A case of spontaneous pneumothorax due to metastasis of urinary bladder carcinoma to the lung

We reported a case of spontaneous pneumothorax associated with pulmonary metastasis of urinary bladder carcinoma. A 73-year-old male patient complaining of chest pain and dyspnea was admitted, who had undergone transurethral resection of the urinary bladder carcinoma and diagnosis of inactive pulmonary tuberculosis based on the chest X-ray film finding one year previously. On admission, chest X-ray film showed right pneumothorax, and the re-expanded chest X-ray film revealed a cystic lesion in the right upper lung field adjacent to the anterior chest wall, corresponding to the previous pulmonary lesion. Three months later, right thoracotomy was performed because of relapse accompanying by profuse air-leakage. There was a pleural fistula on the surface of the cystic lesion and necrotic tissue in it. Drainage bronchi were involved by surrounding tumorous tissue. Histological examination of the specimen from the cyst wall disclosed metastasis of transitional cell carcinoma of the bladder. The probable mechanism of the spontaneous pneumothorax probable consisted of stenosis of the right B3 bronchus caused by the metastatic tumor produced a check valve mechanism leading to cystic lesion, the rupture of which caused the spontaneous pneumothorax.     

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.     

Three cases of pulmonary eosinophilic granuloma]

CASE 1. A 55-year-old man was admitted because of an abnormality in chest radiographs. Chest HRCT showed multiple cystic lesions with thick and thin walls, and nodules; and strongly suggested pulmonary eosinophilic granuloma (EG). Open lung biopsy revealed granuloma formation and a fibrotic area consistent with EG. CASE 2. A 28-year-old woman was admitted because of chest pain and dyspnea. Chest radiography revealed bilateral pneumothorax. Chest HRCT showed multiple cystic lesions with thick walls involving the entire lung. As lung biopsy revealed, proliferative lesions and cavitation containing S-100 protein-positive histiocytes, we diagnosed this case as pulmonary EG in the active stage. CASE 3. A 32-year-old woman was admitted because of dyspnea. Chest CT showed bullous changes in the lung. As an open lung biopsy revealed, honeycomb changes with S-100 protein-positive histiocytes, this case was diagnosed as the regressive phase of EG. It is known that the chest radiography and HRCT findings of EG are characteristic and vary with the stage, so a surgical lung biopsy is necessary for diagnosis of EG. It is considered that the prognosis of the active phase of EG involving the entire lung is poor.     

A case of catamenial pneumothorax due to diaphragmatic endometriosis confirmed by video-assisted thoracoscopic surgery]

We describe a case of catamenial pneumothorax caused by diaphragmatic endometriosis and histologically confirmed thoracoscopically. The patient was a 33-year-old woman who had had recurrent right chest pain starting on the day preceding each menstruation since she was 26 years of age. In June 2001, at the age of 33, she felt right chest pain and developed dyspnea, and so came to our hospital. Chest radiography showed bilateral pneumothorax. Timely thoracoscopy revealed the characteristic appearances of catamenial pneumothorax such as a blueberry spot on the central tendon of the right diaphragm and a purplish-red locus in the right apex. Histopathological examination further confirmed the presence of endometrial tissue on the diaphragm as well as in the lesion of the right lung. Despite resection of the endometrial tissues from the right hemidiaphragm and the lung under thoracoscopy, right pneumothorax recurred after one month. Since then, the patient's condition has been well controlled by therapy with gonadotropin releasing hormone, with no recurrence of catamenial pneumothorax.     

Erdheim-Chester disease presenting with pneumothorax

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis most commonly characterized by symmetrical skeletal involvement and may present with pulmonary involvement leading to chronically progressive pulmonary symptoms. Characteristics on chest radiography include non-specific findings of diffuse interstitial and pleural thickening, micronodules, ground-glass opacities and parenchymal condensation as a result of infiltration by lipid-laden histiocytes. We present the case of a 50-year-old man with ECD presenting with acute pulmonary symptoms due to rupture of a large cystic lesion with resultant pneumothorax. He was brought by ambulance to our hospital, complaining of acute anterior chest pain and severe dyspnea. Chest radiography showed right-sided pneumothorax with a collapsed lung, a large, left-sided cystic lesion in the upper lung field and accentuated interstitial markings. Bullectomy and surgical biopsy were performed, demonstrating histologically histiocytic infiltrates that were strongly positive for CD68, but negative for S-100 protein and CD1a. Subsequent systemic examinations indicated widespread symmetrical skeletal involvement, leading to a definitive diagnosis of ECD.     

Pulmonary lymphangiomyomatosis (LAM) developing chylothorax

We describe a case of pulmonary lymphangiomyomatosis (LAM) with chylothorax that developed in a 46-year-old Japanese woman. This patient exhibited clinical symptoms of dyspnea and chest X-ray showed right pleural effusion. Thoracocentesis demonstrated chylous effusion. Chest computed tomography (CT) scan revealed multiple cystic lesions. Subsequent thoracoscopy revealed the chylorrhea from swelled vessels on the diaphragm. The clinical diagnosis, based on histological examinations with biopsy specimens obtained by thoracoscopy, was pulmonary LAM. Although the hormone therapy was not effective, chylous effusion was improved by the pleurodesis. Pulmonary LAM developing chylothorax is rare in Japan.     

A case of idiopathic bronchiolitis obliterans accompanied with pneumothorax and pulmonary atypical mycobacteriosis]

A 22-year-old man was urgently admitted for pneumothorax. He continued complaining of exertional dyspnea and dry cough after the pneumothorax healed. About three months later, an atypical pulmonary mycobacteriosis by mycobacterium kansasii was identified. Exertional dyspnea increased after chemotherapy was administered, and the patient was readmitted because of difficulty in daily life activities. Chest radiographs and CT scans showed bilateral pulmonary hyperinflation and a narrowed heart shadow. There was also marked combined ventilatory impairment, as identified by a respiratory function test. Furthermore, the histological findings of surgically removed lung tissue revealed accumulation of lymphocytes in the wall of a small bronchus. Idiopathic bronchiolitis obliterans was diagnosed from the clinical course and clinical findings. The patient is now being monitored and is awaiting lung transplantation.     

An autopsy case of chronic thromboembolic pulmonary hypertension with long-term progressive dyspnea]

A 71-year-old man who had been suffering from slowly progressive dyspnea on exertion for 16 years was admitted because of hemoptysis and severe dyspnea in 1997. His medical history included respiratory failure in 1986. At that time, chest X-ray films showed cardiomegaly and striking enlargement of hilar pulmonary arteries, while a lung perfusion scan revealed segmental defects. A diagnosis of chronic thromboembolic pulmonary hypertension (CTPH) was made on the basis of multiple filling defects detected by pulmonary angiography and marked pulmonary hypertension (104/29 mmHg) measured by right heart catheterization. The patient died unexpectedly 1 month later. Autopsy revealed a large new thrombotic lesion straddling the organizing thrombotic lesion in the right pulmonary artery. It is important to keep CTPH in mind when examining and treating patients with unexplained dyspnea. In this respect, our case was considered of value to an understanding of the natural course of CTPH.     

Case of pneumothorax associated with pulmonary Mycobacterium fortuitum infection

A 39-year-old man with dyspnea was revealed to have severe pneumothorax and received partial resection of the left upper lobe after unsuccessful drainage. Necrotizing epitheloid granuloma was found in the resected lung and Mycobacterium fortuitum was detected from the lesion. Chemotherapy with levofloxacin and clarithromycin was started one year after surgery because of the newly found nodular shadow near the lesion. The case experienced pyothorax due to pulmonary tuberculosis three years before and Mycobacterium avium pleuritis one year before this episode. Three-time mycobacterial pleural infection in three years seems to be uncommon. Furthermore this is the first report of pneumothorax associated with pulmonary Mycobacterium fortuitum infection.     

Simultaneous bilateral pneumothorax complicating Pneumocystis carinii pneumonia in a homeless man with AIDS]

Although pneumothorax is a well-known complication of AIDS related Pneumocystis carinii pneumonia, simultaneous bilateral pneumothorax has not been reported in Japan. A 54-year-old homeless man was admitted with emaciation and dyspnea. Chest X-ray showed diffuse ground glass opacity. Computed tomography of the chest demonstrated ground glass opacity and cyst-like lesions in both upper lobes of the lung. The patient was HIV positive. Grocott's stain of a bronchial lavage specimen demonstrated Pneumocystis carinii microorganisms. Intravenous trimethoprim-sulfamethoxazol and steroid administration was started. Seven days later, the patient developed tension pneumothorax of the right lung. Immediately after the thoracostomy, contralateral pneumothorax developed. Another chest tube was inserted. Subsequently, the patient died from bilateral pneumothorax. The endemicity of HIV infections among the homeless population is already a significant problem in Western countries. We should be aware that HIV infection may become a problem among the Japanese homeless as well.     

Refractory pneumothorax secondary to lung cancer in a patient with idiopathic pulmonary fibrosis

An unusual case of refractory pneumothorax secondary to lung cancer in a 69-year-old man patient with idiopathic pulmonary fibrosis (IPF) is described. High-pressure suction applied through chest tube did not resolve the large right pneumothorax. Acute exacerbation of IPF has also appeared. Respiratory state worsened acutely, and the patient died on the fifth hospital day. In the present case, the large right pneumothorax was initially thought to be associated with IPF because pneumothorax is common in patients with IPF. However, postmortem microscopic examination revealed that the refractory pneumothorax was secondary to perforation of the pleura due to a necrotic peripheral lung cancer.     

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.     

Lymphangiomyomatosis with a large retroperitoneal tumor]

A case of lymphangiomyomatosis (LAM) in a 35-year-old woman is reported. Because she was already severely dyspneic when she was admitted to our hospital, neither TBLB nor open lung biopsy was performed, and no accurate diagnosis was made. She died of respiratory failure three years after the development of exertional dyspnea, and autopsy revealed LAM. Pneumothorax, a well-known and frequent complication of LAM, did not occur until one month prior to her death. Although pneumothorax frequently complicates LAM, there are a few reports of cases in which pneumothorax did not occur during the course of the disease. We emphasize that LAM should be considered in the differential diagnosis of diffuse interstitial lung disease, even when the patient has no pneumothorax. The retroperitoneal tumor (15 x 10 x 10 cm), which was evident clinically, was later shown to be intra-abdominal involvement of LAM. Although only a few cases of LAM with a large retroperitoneal tumor have been reported, minor intra-abdominal involvement is relatively common. This case also suggested that the finding of a retroperitoneal tumor may be a diagnostic clue in LAM.     

Unilateral pulmonary edema: a rare presentation of congestive heart failure

The authors present a case of an 85-year-old woman known to suffer from severe congestive heart failure who presented with dyspnea and a unilateral infiltrate in the right lung on chest x-ray. Following clinical judgment, she was diagnosed with unilateral pulmonary edema and was treated accordingly, with rapid improvement of symptoms and disappearance of the infiltrate within 12 hours. The patient had been hospitalized many times during the previous years with pulmonary edema affecting both lung fields. Unilateral pulmonary edema is an unusual clinical condition that has been reported as a manifestation of left heart failure, mostly affecting the right lung. The authors emphasize the possible presentation of unilateral pulmonary edema in a patient with heart failure and recurrent bilateral pulmonary edema.     

Two cases of pneumothorax using the thoracic vent (TV) on an outpatient basis]

In case 1, a 73-year-old man developed dyspnea on exertion while travelling abroad. The symptom continued until the patient returned to Japan 5 days after onset and visited our clinic on the next day. SaO2 was 93% and chest radiography demonstrated pneumothorax with 75% collapse of the right lung. Insertion of a TV successfully prevented air leakage stopped within 2 days. The TV was removed after the lung collapse disappeared, as shown on the chest radiograph taken 4 days after its insertion. Case 2 occurred in a 62-year-old woman with underlying idiopathic interstitial pneumonitis, who had had dyspnea on exertion for approximately 5 years. The patient visited our clinic because the dyspnea on exertion became abruptly exacerbated. A TV was inserted after an SaO2 of 82% was measured and the chest radiograph revealed pneumothorax with a 50% collapse of the right lung. Air leakage stopped after 5 days, and the TV was removed on the 8th day after insertion. We concluded that TV is very useful for improving the QOL of patients and enables outpatient management of pneumothorax, resulting in a reduction in the cost of treatment.     

A case of pulmonary eosinophilic granuloma with involvement of the ocular fundus]

A 37-year-old man was admitted to our hospital because of abnormal shadows on chest X-ray film at routine medical examination. The chest X-ray showed diffuse granular shadows in the bilateral upper and middle lung fields. Chest CT showed multiple cystic lesions and nodular lesions. Bronchoalveolar lavage (BAL) and transbronchial lung biopsy (TBLB) were performed. Electron microscopic examination of BAL fluid revealed Langerhans' cells, and lung tissue specimen obtained by TBLB showed granuloma with infiltration of histiocytes and eosinophils. During the clinical course, the patient complained of cough and decrease of right visual acuity in association with an increase of abnormal shadows on chest X-ray. The lung disease gradually resolved after the cessation of smoking in spite of no therapy. In the right eye, an exudative lesion with retinal elevation was noted in the ocular fundus. Corticosteroid administration was effective for the treatment of this lesion. There was no pathological evidence of the right ocular lesion. However, the correlation of its clinical course with the lung disease suggests a relationship between the ocular lesion and pulmonary eosinophilic granuloma.     

A case of primary pulmonary proteinosis with bilateral pneumothorax]

A 40-year-old man was admitted to our hospital because of chronic cough and mild exertional dyspnea. Radiographs of the chest showed diffuse infiltrative shadows in both lung fields. A diagnosis of pulmonary alveolar proteinosis (PAP) was made by examination with a flexible bronchoscope and bronchoalveolar lavage, with transbronchial biopsy. After diagnosis, the patient underwent whole-lung lavage with temporary improvement. Due to the recurrence of his illness, he needed a total of four whole-lung lavages over the course of his illness. However, the exertional dyspnea became progressively worse. Bilateral pneumothorax developed suddenly and led to his death. This case indicates the possibility that deterioration of PAP despite whole-lung lavage may sometimes be followed by pneumothorax.     

肺淋巴管肌瘤病并肺母细胞瘤1例临床分析并文献复习

目的 加强肺淋巴管肌瘤病（LAM）合并恶性肿瘤的临床、影像、病理特点的认识,提高对该病的早期认识和治疗水平.方法 分析2013年3月收治的1例肺LAM并肺母细胞瘤患者临床资料及诊治经过,并结合相关文献进行复习.截止至2014年2月,以“Lymphangiomyomatosis、cancer”为检索词,在PubMed检索系统进行检索,论著3篇,综述1篇;在万方数据库中以“淋巴管肌瘤病、恶性肿瘤”为检索词进行检索,病例报告1篇.结果 患者,女,37岁,慢性病程,主要症状为咳嗽、咯血、胸痛3个月,既往有“双肾错构瘤”史,曾行右肾错构瘤切除术,术后病理示血管平滑肌脂肪瘤,左肾未予处理.曾有“自发性气胸”病史.胸部高分辨率CT示右中下肺巨大肿块并纵隔淋巴结肿大,考虑恶性病变;肺LAM.血清血管内皮生长因子D为1 092.61 ng/L.免疫组化示CD56灶（＋）,Vim小细胞（＋）,Syn（＋）,CD99（＋/-）,组织改变考虑为肺母细胞瘤.复习国内外文献,尚无LAM合并肺母细胞瘤的病例报道.结论 近十年来,随着LAM分子发病机制的深入研究,LAM目前可定义为低度恶性转移性肿瘤.本例患者为LAM合并高度恶性肿瘤肺母细胞瘤,在国内外尚属首例报道.但究其机制,是LAM细胞癌变为肺母细胞瘤,或是二者同时存在,目前尚无定论.     

A case of idiopathic interstitial pneumonia with pneumothorax and pneumomediastinum, accompanied by rapid progression of cystic changes on CT

A case of idiopathic interstitial pneumonia (IIP) with pneumothorax and pneumomediastinum was presented. A 52-year-old male was admitted with the complaint of cough, fever and dyspnea. Chest roentgenogram revealed bilateral reticulonodular shadow and right pneumothorax. Radiological, laboratory and clinical findings were consistent with the diagnosis of acute exacerbation of IIP. Repeated chest CTs showed diffuse interstitial opacities and the presence of pneumothorax and pneumomediastinum. Furthermore, honeycombing and bullous change progressed markedly for two months. The mechanisms of pneumothorax and pneumomediastinum were speculated to be due to the rupture of those cystic lesions which had grown rapidly on follow-up CTs. It was suggested that the formation of honeycombing occurred within a few months and the increasing of honeycombing might be accompanied by so-called acute exacerbation of IIP. We propose that follow-up CTs were useful for the chronological study for interstitial lesions of IIP.     

A case of silicoproteinosis with pneumothorax]

A 46-year-old man was admitted because of an increasingly severe cough and dyspnea on exertion. For 13 years, he had inhaled sand containing 100% crystalline silica (SiO2). Chest radiographs revealed right pneumothorax and diffuse small nodular and ground-glass opacities in both lungs (especially in the upper lung fields). A chest CT scan disclosed several bullae in both upper lobes, and an open lung biopsy was performed along with resection of these bullae. Subsequently, silicotic nodules containing silica and PAS-positive materials were recognized in the alveolar spaces in the histological findings, and a diagnosis of silicoproteinosis was made. We have reported on this case of silicoproteinosis with pneumothorax which progressed for over one year and which showed unusual radiological findings dissimilar to those of primary pulmonary alveolar proteinosis.