Pulmonary mucormycosis with fatal massive hemoptysis.

A case of pulmonary mucormycosis in a 57-year-old woman with acute promyelocytic leukemia (APL) who died of massive hemoptysis is reported. Chest radiography revealed changes that began with a small focal infiltration and progressed to a large round nodule with cavity formation. Postmortem examination showed the nodule to be composed mainly of infarcted lung tissue with saprophytic growth of Mucor. An adjacent proximal branch of the left pulmonary artery was thrombosed with mucoraceous hyphae, and it had ruptured into the cavitary space around the necrotic tissue and then into a conducting bronchus. In general, both fatal massive hemoptysis and cavity formation are rare in pulmonary mucormycosis. In our present case, the histological findings suggested that both phenomena were closely related to the pulmonary infarction caused by Mucor invasion of the pulmonary artery.     

A histologic study of extralobar pulmonary sequestration and its anomalous artery as a clue to its development

In 1997, an anomalous small mass of soft tissue was found in the left thoracic cavity of a Japanese male cadaver during student dissection practice. It was linked only by branches from the thoracic aorta, hemiazygos vein and greater splanchnic nerve, with no connection to the normal lung by vessels or airway. Examination of cut sections clarified that this anomalous small tissue mass was an extralobar pulmonary sequestration (EPS). Many cases of pulmonary sequestration have been reported from the clinical aspect, but there are few from a purely anatomical viewpoint. In the present case, detailed gross anatomical and histologic observations clarified that the anomalous artery supplying the sequestration resembled an elastic artery. In view of the findings of other reports, the anomalous arteries are divided into two types, the elastic type such as the pulmonary artery and the muscular type such as the bronchial artery. So it is thought that the histologic observation of an anomalous artery in comparison with normal pulmonary and bronchial arteries may offer an important clue to consider in elucidating the origin and development of pulmonary sequestration.     

儿童先天性体动脉-肺动脉瘘4例并文献复习

目的总结临床表现为大量咯血的先天性体动脉-肺动脉瘘患儿的临床特点及诊治经验。方法回顾性收集首都医科大学附属北京儿童医院2007年3月至2008年2月诊断为先天性体动脉-肺动脉瘘4例患儿的临床资料,总结其临床表现、胸部X线片、胸部64排CT增强扫描三维容积再现（3D-VR）、数字减影血管造影（DSA）、治疗及随访情况。结果男1例,女3例,最大发病年龄为11岁,最小发病年龄为2个月,主要表现为咯血。1例有杵状指,3例未见特异性体征。胸部X线片检查：3例未见异常,1例示肺间实质浸润。胸部64排CT增强扫描3D-VR检查：1例提示支气管动脉迂曲,但未见异常交通;1例导管栓塞治疗（TCE）后示右侧支气管动脉扩张迂曲,并与肺动脉相通可能性大,考虑支气管动脉-肺动脉瘘;1例提示支气管动脉-肺动脉瘘;1例未见异常。3例DSA提示为支气管动脉-肺动脉瘘,病变部位均位于右下肺,并行TCE。随访至2009年2～5月,1例复发,表现为痰中带血,胸部64排CT增强扫描未见异常,余3例未复发。3例怀疑为遗传性出血性毛细血管扩张症（HHT）,1例考虑为HHT高度危险者。结论体动脉-肺动脉瘘可造成大量咯血。胸部X线片一般无特异性表现,胸部64排CT增强扫描3D-VR可显示病变部位,明确诊断需行DSA。可采用TCE治疗。TCE远期效果应进行长期随访予以明确。     

Isolated pulmonary mucormycosis in an apparently normal host: a case report.

Mucormycosis is a rare fungal disease commonly affecting individuals with diabetes mellitus, hematological malignancy, and immune deficiency. Isolated pulmonary mucormycosis is extremely rare. This article reports a case of isolated pulmonary mucormycosis that presented as a solitary cavity infiltrate in a patient with no underlying risk factors.     

Fatal hemoptysis from Behcet's disease in a child.

OBJECTIVE: To present a case of pulmonary artery aneurysms (PAAs) in a child with Behcet's disease (BD). NATURE OF THE STUDY: Case report and review of the literature. MATERIALS AND METHODS: The patient had a complete autopsy, drug screen and serological testing. A medical history was obtained and the literature was reviewed. RESULTS: A 10-year-old African-American boy had massive hemoptysis while at home and died shortly thereafter in the emergency department of a local hospital. He had had two other episodes of hemoptysis in the past 2 months. At autopsy, he had bilateral inflammatory aneurysms of the lower lobe branches of the pulmonary arteries. The left aneurysm had eroded into the adjacent bronchus and accounted for the fatal hemoptysis. The right ventricle had endocardial fibrosis. Systemic vasculitis was absent. The child did not have oral, genital, eye or skin lesions at autopsy, or by clinical history. Based on the autopsy findings, a diagnosis of BD was made. CONCLUSIONS: We present the very unusual case of a 10-year-old African-American boy who died from hemoptysis resulting from bilateral pulmonary aneurysms caused by BD.     

Pulmonary mucormycosis presenting as massive fatal hemoptysis in a hemodialytic patient with chronic renal failure

Summary A 38 year old female patient died following massive hemoptysis occurring during maintenance hemodialysis for chronic renal failure. In addition to renal dysfunction, laboratory data showed low levels of serum immunoglobulins; chest X-rays did not reveal any abnormal shadows. For the last 11 months, the patient had not received deferoxamine. At autopsy, major bronchi were plugged with coagulated blood. Irregular thickening of the right main bronchial wall close to the lung hilus was noted. Light microscopic examination disclosed mycotic granulomata in the bronchial wall, where the pulmonary arterial wall was also involved. Immunostaining using specific antibody identifiedMucor hyphae. The mucormycosis is a serious complication of chronic renal failure and hemodialyis. In the current case, it is likely that immune dysfunction rather than deferoxamine was the important predisposing factor to the growth of the fungus.     

Pulmonary aspergillosis and the importance of oxalate crystal recognition in cytology specimens

A 62-year-old man, previously healthy but alcoholic, and who was clinically thought to have bacterial pneumonia, presented with a pulmonary infiltrate in the right apex, and suddenly died of exsanguinating hemoptysis. Sputum cultures yielded Aspergillus niger and Candida krusei while sputum cytology revealed numerous birefringent crystals in a background of acute inflammatory exudate. Autopsy findings showed invasive aspergillosis with a large mycetoma-containing cavity in the lung that was associated with localized massive oxalosis. This case further substantiates the fact that the presence of calcium oxalate crystals in pulmonary biopsy and cytology specimens can be regarded as an important diagnostic aid in the diagnosis of pulmonary aspergillosis due to A niger.     

Hepatic and small bowel mucormycosis after chemotherapy in a patient with acute lymphocytic leukemia

Mucormycosis is a rare but invasive opportunistic fungal infection with increased frequency during chemotherapy-induced neutropenia. The clinical infections due to Mucor include rhinocerebral, pulmonary, cutaneous, gastrointestinal and disseminated diseases. The first two are the most common diseases and all entities are associated with a high mortality rate. Still hepatic involvement of Mucor is rarely reported. We experienced a case of hepatic and small bowel mucormycosis in a 56-year-old woman after induction chemotherapy for B-cell acute lymphocytic leukemia. Initial symptoms were a high fever unresponsive to broad spectrum antibiotics and pain in the left lower abdominal quadrant. It was followed by septic shock, deterioration of icterus and progressively elevated transaminase. An abdominal CT demonstrated multiple hypodense lesions with distinct margins in both lobes of liver and pericolic infiltration at small bowel and ascending colon. Diagnosis was confirmed by biopsy of the liver. The histopathology of the liver showed hyphae with the right-angle branching, typical of mucormycosis. The patient was managed with amphotericin B and operative correction of the perforated part of the small bowel was performed. However, the patient expired due to progressive hepatic failure despite corrective surgery and long-term amphotericin B therapy.     

Life-threatening hemoptysis caused by chronic idiopathic pulmonary hilar fibrosis with unilateral pulmonary vein occlusion

We present the case of a patient who has a life-threatening hemoptysis caused by occlusion of the right pulmonary vein owing to external compression by excessive fibrous tissue. Because the patient's lung was essentially nonfunctional and hemoptysis persisted, we performed a pneumonectomy on her. Pathological analysis of the lung showed severe hypertensive changes in the arterial and venous microvasculature of the lung. Parenchymal-pleural to intercostal systemic venous connections had developed, facilitating venous drainage of the lung circulation. Upon review of the patient's history and the pathological and radiological evidence, we concluded that the pathological changes were caused by a variant of mediastinal fibrosis termed chronic idiopathic pulmonary hilar fibrosis, of which only a few cases have been described in the literature. A detailed case history and review of the literature are presented.     

A case of pulmonary mucormycosis presented as Pancoast syndrome and bone destruction in an immunocompetent adult mimicking lung carcinoma

Pulmonary mucormycosis is a rare opportunistic infection caused by Mucormycosis. This fungal infection is uncommon in immunocompetent individuals. Because of its various clinical and imaging manifestations, it is a diagnostic challenge to distinguish pulmonary mucormycosis from other pulmonary diseases, such as carcinoma. Herein, we report a case of pulmonary mucormycosis presenting as Pancoast syndrome and bone destruction of ribs. A 46-year-old Chinese woman was admitted due to pain in chest, right neck and arm for four months and hoarseness for one week. The pre-admission diagnosis via chest CT was pulmonary carcinoma. The subsequent bronchoalveolar lavage fluid analysis and bronchoscopic biopsy were negative for malignant cells, except chronic inflammation. Imaging-guided percutaneous biopsies were carried out after admission and the final pathological diagnosis was pulmonary mucormycosis. Although the patient was started on oral posaconazole of 400 mg bid, the disease condition continued to deteriorate. She finally died of respiratory failure.     

Pulmonary mucormycosis with embolism: two autopsied cases of acute myeloid leukemia

Mucormycosis is an increasingly important cause of morbidity and mortality for patients with hematological malignancies. The diagnosis of mucormycosis usually requires mycological evidence through tissue biopsy or autopsy because the signs and symptoms are nonspecific and there are currently no biomarkers to identify the disease. We herein present two autopsied cases of acute myeloid leukemia with prolonged neutropenia who developed invasive mucormycosis accompanied by pulmonary artery embolism. Our cases were featured by unexplained fever and rapidly progressive dyspnea. Computed tomography scan detected nodular lesions or nonspecific consolidations in the lungs. Cultures, cytological study, and serum fungal markers consistently gave negative results. Autopsy revealed embolism of the pulmonary artery which consisted of fibrin clots by filamentous fungi. Genomic DNA was extracted from the paraffin-embedded clots and was applied to polymerase chain reaction amplification, leading to the diagnosis of infection by Rhizopus microsporus. We should carefully search for life-threatening pulmonary embolism when patients with hematological malignancies develop pulmonary mucormycosis.     

肺癌侵犯肺动脉干19例

目的 观察肺癌侵犯肺动脉干的病理解剖,ＣＴ征象,为手术提供参考。方法 对１９例肺癌侵犯肺动脉干的切除标本进行病理解剖。与术前ＣＴ比较。结果 肿瘤包绕肺动脉全周８例,部分包绕１１例。肿瘤侵犯以管壁外膜和中膜为主,尤其局限在中膜的外弹性膜周围,内膜未受侵。管壁炎性浸润,水肿或肉芽及结缔组织增生突出。ＣＴ表现管周脂肪异常１０例,肿块与肺动脉干肾密相贴或包绕１６例,压迫及移位２例,低肺血流灌注３例。     

肝素抑制低氧性肺动脉高压大鼠肺血管内皮生长因子1的表达

目的： 观察肝素抑制大鼠低氧性肺动脉高压过程中,血管内皮生长因子1（VEGF-1）在肺小动脉血管内皮细胞和肺组织中的表达变化。方法：成年雄性SD大鼠24只,随机分为对照组（A组）、低氧4周组（B组）和低氧+肝素4周组（C组）,每组8只。测各组大鼠平均肺动脉压（mPAP）、右室肥大指数（RVHI）和血管形态学指标;HE染色观察肺动脉血管形态学改变,免疫组化法检测肺动脉血管内皮细胞VEGF-1蛋白表达;RT-PCR检测肺组织VEGF-1基因表达。结果：mPAP、RVHI、肺小动脉重塑指标、肺小动脉血管内皮细胞VEGF-1蛋白表达和肺组织VEGF-1基因表达水平在C组高于A组,低于B组。相关分析表明,VEGF-1蛋白表达与肺血管重塑呈正相关（r=0.974,P<0.01）。VEGF-1mRNA与VEGF-1蛋白呈正相关（VEGF120 mRNA,r=0.919, P<0.01;VEGF164 mRNA,r=0.896, P<0.01）。结论：肝素可能在转录和翻译水平抑制VEGF-1的表达,从而抑制大鼠低氧性肺动脉高压的形成。     

Mycotic pseudoaneurysm of a pulmonary artery branch caused by Cladosporium

We report the case of a 53‐year‐old male with a history of acute myelogenous leukemia, who suffered the rupturing of a right‐sided pulmonary artery pseudoaneurysm combined with pneumonia. He underwent a right‐sided lower lobectomy. The resected lung tissue demonstrated a mycotic pseudoaneurysm of a pulmonary artery branch together with a filamentous fungal infection. Pseudoaneurysms are caused by the breaching of all layers of a blood vessel wall. The extravasated blood is trapped by the surrounding extravascular tissue or clots. Cladosporium was detected during a polymerase chain reaction‐based analysis followed by DNA sequencing of formalin‐fixed paraffin‐embedded lung tissue samples. Although previous cases of pulmonary artery pseudoaneurysms caused by fungal infections, e.g., Candida or Aspergillus sp., have been reported, to the best of our knowledge this is the first case to involve cladosporiosis.     

Fulminant myocarditis and pulmonary cavity lesion induced by disseminated mucormycosis in a chronic hemodialysis patient: Report of an autopsied case

Mucormycosis is a rare fungal infection occurring in the immunocompromised host. It is difficult to diagnose, and its cardiac involvement is extremely rare. Here, we report a 64‐year‐old Japanese man with a 5‐year history of hemodialysis with disseminated mucormycosis causing fulminant myocarditis and pulmonary necrosis under glucocorticoid use. Two months before, he had received an implantable cardioverter defibrillator and started to take amiodarone for recurrent ventricular arrhythmias due to hypertensive cardiomyopathy. He developed amiodarone‐induced interstitial pneumonia and then received glucocorticoid therapy. Although the interstitial pneumonia partially improved, a lung cavitary lesion developed in the upper right lobe. Antibiotics had no effect, and serologic tests, blood and sputum cultures and bronchoalveolar lavage fluid were all negative for infectious pathogens. Eventually, he died of fulminant myocarditis. Autopsy revealed disseminated mucormycosis with vascular invasion and fungal thrombi, hemorrhage and infarction in lung (cavity lesion), heart (severe myocarditis), brain, thyroid and subcutaneous tissue around the implantable cardioverter defibrillator. The lung cavitary lesion was the only clinical finding suggestive of mucormycosis before autopsy. When an immunocompromised patient shows a progressive lung cavity lesion, the possibility of mucormycosis should be considered so that a broad‐spectrum antifungal agent can be empirically administered in a timely fashion.     

Hematogenously disseminated skin disease caused by Mucor velutinosus in a patient with acute myeloid leukemia

We report here a case of disseminated skin infection caused by Mucor velutinosus, a recently described new species. We believe this to be the first published report of a clinical case of mucormycosis due to M. velutinosus, as well as a rare case of dissemination from a deep site to skin.     

The Ross procedure--valvular pathology of synchronous pulmonary autograft and homograft insufficiency

We report the morphological changes in a patient with combined pulmonary autograft and homograft dysfunction after 9 years of implantation. The case highlights long-term adaptive changes in the autograft to withstand systemic blood pressure with formation of exuberant pannus rich in elastic tissue. In the homograft, maturation of pannus had produced shortening of the 3 cusps with resultant incompetence.     

Sarcoma of the pulmonary artery: report of four cases with electron microscopic and immunohistochemical examinations,and review of the literature

Herein we report the clinicopathological features of four cases of pulmonary artery sarcoma that appeared at our institution during a period of 30 years. The patients, 2 males and 2 females, were 50–62 years old. Tumour was found in the pulmonary trunk and right pulmonary artery in all cases, in the pulmonary valve and left pulmonary artery in three of the four cases, and in the right ventricular outflow tract in one case. There was direct extension or metastases to the lungs in two cases, the heart in one case, mediastinum or lymph nodes in two cases and the pleura in one case. Ultrastructural examination in one case revealed cells with features of smooth muscle cells and myofibroblasts. Immunohistochemical examination of three cases gave the following results: vimentin and smooth muscle specific actin was positive in all three cases, desmin in one case and cytokeratin in one case. No positivity was found for Factor VIII. This and other studies indicate that histologically most pulmonary artery sarcomas are leiomyosarcomas or undifferentiated spindle cell sarcomas. Immunohistochemical and ultrastructural examinations favour an origin from myofibroblasts, probably derived from multipotent (undifferentiated) cells in the wall of the vessel. Most lesions show extensive intrathoracic growth although they rarely metastasize outside the thoracic cavity. They have a poor prognosis although some cases are currently being diagnosed during life.     

改变肺血流量对兔油酸肺水肿的影响

本实验在保持兔胸膜腔完整条件下造成一侧肺血流阻断，另一侧肺血流增加的模型。在此模型上观察改变肺动脉血流量对油酸肺损伤，肺水肿的影响。结果表明，增加一侧肺动脉血流量显著加重油酸肺损伤水肿程度（Ｐ＜０．０５）；阻断肺动脉血流显著减轻渍酸肺水肿程度（Ｐ＜０．０５），但阻断肺动脉血流不能制止油酸肺损伤的发生。肺内白细胞扣押结果表明，油酸中毒早期（１５分钟），白细胞在肺内扣押最多，增加肺动脉血流，显著增加白     

Mucormycosis caused by unusual mucormycetes, non-Rhizopus, -Mucor, and -Lichtheimia species

Rhizopus, Mucor, and Lichtheimia (formerly Absidia) species are the most common members of the order Mucorales that cause mucormycosis, accounting for 70 to 80% of all cases. In contrast, Cunninghamella, Apophysomyces, Saksenaea, Rhizomucor, Cokeromyces, Actinomucor, and Syncephalastrum species individually are responsible for fewer than 1 to 5% of reported cases of mucormycosis. In this review, we provide an overview of the epidemiology, clinical manifestations, diagnosis of, treatment of, and prognosis for unusual Mucormycetes infections (non-Rhizopus, -Mucor, and -Lichtheimia species). The infections caused by these less frequent members of the order Mucorales frequently differ in their epidemiology, geographic distribution, and disease manifestations. Cunninghamella bertholletiae and Rhizomucor pusillus affect primarily immunocompromised hosts, mostly resulting from spore inhalation, causing pulmonary and disseminated infections with high mortality rates. R. pusillus infections are nosocomial or health care related in a large proportion of cases. While Apophysomyces elegans and Saksenaea vasiformis are occasionally responsible for infections in immunocompromised individuals, most cases are encountered in immunocompetent individuals as a result of trauma, leading to soft tissue infections with relatively low mortality rates. Increased knowledge of the epidemiology and clinical presentations of these unusual Mucormycetes infections may improve early diagnosis and treatment.