Systemic Amyloidosis Mimicking Lung Cancer.
Ontology highlight
ABSTRACT:
SUBMITTER: Fisch AS
PROVIDER: S-EPMC6938147 | biostudies-literature | 2020 Jan
REPOSITORIES: biostudies-literature
Publications
Systemic Amyloidosis Mimicking Lung Cancer.
Fisch Adam S AS Fintelmann Florian J FJ Garcia-Moliner Maria L ML Gaissert Henning A HA Lennes Inga I Butera James N JN Stone James R JR Hariri Lida P LP
American journal of respiratory and critical care medicine 20200101 1
Similar Datasets
Project description:Localized nodular pulmonary amyloidosis can form pulmonary nodules associated with cystic air spaces, but due to its rarity, it cannot be included in the differential diagnosis without appropriate knowledge. Among the differential diagnoses of nodules with cysts in the lungs is primary lung cancer, however, diagnosis based solely on imaging findings is challenging. A 59-year-old Japanese female was referred to our hospital for an abnormality noted on the chest radiograph of an annual health check. She had no history of smoking or medical issues. Chest computed tomography revealed a 1.2 cm pulmonary nodule with surrounding multilocular cystic air spaces in the superior lingular segment. We suspected it was a nodule of primary lung cancer arising in the pulmonary cyst and performed video-assisted thoracic surgery. As the intraoperative frozen examination after a wedge resection revealed fibrotic tissue without malignancy, we did not do any further resection. The histopathological examination of the permanent section revealed unstructured eosinophilic deposits positive for direct fast scarlet staining, which were consistent with amyloidosis. The surrounding pulmonary cysts contained the check valve made by amyloid deposition. Localized nodular pulmonary amyloidosis can give rise to pulmonary cysts and mimic primary lung cancer associated with cystic air spaces. It should be raised as a potential differential diagnosis for pulmonary nodules with cystic air space formation, particularly in patients without a smoking history.
| S-EPMC11231505 | biostudies-literature
Project description:Pulmonary embolism (PE) is a common finding in patients with underlying malignancy and is the commonest cause of acute cor pulmonale. A 65-year-old woman with a background of non-small-cell lung cancer presented to the emergency department with nausea and vomiting after starting erlotinib; she was pyrexial and had raised C-reactive protein. Despite aggressive fluid resuscitation and antibiotics the patient remained tachycardic, hypotensive, profoundly hypoxic and had a persistent raised jugular venous pulse. Massive PE was therefore suspected. A bedside echocardiogram demonstrated a dilated right ventricle and evidence of pulmonary hypertension. A CT pulmonary angiogram excluded a PE but revealed progression of the right hilar tumour causing complete obstruction of the right upper and middle lobe pulmonary arteries. This case highlights an important differential diagnosis when assessing patients with an underlying intrathoracic tumour with findings suggestive of PE and the importance of obtaining further imaging before considering thrombolysis.
| S-EPMC4543115 | biostudies-other
Project description:An aspergilloma is a conglomeration of Aspergillus hyphae, fibrin, mucus, and cellular debris, typically found within a pulmonary cavity or ectatic bronchus. Computerized tomography (CT) scans often depict a cavity containing a solid mass and a notable crescent sign. Though these signs are indicative of aspergilloma, the European Respiratory Society emphasizes the need for a more detailed diagnostic criteria. A patient with a history of hemoptysis was initially diagnosed with an aspergilloma based on CT chest findings, showing a cavitary lesion in the left upper lobe with an intracavitary lobular opacity. Post resection, histological examination contradicted the initial diagnosis, revealing lung cancer instead. This case underscores the importance of tissue diagnosis from the outset or employing a multifaceted diagnostic criteria encompassing radiological findings, serology and microbiology tests. In the absence of an initial tissue diagnosis, rigorous follow-up, including early interval scanning, is crucial.
| S-EPMC10629857 | biostudies-literature
Project description:We herein report a case of pulmonary hyalinizing granuloma (PHG), which is a rare pulmonary mass. A 69-year-old man with no symptoms presented to our hospital because of the appearance of an abnormal shadow on chest X-ray. Computed tomography revealed a right middle-lobe mass with spicula and infiltration into the upper lobe. Since a bronchofiberscopic examination showed no malignant cells in the specimen, the patient underwent thoracoscopic surgery, which revealed PHG. Spiculation and interlobar infiltration, which comprise the characteristic features of primary lung cancer, are uncommon presentations of this rare entity.
| S-EPMC6355427 | biostudies-other
Project description:β2-Microglobulin is responsible for systemic amyloidosis affecting patients undergoing long-term hemodialysis. Its genetic variant D76N causes a very rare form of familial systemic amyloidosis. These two types of amyloidoses differ significantly in terms of the tissue localization of deposits and for major pathological features. Considering how the amyloidogenesis of the β2-microglobulin mechanism has been scrutinized in depth for the last three decades, the comparative analysis of molecular and pathological properties of wild type β2-microglobulin and of the D76N variant offers a unique opportunity to critically reconsider the current understanding of the relation between the protein's structural properties and its pathologic behavior.
| S-EPMC4400370 | biostudies-literature
Project description:The term "amyloidosis" encompasses the heterogeneous group of diseases caused by the extracellular deposition of autologous fibrillar proteins. The global incidence of amyloidosis is estimated at five to nine cases per million patient-years. While amyloid light-chain (AL) amyloidosis is more frequent in developed countries, amyloid A (AA) amyloidosis is more common in some European regions and in developing countries. The spectrum of AA amyloidosis has changed in recent decades owing to: an increase in the median age at diagnosis; a percent increase in the frequency of primary AL amyloidosis with respect to the AA type; and a substantial change in the epidemiology of the underlying diseases. Diagnosis of amyloidosis is based on clinical organ involvement and histological evidence of amyloid deposits. Among the many tinctorial characteristics of amyloid deposits, avidity for Congo red and metachromatic birefringence under unidirectional polarized light remain the gold standard. Once the initial diagnosis has been made, the amyloid subtype must be identified and systemic organ involvement evaluated. In this sense, the (123)I-labeled serum amyloid P component scintigraphy is a safe and noninvasive technique that has revolutionized the diagnosis and monitoring of treatment in systemic amyloidosis. It can successfully identify anatomical patterns of amyloid deposition throughout the body and enables not only an initial estimation of prognosis, but also the monitoring of the course of the disease and the response to treatment. Given the etiologic diversity of AA amyloidosis, common therapeutic strategies are scarce. All treatment options should be based upon a greater control of the underlying disease, adequate organ support, and treatment of symptoms. Nevertheless, novel therapeutic strategies targeting the formation of amyloid fibrils and amyloid deposition may generate new expectations for patients with AA amyloidosis.
| S-EPMC4218891 | biostudies-other
Project description: Not available
| S-EPMC3796600 | biostudies-literature
Project description: Not available
| S-EPMC8885899 | biostudies-literature
Project description:BackgroundCardiac amyloidosis, a progressive cardiac disease, results from the accumulation of undegraded proteinaceous substrates in the extracellular matrix of the heart. It may present as acute coronary syndrome (ACS); therefore, a clear distinction remains challenging in clinical practice. We describe a case of cardiac amyloidosis mimicking ACS.Case summaryA 72-year-old man experienced chest discomfort for 2 days. He gradually developed dyspnoea during the preceding month. Electrocardiogram (ECG) showed sinus rhythm with right bundle branch block and low voltage. Echocardiography revealed concentric left ventricular thickening, biatrial dilation, and preserved ejection fraction with predominantly left ventricular basal hypokinesis. Serial testing of the cardiac biomarkers showed persistently increased high-sensitive cardiac troponin T levels and normal serum creatine kinase myocardial band levels. He was diagnosed with ACS with haemodynamic stability. However, coronary angiography demonstrated non-obstructive coronary arteries. Furthermore, significant macroglossia and periorbital purpura were noticed. Laboratory investigations revealed elevated serum immunoglobulin free light chain (FLC) kappa and lambda levels with an increased FLC ratio. Histological analysis of the biopsied abdominal skin confirmed amyloidosis.DiscussionCardiac amyloidosis often presents as restrictive cardiomyopathy. The usual symptoms include dyspnoea and peripheral oedema. Chest pain may manifest rarely, leading to misdiagnosis as coronary artery disease. Some findings suggestive of cardiac amyloidosis include clinical signs such as amyloid deposits, dyspnoea, low ECG voltage, and basal-predominant hypokinesis with relative apical sparing in echocardiography. Serum FLC test and abdominal skin biopsy can confirm the diagnosis of amyloidosis when a myocardial biopsy is not feasible.
| S-EPMC7793193 | biostudies-literature
Project description:A 26-year-old woman was diagnosed with and treated for systemic lupus erythematosus (SLE) in 2002. She was admitted 11 years later with nephrotic-range proteinuria and lupus nephritis and received two doses of rituximab after failing on steroids and mycophenolate mofetil. Four months later, she presented with fever and joint pain/swelling. Gram stains, joint aspirates, and blood culture all yielded negative results for bacteria. She was discharged after treatment for a possible flare of lupus, but two weeks later, she presented again with a cough and shortness of breath in addition to the flare symptoms. Synovial fluid Smears, and cultures yielded positive results for Mycobacterium tuberculosis; similarly, sputum polymerase chain reaction test and culture confirmed pulmonary tuberculosis. Tuberculosis is difficult to diagnose in SLE patients; it may present like or precipitate SLE flare. In this patient a presumed SLE flare turned out to be an aggressive miliary, disseminated tuberculosis.
| S-EPMC6143694 | biostudies-literature