Case of recurrence of AA type primary solitary pulmonary amyloidosis]

The patient was a 64-year-old asymptomatic man. His chest X-ray film, at age 61, showed a solitary nodule in the right middle lobe. Thoracoscopic surgery was performed. The nodule was pathologically diagnosed as a solitary nodular amyloidosis. After 3 years, his chest CT showed a solitary nodule in the left upper lung. Thoracoscopic surgery for the pulmonary nodule was performed again, and it was also pathologically diagnosed as a solitary nodular amyloidosis with AA type amyloid protein. Solitary nodular pulmonary amyloidosis is usually AL type, and few cases of solitary AA type amyloidosis have been reported. We reported a rare case of recurrence of solitary pulmonary amyloidosis.     

Spontaneous resolution of multiple nodular pulmonary AA amyloidosis

A 62-year-old man presented with a two-week history of dry cough. A chest computed tomography (CT) showed three nodular masses of soft tissue density without calcification or cavitary formation in the right lung. F-18 fluorodeoxyglucose PET/CT scan revealed high FDG uptake in two out of three pulmonary nodules. Transbronchial lung biopsy specimens consisted of amorphous eosinophilic deposits that were demonstrated to be amyloid because they were positive for Congo Red staining. After oxidation with permanganate solution, the Congo Red staining disappeared, indicating that this amyloid was amyloid A protein-derived type. There was no evidence of any systemic diseases. We diagnosed the patient as having multiple nodular pulmonary AA amyloidosis. The patient was conservatively managed without treatment, and the pulmonary nodules disappeared spontaneously three months later.     

Sj?gren's syndrome with solitary nodular pulmonary amyloidosis]

We describe a case of limited pulmonary amyloidosis with Sj?gren syndrome. A 58-yr-old woman was referred to our hospital because of an abnormal chest radiograph (solitary small nodule) that was examined to investigate the cause of a persistent cough. A chest CT revealed a solitary small nodule in the left lower lung field. The specimens obtained by thoracoscopic surgery showed AL (kappa) amyloid deposits with lymphoplasmacytic infiltrate. Immunofixation of the serum and concentrated urine failed to demonstrate monoclonal immunoglobulins, and no amyloid deposits in the stomach were detected. She was subsequently diagnosed as having primary Sj?gren's syndrome. Nodular pulmonary amyloidosis with Sj?gren syndrome is very rare condition, and most cases present multiple nodules. As far as is known, this is the first report of a solitary nodule in pulmonary amyloidosis with Sj?gren syndrome.     

FDG-PET findings of nodular pulmonary amyloidosis with a long-term observation]

A 60-year-old woman was referred to our hospital because of an abnormal chest radiograph in May, 2000. She was found to have rheumatoid arthritis in March, 1998, and pharmacologic therapy with anti-rheumatic drug was started. The chest CT scan revealed bilateral multiple lung nodular lesions of various sizes up to 30 mm. F-18 fluorodeoxyglucose positron emission tomography (FDG-PET) demonstrated a lesion with intense FDG activity in the right lung with a standardized uptake value (SUV) of 10.1. Fiberoptic bronchoscopy revealed no endobronchial lesions. Video-assisted thoracoscopic surgery was done to ascertain the pathological diagnosis. Histological examination showed that the pulmonary nodules were composed of amyloid A (AA) protein. Secondary Sj?gren syndrome was subsequently diagnosed. A diagnosis of localized nodular pulmonary amyloidosis with AA type amyloid protein was made, and therapy with anti-rheumatic drugs was continued. After six years of therapy, the size of pulmonary amyloidoma was reduced, and the accumulation of FDG returned to normal. We reported this interesting case in which FDG-PET apparently reflected the disease activity of pulmonary amyloidosis.     

Rheumatic heart disease associated with secondary renal amyloidosis

Introduction: Amyloidosis is a disorder of protein folding in which normally soluble proteins undergo conformational changes and are deposited in the extracellular space in an abnormal fibrillar form. Accumulation of these fibrils causes progressive disruption of the structure and function of tissues and organs, and the systemic forms of amyloidosis are frequently fatal. The conditions that underlie amyloid deposition may be either acquired or hereditary. Amyloid-A (AA) amyloidosis is the most common form of systemic amyloidosis worldwide, AA amyloidosis occurs in the course of chronic inflammatory diseases, hereditary periodic fevers, and with certain neoplasms such as Hodgkin disease and renal cell carcinoma. Amyloidosis due to rheumatic heart disease (RHD) is not common but can be seen. We report here a patient with RHD and AA renal amyloidosis. Case Report: we present a 30 year-old Egyptian male with a history of RHD, accidently discovered to have nephrotic range proteinuria and rising serum creatinine. Serology studies were negative or normal, including antinuclear antibody (ANA) and antineutrophil cytoplasmic antibody (ANCA). C3 and C4 complement levels were normal. Kidney biopsy revealed AA renal amyloidosis. CT chest and abdomen revealed bilateral hilar and mediastinal lymphadenopathy and para-aortic lymph nodes. Endobronchial biopsy and bronchoalveolar lavage revealed non-specific chronic inflammatory changes. The patient's secondary amyloidosis was presumed to be related to the long standing RHD after exclusion of other causes of secondary amyloidosis. The patient finally died due to heart failure and acute pulmonary edema. Conclusion: Long standing RHD can lead to secondary AA amyloidosis. Keywords: AA Amyloidosis; Kidney Biopsy; Rheumatic Heart Disease; Renal Amyloidosis.     

A case of lung adenocarcinoma presenting a solitary, predominantly solid pulmonary nodule that showed slow growth over 7 years]

A small, solitary, predominantly solid pulmonary nodule (7 x 6 mm) was found in a 63-year-old woman during a CT screening for lung cancer. After 7 years, another chest CT examination revealed that the lesion had grown to a size of 15 x 10 mm. The patient then underwent surgery to remove the nodule, because primary lung cancer was strongly suspected. The resected specimen proved to be a poorly differentiated adenocarcinoma of type D according to the criteria of Noguchi et al. The tumor doubling time (TDT) in this case was estimated to be 661 days, which was longer than in other reported cases of Noguchi type D adenocarcinoma. High-resolution CT (HRCT) of the nodule revealed a predominantly solid lesion with a polygonal shape. No further changes were observed in a one-year follow-up CT, suggesting a benign tumor. We therefore suggest that the follow-up of small, solitary pulmonary nodules is of diagnostic value.     

Hereditary hemorrhagic telangiectasia with multiple hepatic and pulmonary nodular lesions

A 50-year-old female visited the hospital for further evaluation of multiple pulmonary and hepatic nodules. First, she visited her primary physician for general fatigue due to anemia. She had recurrent epistaxis, and her mother had suffered from hereditary hemorrhagic telangiectasia (HHT). Telangiectasias were present in the stomach. This patient was diagnosed with HHT. Computed tomography (CT) revealed multiple pulmonary and hepatic nodules. The pulmonary nodules were due to bleeding from arteriovenous malformations of the lung. Abdominal CT and angiography showed a dilated and meandering hepatic artery, arteriovenous shunts and multiple hepatic nodules. Gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid (Gd-EOB-DTPA)-enhanced magnetic resonance imaging (MRI) showed enhancement in the early dynamic phase and in the liver-specific phase. A liver tumor biopsy of a hepatic nodule showed nodular regenerative hyperplasia (NRH). This report presents a case of HHT with multiple pulmonary and hepatic nodular lesions. Gd-EOB-DTPA-enhanced MRI was useful for making a diagnosis of NRH.     

肺结节患者心理健康状况及影响因素分析

目的分析肺结节患者的心理健康状况及影响因素。 方法通过问卷调查的形式对偶发肺结节的成人进行横断面调查。 结果通过对178例肺结节患者的焦虑和抑郁程度评价，发现大多数肺结节患者有轻度或中度焦虑。根据IES-R评分方差分析，女性患者比男性患者更易出现焦虑状态(P＝0.036)。其他组别的IES-R评分之间无统计学差异。单变量Logistic回归分析显示，结节直径越大焦虑水平程度越高(P＝0.041)，"在CT上看到肺结节"(P＝0.001)会增加焦虑水平，如何解释"肺结节很常见"可以减轻患者焦虑程度(P＝0.006)。多因素回归分析显示，年轻(P＝0.010)、低月收入(P＝0.014)和结节直径较大(P＝0.038)与较高的焦虑水平相关，"在CT上看到肺结节"可导致焦虑水平升高(P＝0.002)。EQ-5D-5L效用得分为0.9578±0.0634，EQ-5D-5L量表对肺结节患者健康相关生活质量的评价是可以接受的，EQ-5D-5L效用与事件影响量表修订版(IES-R)得分呈负相关(r＝0.3495，P<0.0001)。 结论肺结节人群中女性较男性更容易焦虑；如果患者自己在CT上看到肺结节，会增加焦虑程度。告诉患者肺结节很常见，可以降低焦虑水平。     

A case of solitary nodular pulmonary amyloidosis]

A 64-year-old man without respiratory symptoms was introduced to our hospital because of a nodule of 20 mm in diameter found in the left lung in a periodic health examination. The chest radiograph and CT scan showed a well-defined nodule in the middle of the left lung field, and enlarged mediastinal lymph nodes. Lung cancer was suspected, and transbronchial and CT guided biopsies were performed, but did not lead to a definitive diagnosis. Since the patient refused further examinations, we carefully followed up the nodule in the chest radiograph. After a year and a half, the nodule and the lymph nodes became enlarged, and the patient was admitted to the hospital for a surgical biopsy. Video-assisted thoracoscopic surgery followed by thoracotomy was performed for both a biopsy and nodule resection. Histologically, the nodule tissue was rich in amorphous substances positively stained with Congo Red, which was consistent with amyloidosis. No findings of systemic amyloidosis or secondary amyloidosis were demonstrated.     

Pulmonary amyloidosis in Sjogren's syndrome: A case report and systematic review of the literature

Amyloidosis is a heterogeneous group of disorders characterized by misfolding of extracellular proteins. The two most common forms are light‐chain (AL) and reactive (AA) amyloidosis. Pulmonary amyloidosis secondary to Sjogren's syndrome (SS) is rare, and there is a paucity of data on its clinical and radiological features. We describe the case of a 41‐year‐old woman with diffuse septal pulmonary AL (λ) amyloidosis related to SS, in the absence of systemic amyloidosis. A systematic search for pulmonary amyloidosis in SS yielded 37 cases. Amyloidosis occured almost exclusively in women (96.5%). Cough and dyspnoea (56%) were the most common symptoms. The diagnosis of pulmonary amyloidosis was made subsequent to that of SS, with a median delay of 7 years. Radiologically, diffuse nodules with (45.5%) or without (33.3%) multiple cysts were the most common pattern. Most cases were related to nodular AL amyloidosis (both κ and λ). Diffuse septal AA amyloidosis has been reported in SS. The index patient is the first documented case of diffuse septal pulmonary AL amyloidosis in SS, without systemic amyloidosis. Surgical lung biopsy (78%) is usually required to establish the diagnosis and rule out lymphoma. There is no data to support any definitive therapeutic intervention for SS‐related pulmonary amyloidosis. Observation is sufficient for nodular amyloidosis. Further studies are necessary to assess the efficacy of current therapies in diffuse septal amyloidosis secondary to SS.     

Three cases of the nodular pulmonary amyloidosis with a longterm observation

Longterm observation with chest radiograph and computed tomography (CT) scan was performed for pulmonary amyloidosis. There are few reports of primary pulmonary amyloidosis with a longterm observation. We encountered three cases of nodular pulmonary amyloidosis observed by intermittent chest radiograph or CT for 5 years or more. The patients were a 54-year-old man, and 67- and 68-year old women. For diagnosis, transbronchial biopsy and percutaneous lung biopsy were performed. Amyloid nodules grew slowly and two cases showed findings of cavity and calcification.     

A clinicopathological study of pulmonary cryptococcosis--chest CT and pathologic correlation]

We reviewed the clinicopathological features in 12 patients (7 males and 5 females; mean age 54 yr) with pulmonary cryptococcosis. Eleven of the patients were asymptomatic and the disease was detected by chest radiograph abnormalities. The underlying systemic disease had been diagnosed as diabetes mellitus in two. Chest CT scans showed a solitary nodule in 9 of the 12 patients, multiple nodules in 2, and infiltration in 1. The nodular diameter was less than 2 cm in 10 of the 12. All nodules were located in the subpleural region. On the chest CT, cavitary nodules, scattered nodules, or both, and spiculated nodules were difficult to distinguish from pulmonary tuberculosis and primary lung cancer, respectively. According to McDonnell's pathological classification of pulmonary cryptococcosis, the resected 8 lungs revealed peripheral pulmonary granuloma in 5 and granulomatous pneumonia in 3. It is important to perform a pathological examination for the diagnosis of pulmonary cryptococcosis to avoid misdiagnosis as lung cancer or pulmonary tuberculosis.     

Evaluation and management of solitary and multiple pulmonary nodules.

The evaluation and management of a patient with an SPN is guided by principles that were derived from earlier surgical studies. Stability or no growth for at least 2 years, the presence of calcium in characteristic patterns, and age less than 35 years without any associated risk factors are reliable indicators of a benign process. Fluoroscopy and localized tomography are helpful in evaluation of an SPN. If the nodule is still considered indeterminate, CT scanning, with the use of thin section cuts through the nodule, is now widely employed. If calcium is present in a characteristic pattern, the nodule is considered benign. If the nodule is very dense or more dense than a phantom reference nodule, the nodule has a high likelihood of being benign. Nodules that are less dense than the phantom nodule are indeterminate, and approximately 25% of these nodules will be benign. Computed tomography scan of the chest and upper abdomen is indicated in patients with a previous history of malignancy or when there is a high suspicion that the nodule is malignant. The further evaluation and management of SPNs that are indeterminate after CT examination are controversial. Some recommend tissue biopsy via transbronchoscopic or transthoracic approach, whereas others recommend immediate thoracotomy. Observation is indicated in certain situations when the chance of malignancy is quite low, the patient is not an operable candidate, or when the patient refuses further invasive evaluation. The physician's role in the management of a patient with an SPN is to educate and advise. The physician must be aware of the patient's anxieties, fears, and attitude and provide an opportunity for active participation by the patient in the decision-making process. Multiple pulmonary nodules are most commonly encountered in patients with metastatic disease to the lungs. Other less commonly encountered diseases that present as multiple pulmonary nodules include infections, arteriovenous malformations, Wegener's granulomatosis, and lymphoma. The evaluation and management of the patient with multiple pulmonary nodules are usually guided by the history, physical examination, and laboratory findings.     

The role of PET-CT in decision making on the treatment of localized nodular form of pulmonary AL-amyloidosis

Depending on the extent of organism affected, there is a systemic (amyloid is deposited in the interstitial space of multiple tissues and organs) and localized (amyloid is deposited in one or a few solitary lesions) form of amyloidosis. Localized forms of amyloidosis have a significantly better prognosis than the systemic ones. The respiratory tract might be affected by diffuse interstitial involvement, associated with systemic AL-amyloidosis, as well as localised involvement of respiratory tract (localised laryngotracheobronchial amyloidosis) or pulmonary parenchyma called nodular form of localized pulmonary amyloidosis. Tracheobronchial form may affect larynx and bronchial tree, and forms plaques or nodules in the epithelium of the respiratory tract. Nodular form causes spherical or irregular lesions in the pulmonary parenchyma, indistinguishable from pulmonary parenchyma metastases. We describe a two-year follow up of a patient with nodular form of pulmonary amyloidosis. The patient had multiple lesions in both lungs, clearly visible on HRCT (High Resolution Computer Tomography) that intensively accumulated fluorodeoxyglucose (FDG) during the first PET-CT. At the time of diagnosis, the largest lesion SUV for FDG accumulation was 8.2. Histochemical analysis showed that amyloid consisted of the light λ chains, i.e. AL-amyloid. Investigations to detect a systemic form of amyloidosis, if present, were negative. The patient had no monoclonal immunoglobulin either in the urine or serum (negative immunofixation) and had normal levels of free light chains in the serum. Her symptoms were previously suggestive of the Sj?grens syndrome. However, the rheumatologist consulted at the time of diagnosis of the nodular form of pulmonary amyloidosis did not find any signs of an active systemic connective tissue disorder. CRP was repeatedly normal. When systemic AL-amyloidosis was excluded, we decided to only monitor lesion development with no treatment intervention. The patient had 3 PET-CTs. CT showed that no lesions enlarged, some lesions decreased in size slightly. It should be emphasized that follow-up PET-CTs did not show increased FDG accumulation. We assume that the increased FDG accumulation in pulmonary lesions seen during the first PET-CT was due to the activity of the cells that formed this amyloid and that this activity spontaneously ceased, leading to normalization of FDG accumulation in pulmonary nodules. PET-CT is useful for monitoring of the development of pulmonary nodular amyloidosis. Normalization of originally increased FDG accumulation in amyloid lesions suggests cessation of the process of amyloid formation and is a positive prognostic sign.     

Coexistence of Sjögren's syndrome and pulmonary nodular amyloidosis

There are very few cases in the literature on the coexistence of Sjögren's syndrome and pulmonary nodular amyloidosis being treated with rituximab. When nodules with central calcification and cystic lesions are seen on computed tomography, amyloid lung should be considered. Biopsy is recommended as it can be confused with malignancies. In this article, we present a 66-year-old female patient who has been followed up for Sjögren's syndrome for 26 years. Multiple cystic lesions with central calcification in the lung were detected and it was evaluated as amyloid nodule in the biopsy performed. The patient is being followed and is stable under rituximab treatment. Pulmonary noduler amyloidosis is very rare in Sjögren patients and there are very few cases where rituximab is used for treatment. We decided to publish in order to guide clinicians who will encounter similar cases.     

Computed tomography halo sign in pulmonary nodules: frequency and diagnostic value

On computed tomography (CT) scanning, a ground-glass opacity zone surrounding a pulmonary nodule has been named the computed tomography (CT) halo sign. To investigate the frequency and diagnostic value of the CT halo sign, the authors reviewed the CT examinations of 305 patients with proven diseases producing solitary or multiple nodules. The CT halo sign was seen in 22 patients (7%). Eleven patients had a solitary nodule; five patients had multiple nodules; and six patients had nodules associated with areas of pulmonary consolidation, or ground-glass opacity, or both. Solitary nodules were the result of bronchioloalveolar carcinoma (n = 5), tuberculoma (n = 2), squamous cell carcinoma, non-Hodgkin lymphoma, myxovirus infection, and metastasis (n = 1 each). Multiple nodules were the result of metastasis (n = 2), Kaposi sarcoma (n = 2), and Wegener granulomatosis (n = 1). Nodules associated with areas of consolidation or ground-glass opacity were the result of metastasis (n = 2), bronchioloalveolar carcinoma, bronchiolitis obliterans organizing pneumonia, eosinophilic pneumonia, and invasive pulmonary aspergillosis (n = 1 each). The data showed that the CT halo sign is a nonspecific finding. It is known that in immunocompromised patients the CT halo sign should suggest invasive pulmonary aspergillosis, Kaposi sarcoma, and lymphoproliferative pulmonary disorders. However, in immunocompetent patients, the authors found that a solitary nodule with the CT halo sign and pseudocavitations has a high likelihood of being a bronchioloalveolar carcinoma.     

Computerized tomographic densitometry of the solitary pulmonary nodule using a nodule phantom

The successful application of computerized tomographic density analysis for evaluating the solitary pulmonary nodule has previously been described and the technique has since been simplified by using a calcium-equivalent nodule reference phantom. Because published experience with this technique remains limited, the current research reports the Cleveland Clinic experience with the CIRS model III pulmonary nodule reference phantom. Without knowledge of the final diagnosis, the nodule phantom was used as the standard for categorizing 31 nodules in 29 patients as either benign or indeterminate. Secure diagnoses were obtained in all cases. Eleven nodules were called benign and ten proved so, whereas 20 nodules were classified as indeterminate. One lesion, an adenocarcinoma, was falsely called benign using this technique. The excellent density discrimination achieved with CT makes this a superior tool for analysis of the solitary pulmonary nodule, and the nodule reference phantom has greatly simplified the technique of CT densitometry. As demonstrated by this and prior studies, calcification is not a unique feature of the benign lesion and successful clinical application of this technique requires cautious interpretation of results.     

Nodular Pulmonary Amyloidosis Associated with Sjögren's Syndrome

Amyloidosis is a rare disease characterized by the deposition of abnormal proteins in extracellular tissues. We herein report a case with instructive radiologic features of nodular pulmonary amyloidosis associated with Sjögren''s syndrome. A 67-year-old woman was referred to our department because of an abnormal chest radiograph. Chest computed tomography revealed multiple round cysts accompanied by calcified nodules. The patient was clinically diagnosed with primary Sjögren''s syndrome and pathologically diagnosed with nodular pulmonary amyloidosis (light chain, kappa). Although multiple lung cysts have many etiologies, the presence of calcified nodules associated with multiple lung cysts is useful for narrowing down the differential diagnosis.     

Feasibility of a prototype carbon nanotube enabled stationary digital chest tomosynthesis system for identification of pulmonary nodules by pulmonologists

BackgroundScreen detected and incidental pulmonary nodules are increasingly common. Current guidelines recommend tissue sampling of solid nodules >8 mm. Bronchoscopic biopsy poses the lowest risk but is paired with the lowest diagnostic yield when compared to CT-guided biopsy or surgery. A need exists for a safe, mobile, low radiation dose, intra-procedural method to localize biopsy instruments within target nodules. This retrospective cross sectional reader feasibility study evaluates the ability of clinicians to identify pulmonary nodules using a prototype carbon nanotube radiation enabled stationary digital chest tomosynthesis system.MethodsPatients with pulmonary nodules on prior CT imaging were recruited and consented for imaging with stationary digital chest tomosynthesis. Five pulmonologists of varying training levels participated as readers. Following review of patient CT and a thoracic radiologist’s interpretation of nodule size and location the readers were tasked with interpreting the corresponding tomosynthesis scan to identify the same nodule found on CT.ResultsFifty-five patients were scanned with stationary digital chest tomosynthesis. The median nodule size was 6 mm (IQR =4–13 mm). Twenty nodules (37%) were greater than 8 mm. The radiation entrance dose for s-DCT was 0.6 mGy. A significant difference in identification of nodules using s-DCT was seen for nodules <8 vs. ≥8 mm in size (57.7% vs. 90.9%, CI: −0.375, −0.024; P<0.001). Inter-reader agreement was fair, and better for nodules ≥8 mm [0.278 (SE =0.043)].ConclusionsWith system and carbon nanotube array optimization, we hypothesize the detection rate for nodules will improve. Additional study is needed to evaluate its use in target and tool co-localization and target biopsy.     

A case report of right atrial epithelioid hemangioendothelioma with multiple pulmonary metastases

Cardiac epithelioid hemangioendothelioma (EHE) is a very rare tumour of endothelial origin with the lung and liver as the most easily metastatic organs. We describe herein a patient with hemoptysis, severe anaemia, and diffuse pulmonary nodules with halo signs that represented metastasis of cardiac EHE; these radiologic manifestations are relatively uncommon. During the initial workup for the patient’s pulmonary nodules, echocardiography missed the cardiac mass. However, positron emission tomography‐computed tomography revealed increased fluorodeoxyglucose intake in the right atrial wall, and cardiac magnetic resonance imaging (MRI) revealed an irregular nodule with normal T1‐weighted signal intensity and hyperintense T2‐weighted signal intensity. Enhanced abdominal computed tomography (CT) revealed micronodular liver metastases. Video‐assisted thoracic surgery was performed to make a definitive diagnosis. Immunohistochemistry staining proved the diagnosis of EHE with positive results for cluster of differentiation (CD) 34, CD31, erythroblast transformation‐specific‐related gene and Ki‐67. The patient started chemotherapy with docetaxel (75 mg/m²) and gemcitabine (900 mg/m²), but this failed to control his disease and he died from an opportunistic infection related to his immunocompromised status 5 months later. For the work out process of bilateral diffuse pulmonary nodules suspicious for cardiac origin, especially with atrial deviation, echocardiography alone is not sufficient to exclude atrial origin. Cardiac CT or MRI might be a better choice.