Journal of clinical pathology
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Pulmonary fibrosis in surgical lung biopsies is said to have a 'usual interstitial pneumonia-pattern' (UIP-pattern) of disease when scarring of the parenchyma is present in a patchy, 'temporally heterogeneous' distribution. These biopsies are one of the more common non-neoplastic specimens surgical pathologists encounter and often pose a number of challenges. UIP is the expected histopathological pattern in patients with clinical idiopathic pulmonary fibrosis (IPF), but the UIP-pattern can be seen in other conditions on occasion. ⋯ Taken in isolation, the UIP-pattern seen in patients with IPF may appear to overlap with that of RILD and CHrHP, at least when using the broadest definition of this term (patchy fibrosis). However, important distinguishing features are nearly always present in our experience, and the addition of a multidisciplinary approach will often resolve the critical differences between these diseases. In this manuscript, we review the distinguishing clinical, radiologic and histopathological features of UIP of IPF, RILD and CHrHP, based, in part, on the existing literature, but also lessons learned from a busy lung biopsy consultation practice.
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This review addresses the pathology of lung disease in which the predominant finding is diffuse cystic change. Although cysts may be found radiologically in a wide variety of disease states, the entities discussed are those most likely to be encountered in biopsies where the underlying aetiology is unclear. ⋯ Conditions in which cyst formation may occur but does not represent the predominant pathology are also considered, including alveolar septal amyloidosis, light chain disease, follicular bronchiolitis and lymphocytic interstitial pneumonia. Cystic metastases may present a differential diagnostic dilemma.
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Birt-Hogg-Dubé syndrome (BHD) is an autosomal dominant inherited disorder characterised by fibrofolliculomas, renal tumours, pulmonary cysts and pneumothorax. The pulmonary cysts and repeated episodes of pneumothorax are the clinical hallmarks for discovering families affected by the syndrome. This disorder is caused by mutations in the gene coding for folliculin (FLCN). ⋯ However, the mechanisms of the formation of pulmonary cysts and pneumothorax associated with heterozygous mutations in FLCN are poorly understood. Resected lung specimens from patients with BHD are often misdiagnosed by pathologists as non-specific blebs or bullae or emphysema, and patients with BHD who have pulmonary cysts and repeated pneumothorax frequently do not receive appropriate medical investigations. This review discusses the clinical and pathological features of lungs of patients with BHD, focusing on the diagnostic pathology and possible mechanisms of cyst formation.
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Bariatric surgery can effectively reduce body weight and treat obesity associated metabolic diseases such as diabetes mellitus. There are also benefits for an individual's functional status and psychological health. A multi-disciplinary evaluation should be offered to the individual as the first essential step in considering bariatric surgery as a treatment. ⋯ We describe the literature on outcomes after bariatric surgery, including the results for mortality, weight loss, remission of diabetes and associated endocrine disorders such as hypogonadism. Within this review, we will illustrate the impact of bariatric surgery on self-image, psychological health and perceived health and functional status. Finally, we briefly detail the potential complications of bariatric surgery, and offer advice on post-operative care and surveillance.
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Review
Immunohistochemistry in the distinction between primary and metastatic ovarian mucinous neoplasms.
The distinction between a primary and metastatic mucinous carcinoma within the ovary may be problematic. In most cases, the distinction can be made by careful pathological examination encompassing both the gross and microscopic findings and taking into account the distribution of the disease. ⋯ There is a significant degree of immunophenotypic overlap between primary ovarian mucinous neoplasms and metastatic mucinous carcinomas from the gastrointestinal tract, especially the upper gastrointestinal type; this is because most primary ovarian mucinous carcinomas and borderline tumours are of so-called intestinal or enteric type and exhibit some degree of positivity with enteric markers. Mullerian type primary ovarian mucinous neoplasms also exist and exhibit distinct immunohistochemical differences to the more common intestinal type.