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.     

A case of primary pulmonary MALT lymphoma as a nodular shadow on CT scan, and relapsed with diffuse micronodular shadows after surgical resection at 7 years ago]

A 76-year-old man without symptoms was admitted to our hospital for investigation of an abnormal chest shadow in 1994. His chest radiograph showed a nodular shadow in the lingual lobe. Segmentectomy was performed and the histological diagnosis was MALT (mucosa-associated lymphoid tissue) lymphoma. In June 2001, there were no abnormal shadows on the chest radiograph or in 10 mm slice CT. However, high-resolution CT with 2-mm slice thickness revealed diffuse micronodular shadows in both lungs. A relapse of MALT lymphoma was diagnosed by immunohistochemical analysis of tissue specimens obtained by transbronchial lung biopsy. It was observed that the patterns of radiological findings in primary pulmonary MALT lymphoma cases may differ between the initial state and a relapse.     

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.     

累及肺部的血管内淋巴瘤病一例并文献复习

目的 报道1例以肺部症状和体征为主要表现的血管内淋巴瘤病.方法 对2008年3月入住北京协和医院的36岁男性患者进行临床、影像学、病理组织学检查,明确诊断后行环磷酰胺+表柔比星+长春新碱+泼尼松(CHOP)方案化疗.从文献中检索到14例以肺部症状和体征为主要表现,无皮肤和中枢神经系统临床表现的血管内淋巴瘤病.对15例的临床、影像学、病理组织学特点、治疗及预后进行总结.结果 患者以发热和肺部多发结节就诊,经开胸肺活检病理证实为T细胞性血管内淋巴瘤病,虽经CHOP方案化疗,但病情不断加重,诊断20 d后死亡.总结以肺部及呼吸系统症状和体征为主要表现的血管内淋巴瘤病主要见于老年人,年龄为36～79岁,中位年龄59岁;男11例,女4例;主要症状为气短(10/15)、发热(9/15)、咳嗽(5/15)、体重减轻(5/15)和乏力(2/15)等;肺功能检查以弥散功能障碍为主,可伴有不同程度的阻塞性通气功能障碍,1例有肺动脉高压,8例的血清乳酸脱氢酶升高.胸部CT示双肺弥漫性条索状阴影和(或)多发结节影,部分结节呈楔形,位于胸膜下,常伴有肺支气管血管束增粗.10例为B细胞性,3例为T细胞性,2例未区分细胞类型.有随访资料的B细胞性血管内淋巴瘤病6例,均行CHOP方案化疗,4例存活,2例死于呼吸衰竭;3例T细胞性血管内淋巴瘤病的预后均较差,经CHOP方案化疗的效果不佳,分别于诊断后20 d和3个月死亡.结论 以肺部症状和体征为主要表现的血管内淋巴瘤病较为少见,其临床症状无特异性,肺功能检查主要为弥散功能障碍,常伴有血清乳酸脱氢酶升高,胸部CT示双肺弥漫性条索状阴影和(或)多发结节影,部分结节呈楔形,位于胸膜下,常伴有肺支气管血管束增粗,纵隔肺门淋巴结无肿大.经支气管肺活检或开胸肺活检病理检查可明确诊断.     

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.     

A case of multicentric Castleman's disease with pulmonary involvement]

In a man aged 34 who had been experiencing frequent coughing since November 2001, a chest radiograph showed infiltration shadows in both lung fields. Chest CT showed diffuse centrilobular nodules and multiple mediastinal lymphadenopathy. Laboratory examination revealed high values for C-reactive protein and the erythrocyte sedimentation rate, together with polyclonal hyperimmunoglobulinemia and an elevated interleukin-6 level. We suspected multicentric Castleman's disease, and so performed thoracoscopic mediastinal lymph node biopsy and lung biopsy. The former disclosed follicular hyperplasia and plasma cell infiltration in the interfollicular area, suggesting a diagnosis of Castleman's disease, plasma cell type. The lung biopsy showed heavy infiltration of plasma cells. The diagnosis was therefore multicentric Castleman's disease (MCD) with pulmonary involvement. The chest CT findings were tpical characteristics of pulmonary involvement in patients with MCD.     

A case of multiple nodular pulmonary amyloidosis complicated with primary Sjogren syndrome]

A 79-year-old woman was admitted to the Department of Orthopedics Surgery for treatment of osteoarthritis in her knee. Multiple pulmonary nodular lesions were found on preoperative chest x-ray film screening. Metastatic lung tumor was suspected, but no tumorous lesions were detected in other organs. CT guided lung biopsy was performed. Histopathological examination revealed amyloid consisting of homogenous eosinophilic materials. No amyloid deposits were detected in other organs, so we diagnosed localized nodular pulmonary amyloidosis. She was subsequently given a diagnosis of primary Sj?gren syndrome. We believe that such a case of multiple nodular pulmonary amyloidosis with Sj?gren syndrome is rare, and the case showed interesting radiological findings, such as mimicking metastatic lung tumor.     

Two cases of idiopathic pulmonary hemosiderosis: analysis of chest CT findings]

Chest CT findings are reported in two cases of idiopathic pulmonary hemosiderosis. In both cases, CT was performed after remission of an acute exacerbation following corticosteroid therapy. Case 1 was a 17-year-old woman with Down's syndrome. Chest radiograph showed diffuse ground-glass like and reticulonodular shadows, which were predominant in the bilateral lower lung fields. Chest CT showed a diffuse increase of lung filed density, especially in the dorsal zone of both lower lobes. Open lung biopsy revealed hemorrhage and numerous hemosiderin-laden macrophages in the alveoli, and in addition, marked fibrous thickening of the alveolar septa. Case 2 was a 7-year-old girl. Chest radiograph showed diffuse micronodular shadows in both lungs. Chest CT showed diffuse poorly-circumscribed micronodular lesions with uniform distribution, and lung field density was normal except for the right upper lobe with patchy infiltrates. Although lung biopsy was not performed, fibrous thickening of the alveolar septa was presumed to be mild even if present, since pulmonary function and blood gas analysis were within normal limits. In these two cases, lung field density of CT seemed to reflect the degree of diffuse fibrous thickening of the alveolar septa, and it is suggested that CT is valuable in the evaluation of fibrous thickening of the alveolar septa secondary to recurrent pulmonary hemorrhages. Comprehensive review of CT findings of idiopathic pulmonary hemosiderosis was also performed.     

Thoracic ultrasonography for the pulmonary specialist

Thoracic ultrasonography is a noninvasive and readily available imaging modality that has important applications in pulmonary medicine outside of the ICU. It allows the clinician to diagnose a variety of thoracic disorders at the point of care. Ultrasonography is useful in imaging lung consolidation, pleural-based masses and effusions, pneumothorax, and diaphragmatic dysfunction. It can identify complex or loculated effusions and be useful in planning treatment. Identifying intrathoracic mass lesions can guide sampling by aspiration and biopsy. This article summarizes thoracic ultrasonography applications for the pulmonary specialist, related procedural codes, and reimbursement. The major concepts are illustrated with cases. These case summaries are enhanced with online supplemental videos and chest radiograph, chest CT scan, and ultrasound correlation.     

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.     

A case of pulmonary alveolar proteinosis with invasion of macrophages in alveolar interstitial region]

A 24-year-old woman was admitted to our hospital on November 2, 1989 for investigation and treatment of abnormal shadows detected in her routine chest radiograph on July 19, 1989. The chest X-ray film on admission showed diffuse infiltrative shadows in the peripheral regions of the bilateral middle and lower lung fields. In addition centrilobular shadows in the subpleural regions with increases irregular densities were found on chest CT. We therefore suspected pulmonary alveolar proteinosis. Bronchoalveolar lavage from the right middle bronchus and transbronchial lung biopsy of the right upper and lower lobes were performed. Electron microscopic examination of a specimen of bronchoalveolar lavage fluid revealed many multilamellar bodies, characteristic of pulmonary alveolar proteinosis. In addition, proteinaceous material containing multilamellar bodies was observed within the alveolar lumina. We diagnosed this case as pulmonary alveolar proteinosis. Electron microscopy also revealed many macrophages in the alveolar walls, some of which contained a few or numerous multilamellar bodies within the secondary lysosomes. We treated this patient with oral and inhaled Ambroxol, with improvement of her clinical condition.     

Pulmonary involvement in systemic sclerosis: the detection of early changes by thin section CT scan, bronchoalveolar lavage and 99mTc-DTPA clearance

Systemic sclerosis is frequently complicated by fibrosing alveolitis although clinical and radiological abnormalities are not usually apparent until the lung disease is well established. The aim of this study was to investigate pulmonary involvement in systemic sclerosis by thin section CT scan, bronchoalveolar lavage (BAL) and 99mTc-DTPA clearance studies, and assess the value of these tests in defining pulmonary abnormalities in patients with a normal chest radiograph. Patients were divided into those with an abnormal chest radiograph (Group I, n = 14) and those with a normal chest radiograph (Group II, n = 16). CT scans were abnormal in all patients in Group I and 7 of 16 (44%) in Group II. BAL inflammatory cell counts were raised in all 12 (100%) patients studied in Group I and 11 of 15 (73%) in Group II. There was no difference in the type of inflammatory cells observed between the two groups. 99mTc-DTPA clearance was faster than normal controls in ten of 14 patients (71%) in Group I and seven of 15 (47%) in Group II and correlated with carbon monoxide transfer factor (P less than 0.05). Lung biopsies were performed on nine patients in Group I and three in Group II all of whom had abnormal CT scans. Fibrosing alveolitis was confirmed in every case. Group II biopsies could not be distinguished from Group I biopsies; both showed fibrosis as well as inflammation suggesting that pulmonary fibrosis is an early abnormality in systemic sclerosis. Our results indicate that CT scans, BAL and 99mTc-DTPA are frequently abnormal in asymptomatic patients with systemic sclerosis who have normal chest radiographs. When the CT scan is normal abnormalities of BAL and/or 99mTc-DTPA (99mTechnetium diethylenetriamine pentacetate) clearance may indicate lung disease at a still earlier stage. This observation requires further investigation.     

A case of pulmonary sarcoidosis with pneumothorax and pleural effusion after improvement of pulmonary impairment]

A 23-year-old man was admitted to our hospital for a complete medical evaluation of abnormal pulmonary shadows found on a chest radiograph during his annual check-up. Chest radiography and chest CT showed a diffuse spread of micronodules in both lung fields and mediastinal lymphadenopathy. A transbronchial lung biopsy demonstrated evidence of noncaseating epithelioid cell granuloma with multinucleated giant cells, and a diagnosis of sarcoidosis was made. The pulmonary shadows improved without therapy. Twenty months later, the patient was readmitted to the hospital because of chest pain and dyspnea. Pneumothorax appeared on the right in a chest radiograph, but subsided after drainage therapy, and two weeks later, a right side pleural effusion was seen. We determined that the pleura was involved in the sarcoidosis, and the patient was treated with oral prednisolone 20 mg daily. The pleural effusion gradually subsided. This is the first reported case in Japan of pulmonary sarcoidosis with pneumothorax and pleural effusion after improvement of pulmonary impairment.     

Two cases of prostate cancer found primarily from lung metastatic lesions]

We report two cases of prostate cancer found primarily from a metastatic lesion appearing in a chest radiograph. Patient 1 was admitted to our hospital because his chest radiograph and chest CT showed pleural effusion on the left. Thoracocentesis and pleural biopsy were unremarkable, so he was observed as both an outpatient and an inpatient. His general condition worsened, and after the third admission, he died. His autopsy revealed prostate cancer and positive immunohistochemical reactions for PSA and PSAP in both lungs, and prostate specimens demonstrated that prostate cancer had metastasized to the lung. Patient 2 was referred for evaluation of a bilateral multiple nodular shadow in a chest radiograph, and prostate cancer was discovered. Immunohistochemical reactions for PSA and PSAP were positive in both specimens of TBLB and prostate biopsy, confirming that the multiple lung tumors were metastases from prostate cancer.     

高分辨CT、胸部平片及病理学诊断肺磨玻璃结节临床分析

目的分析高分辨CT与胸部平片联合用于肺磨玻璃结节鉴别诊断的价值,以提高肺磨玻璃结节早期检出率,为临床合理治疗提供影像学依据。 方法选择2018年1月至2019年12月经手术病理结果或穿刺活检证实860例肺磨玻璃结节患者作为分析对象,全部患者在术前均接受胸部平片检查与高分辨CT检查,且均经高分辨率CT与胸部平片发现有肺部结节征象;记录手术病理及穿刺活检结果;观察患者胸部平片与高分辨CT检查主要征象,分析各检查方法单独及联合用于肺磨玻璃结节鉴别诊断的价值。 结果860例患者中经证实恶性385例,其中289例经手术病理确诊,96例经穿刺活检明确,包括原位腺癌135例,微浸润腺癌155例,浸润性腺癌72例,其他23例,恶性率为44.8%;860例肺磨玻璃结节患者经胸部平片检查结果显示,肺结节直径范围为1.0～3.0 cm;肺结节的分布：326例左肺,534例右肺;经高分辨CT检查结果显示,良恶性病变肺磨玻璃结节病灶大小比较,差异无统计学意义(P>0.05),恶性结节表现出不规则形、毛刺征、分叶征、界面清楚光整、空泡征、支气管征等征象占比均高于良性组(P<0.05);胸部平片、高分辨CT单独及联合诊断肺磨玻璃结节的曲线下面积分别为0.847、0.876、0.940,均>0.8,诊断价值好,且联合诊断的曲线下面积最大。 结论胸部平片与高分辨CT联合检测,并结合病理学对提高肺磨玻璃结节早期良性病变及恶性肿瘤进行鉴别诊断的正确率有重要价值。     

An early stage case of pulmonary lymphangiomyomatosis

A 35-year-old unmarried woman was admitted to our hospital because of right pneumothorax. Pulmonary lymphangiomyomatosis was suspected from CT and transbronchial lung biopsy findings. The diagnosis was confirmed histologically by open lung biopsy. As her biopsy specimen showed positive findings for both estrogen and progesterone receptor, tamoxifen administration was started. The CT revealed multiple cystic lesions (5-10 mm in diameter) but otherwise normal vascular structures and normal lung density. The plain chest radiograph showed no abnormalities. CT is very useful for the early detection of pulmonary lymphangiomyomatosis.     

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.     

肺淋巴管癌病

目的探讨肺淋巴管癌病（ＰＬＣ）的临床特征，分析相关诊断方法的特点，提高对ＰＬＣ的认识及其诊断水平。方法结合文献资料回顾分析４例临床病例。结果ＰＬＣ在临床上主要表现为咳嗽、气短；肺功能呈正常或限制性通气功能障碍；Ｘ线胸片常见双肺弥漫性网状、网状结节状或结节状等间质纤维样改变，并合并胸腔积液、高分辨ＣＴ可发现小叶间隔的不均匀增厚，不均一的支气管血管束结节状增厚，胸膜不规则增厚，纵隔淋巴结肿大等；胸膜肺组织病理可见胸膜下、肺内淋巴管内癌细胞生长。结论对双肺呈类间质纤维化改变，同时合并胸腔积液和纵隔淋巴结肿大的患者，应进一步行高分辨ＣＴ及胸膜肺组织病理活检，以期排除ＰＬＣ之诊断。     

A case of micronodular pneumocyte hyperplasia diagnosed through lung biopsy using thoracoscopy

We present a case of a 39-year-old woman with sporadic tuberous sclerosis (TSC), whose chest radiograph demonstrated bilateral diffuse nodular shadowing. A transbronchial lung biopsy specimen revealed the possibility of multiple atypical adenomatous hyperplasia (AAH), which had not been reported in TSC. Thoracoscopic lung biopsy was, therefore, performed. The specimens revealed the characteristic histological and immunohistochemical features of micronodular pneumocyte hyperplasia, which has been reported as an extremely rare pulmonary manifestation of TSC. In addition, no evidence of AAH or any other pulmonary involvements of TSC including lymphangioleiomyomatosis were detected in biopsy specimens obtained at thoracoscopy.     

肺放线菌病一例并文献复习

目的 提高对肺放线菌病发病机制、诊断、临床特点及治疗的认识.方法 报道2007年3月解放军总医院附属第一医院呼吸科收治的1例肺放线菌病病例并进行相关文献复习.结果 患者女,66岁.反复出现咳嗽、咳痰、低热4年,多次X线胸片均示右中叶肺炎,经支气管镜检查及病理诊断为肺放线菌病.经林可霉素联合磺胺甲嚼唑/甲氧苄啶治疗6个月后痊愈.结合本例并复习近20年国内外文献报道的187例肺放线菌病(国外162例,国内25例)资料,结果 显示本病男性多见,发病年龄为12～87岁;咳嗽、咳痰、胸痛为常见症状,咳黄色颗粒样物为典型表现,但并不多见.胸部CT可表现为实质团块影、片状影、实变影伴支气管充气征、空洞、纵隔淋巴结肿大、胸腔积液及胸膜增厚等.病变部位以左下叶及右中叶居多,与肺癌、肺结核易混淆.确诊主要依靠经支气管镜活检病理及手术病理检查,痰涂片及培养阳性率低.青霉素、四环素、红霉素、磺胺类或林可霉素等抗生素治疗及手术切除是主要的治疗手段.结论 肺放线菌病是一种少见病,早期诊断和治疗可获得良好疗效.