnosis of CNGM. The mean age was 42.6 years and all patients were female. All had bacteriological evidence of Corynebacteria infection or granulomatous findings on histology. Microbiological evidence of Corynebacteria was present in 76.9%. Antibiotics were used as first line therapy in 92.6%, with a variety of agents used. Steroids were used as an adjunctive therapy in 53.8%. Most patients underwent surgical drainage or clinic-based aspiration of an abscess as part of their treatment. Eleven of thirteen patients (84.6%) experienced complete resolution of symptoms however recovery was protracted with a median time to resolution of >6 months. Conclusions: We present a case series of a rare clinical entity, CNGM, and highlight the outcomes of implementation of an antibiotic and infection service-led clinical approach in these patients.

Desmoid tumours are rare, locally aggressive neoplasms exhibiting high tendency for recurrence, even after complete resection. Only 1 in 5 of them originates from the chest wall, usually measuring less than 10 cm at diagnosis. Herein, we report the case of a woman presenting with symptoms of gradual lung compression by a giant desmoid tumour occupying the entire hemithorax. She underwent complete surgical resection of the tumour and chest wall reconstruction. She had disease recurrence 15 months later and currently remains under regular follow-up. The management of intrathoracic desmoid tumours is challenging because they are usually not diagnosed until they become large enough to cause compression symptoms. While medical management is the primary modality of treatment, surgery could be considered in selected cases where significant symptoms arise, and the functional status is impaired secondary to the tumour. Adjuvant radiotherapy to minimise the risk of local recurrence should also be considered.

ADAPTeR is a prospective, phase II study of nivolumab (anti-PD-1) in 15 treatment-naive patients (115 multiregion tumor samples) with metastatic clear cell renal cell carcinoma (ccRCC) aiming to understand the mechanism underpinning therapeutic response. Genomic analyses show no correlation between tumor molecular features and response, whereas ccRCC-specific human endogenous retrovirus expression indirectly correlates with clinical response. T cell receptor (TCR) analysis reveals a significantly higher number of expanded TCR clones pre-treatment in responders suggesting pre-existing immunity. Maintenance of highly similar clusters of TCRs post-treatment predict response, suggesting ongoing antigen engagement and survival of families of T cells likely recognizing the same antigens. In responders, nivolumab-bound CD8+ T cells are expanded and express GZMK/B. Our data suggest nivolumab drives both maintenance and replacement of previously expanded T cell clones, but only maintenance correlates with response. We hypothesize that maintenance and boosting of a pre-existing response is a key element of anti-PD-1 mode of action.

Pulmonary alveolar microlithiasis (PAM) is an uncommon genetic disorder associated with alveolar cell injury. This injury is caused in most cases by inactivating mutations in SLC34A2 gene, which is responsible for the production of a sodium-dependent phosphate co-transporter. The dysfunction or deficiency of this transporter leads to the aggregation of local phosphate intra-alveolarly and formation of microliths. Most of the patients are asymptomatic at the time of the diagnosis but as the disease progress it leads to fatal respiratory or cardiac failure. We describe a case of a 63-year-old man referred to our department for a surgical lung biopsy. He has been symptomatic for one year with progressive shortness of breath and deteriorating exercise tolerance. The imaging was suggestive of extensive interstitial bilateral lung disease. Histological findings after the lung biopsy by video-assisted thoracic surgery (VATS) established the diagnosis of pulmonary alveolar microlithiasis. His sister suffered from the same disease and passed away at the age of 54. It is remarkably rare for PAM to have such a late onset with a previous normal X-ray and only a few cases have been reported worldwide.

Interstitial lung diseases are not infrequently complicated by development of malignancies and whilst most cases are carcinomas, rare cases of lymphoma have been reported,(1 2) these being diffuse large B-cell lymphomas and often associated with connective tissue disorders (CTDs). Reactive pulmonary lymphoid hyperplasia (RPLH), typically in the form of lymphoid interstitial pneumonia (LIP) often arises in patients with CTDs and only rarely shows malignant transformation,(3) with many of the early putative cases of transformation to MALT (mucosa-associated lymphoid tissue) lymphoma from LIP being lymphoma 'de novo'.(4) Herein we present a case of pulmonary non-Hodgkin lymphoma of MALT origin arising on a background RPLH with coexistent amyloidosis, confirmed by immunohistochemical and clonality studies. A 57 year old Caucasian female presented with chest pain and breathlessness at rest. She also described intermittent cough with small amounts of sputum, intermittent sweats, fatigue and joint pain in hands, feet, wrists, knees. She was known to have systemic lupus erythematosus (SLE) and SLE-related antiphospholipid syndrome for which she received immunosuppressive therapy including mycophenolate, prednisolone and hydroxychloroquin. This article is protected by copyright. All rights reserved.

Pleuroparenchymal fibroelastosis (PPFE) is now a defined clinicopathologic entity in the updated 2013 ATS/ERS classification of idiopathic interstitial pneumonias (IIPs), which has led to a significant increase in cases being diagnosed at our institution. We have therefore reviewed 43 PPFE cases (58 biopsies in total) to assess whether any clinical or histopathologic features provide prognostic information. A semiquantatitive grading system was used to assess extent of fibroblastic foci, intra-alveolar fibroelastosis, visceral pleural fibrosis, chronic inflammation in areas of fibrosis, vascular fibrointimal thickening, and presence of granulomas. Other patterns of interstitial lung disease were also noted, if present. All biopsies showed intra-alveolar fibroelastosis, fibroblastic foci at the leading edge of fibrosis and chronic inflammation within areas of fibrosis, 91% showed vascular fibrointimal thickening of vessels, 73% showed pleural fibrosis, and 35% showed granulomas. Ten cases showed a coexistent IIP (5 showed usual interstitial pneumonia, 5 showed features of hypersensitivity pneumonitis). There was no significant correlation with mortality and severity of histologic parameters, other than a significant decrease in mortality in PPFE with coexistent granulomas, after adjusting for age and gender (hazard ratio, 0.27; P=0.049). Male gender was also associated with an increased risk of mortality, after adjusting for age (hazard ratio, 4.8; P=0.045). PPFE is more common than previously thought, not infrequently showing coexistent pathology, specifically usual interstitial pneumonia and granulomatous lung disease, our data suggesting the latter may have prognostic significance.

Aims Restrictive Allograft Syndrome (RAS) and idiopathic pleuroparenchymal fibroelastosis (IPPFE) are two different diseases reported to share the same histology. RAS relates to chronic allograft dysfunction in lung transplantation with IPPFE being a rare condition in native lungs. Our aim is to compare their histologies, alongside biopsies of usual interstitial pneumonia (UIP), to determine if there are differences that might help to elucidate the pathogenesis. Methods and Results We selected 4 post-mortem allograft lungs from patients who developed clear clinical RAS pattern, 5 biopsies diagnosed as IPPFE, 5 UIP biopsies and 5 sections of normal lung. Histopathologic features were described without knowledge of clinical and radiological features. Both RAS allografts and IPPFE biopsies showed intra-alveolar fibrosis and elastosis (IAFE), but RAS allografts also showed concomitant obliterative bronchiolitis, vascular lymphoplasmacytic inflammation within fibro-intimal thickening, less fibroblastic foci at the advancing edge of the fibrosis (1 against 14.4 FF in 2mm2) and a slight reduction of the capillary network compared to UIP (p=0.07) and controls (p=0.06). The main differences seen in UIP were the lack of IAFE and the presence of honeycomb change. Conclusions RAS and PPFE histopathology both show IAFE, but display various differences, particularly in their vascular morphology that may allow further understanding of pathogenesis. This article is protected by copyright. All rights reserved.

Aims and MethodWe surveyed the usage and perceived utility of standardised risk measures in 29 forensic medium secure units (a 62% response rate).ResultsThe most common instruments were Historical Clinical Risk–20 (HCR–20) and Psychopathy Checklist – revised (PCL–R); both were rated highly for utility. the Risk Matrix 2000 (RM2000), Sex Offender Risk Appraisal Guide (SORAG) and Static-99 were the most common sex offender assessments, but the Sexual Violence Risks–20 (SVR–20) was rated more positively for its use of dynamic factors and relevance to treatment.Clinical ImplicationsMost medium secure units use structured risk assessments and staff view them positively. As HCR–20 and PCL–R/PCL–SV (Psychopathy Checklist – Screening Version) are so widely used they should be the first choices considered by other services.

IgG4-related disease (IgG4-RD) is a fibroinflammatory condition that can affect practically every organ. Although it was first identified in pancreas and salivary glands, major organs like liver, biliary tree, kidney, thyroid glands and lungs are commonly involved, sometimes resulting in organ failure. We describe a case of an 41-year-old man presented with back pain after a rotator cuff injury. A Computed Tomography (CT) revealed incidentally a right lower lobe paravertebral lesion extending across the T5 and T6 vertebral levels and invading into the adjacent pleural surface. The laboratory findings and the CT guided biopsy were inconclusive. Morphological and immunohistochemical findings after a lung biopsy by video-assisted thoracic surgery (VATS) were suggestive to IgG4-related lung disease (IgG4-RLD), which was confirmed with high serum levels of IgG4. This represents the first case of a IgG4-RLD lesion located in the mediastinum and extending to the adjacent pleural surface and vertebrae and should be included in the differential diagnosis of posterior mediastinal masses.