LessonAmyloidosis is a rare differential diagnosis of a mass detected in the chest. Amyloidoma is caused by a local proliferation of clonal B-cells secreting an unstable immunoglobulin light chain which accumulates. FDG-PET scan are useful but not specific. Treatment is generally by local resection for treatment of symptoms.We report two cases of amyloidomas, which are rare entities characterised by large local amyloid deposits. These can occur in the upper respiratory tract, soft tissues and central nervous system.1
Diffuse "true" cystic lung disease is rare, and the specificity of high-resolution computed tomography (HRCT) has reduced the need for biopsy. We present 5 patients with similar clinical and HRCT features of cystic lung disease that were sufficiently atypical to warrant surgical lung biopsies that showed coexistent small airway diseases (SAD). There were 4 female patients and 1 male patient with a mean age of 43 years. All were never smokers. Four had symptoms such as dyspnea (1), cough (2), or both (1). HRCTs showed variably sized thin-walled cystic airspaces without zonal distribution, some with prominent vessels in their walls. One case was unilateral. Surgical lung biopsy showed cystic change comprising localized loss of alveolar density with coexistent SADs [chronic bronchiolitis (n=2), eosinophilic bronchiolitis, probable asthma (n=1), and diffuse idiopathic neuroendocrine cell hyperplasia (n=2)]. Two patients who were tested for Birt-Hogg-Dube-related gene mutations proved negative, and all lacked other features of Birt-Hogg-Dube. We hypothesize that chronic damage to small airways may lead to cystic degeneration in a minority of patients. Precedents in relation to Sjogren syndrome and hypersensitivity pneumonitis raise the possibility of a causal association between pathologies in these 2 anatomic compartments, although HRCT data in relation to common SADs indicate that this would be a rare phenomenon. The driving factor remains unknown.
The decline of the hospital autopsy is a well-known phenomenon that shows no sign of ending. Debate continues for the reasons behind this, but inadequate consent practices are thought to play a role. The furore resulting from organ retention scandals at Bristol Royal Infirmary and The Royal Liverpool Children's Hospital led to widespread soul searching in the medical profession, and a fundamental change in how we treat the dead body. In response, the 2004 Human Tissue Act was created, and consent is now centrally placed to permit all activities dealing with the cadaver, including autopsy. This article reflects on consent practices for hospital autopsy in England and Wales. Relevant policies from 26 National Health Service trusts were examined against the recommended standards set by the Human Tissue Authority. We found numerous failures of multiple trusts to follow these standards. Several trust policies failed to outline basic information to guide staff in conducting the consent process, such as the training requirements of the consent taker, and the desired approach to take consent. Many trusts failed to outline vital recommendations of the Human tissue Authority, such as the requirement of the consent taker to be experienced, trained in dealing with the bereaved and well informed on autopsy practice, as well as the requirement to have witnessed an autopsy. We recommend trusts reassess their practices in order meet the established standards with an emphasis on educating staff and developing a team-based approach to consent taking.
No similar case has previously been reported. The aetiology of myofibroma is unclear, but repeated trauma may be a trigger. Histological and immunohistochemical analysis are recommended when the diagnosis is ambivalent.
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