Gallium imaging in pulmonary artery sarcoma mimicking pulmonary embolism: case report

Primary pulmonary artery sarcoma provides perfusion-ventilation images, as well as arteriographic studies, that can suggest pulmonary embolism. The awareness of atypical correlation among the studies for pulmonary embolism can lead to an early suspicion of pulmonary artery tumor. Imaging with 67Ga-citrate may facilitate earlier diagnosis.     

An intimal sarcoma of the pulmonary artery. An immunohistochemical study

Pulmonary artery intimal sarcomas tend to be presented with symptoms of pulmonary thromboembolism and grow regionally, with little capacity to metastasize. They probably originate from subendothelial cells, that become myofibroblasts. Knowledge of it is important to establish a presurgery diagnosis, with the possibility of a total resection, the only useful treatment until now. We report a case of a pulmonary artery primary sarcoma, in a 73 year old woman, admitted with hemoptysis and pleuritic chest pain, who died ten days after. Autopsy revealed an intraluminal mass at the pulmonary artery trunk, without regional nor distance involvement. Microscopic study showed a pleomorphic tumor with spindle and epithelioid cells, positive for actin, desmin and vimentin. All these data support the diagnosis of primary intimal sarcoma of the pulmonary artery. We want to emphasize the myogenic differentiation of the tumor, uncommon in previously reported cases.     

Rare etiology of multiple pulmonary lacunae

Sarcoma of the common pulmonary artery are rare malignant tumors which can mimic pulmonary embolism. In the case presented here, the inaugural signs were particularly misleading: multiple pulmonary lacunae on computed tomography. The unusual aspect and asymmetric localizations at pulmonary angiography then suggested the doubtful nature of the embolism etiology. Magnetic resonance imaging findings suggested the diagnosis of sarcoma of the pulmonary artery. Certain diagnosis was obtained at pathology examination of the surgical specimen after thoracotomy. A malignant fibrous histiocytoma was identified. Curative resection was not possible and chemotherapy was performed. Unusual parenchymal lesions were then evidenced on the radiography. Better and better magnetic resonance imaging criteria are described in the literature and help distinguish between thromboembolism and sarcoma of the pulmonary artery. Follow-up of the clinical course is thus improved. It is nevertheless necessary to evaluate intravascular extension to determine whether curative surgery is possible.     

Chondrosarcoma of the pulmonary artery

Primary sarcomas of the large vessels are rare. They are observed most commonly in the pulmonary artery. Up to now 28 cases of primary sarcoma of the pulmonary artery have been described in the literature. The clinical features of this disease which presents considerable diagnostic difficulties are dominated by signs of cor pulmonale. In the present paper a primary chondrosarcoma of the pulmonary artery is presented.     

Etest for routine clinical antimicrobial susceptibility testing of rapid-growing mycobacteria isolates

Tumors depend on their blood supply for growth. The blood supply to metastatic neoplasia of lung is usually from the pulmonary circulation or both the pulmonary and systemic circulation. The antineoplastic effect of pulmonary artery occlusion was investigated in a rat model of methylcholanthrene-induced metastatic pulmonary sarcoma. Left pulmonary artery ligation was performed on day 7 after tumor inoculation, and animals were sacrificed on day 14. The tumor burden of the left lung decreased 44% when compared with the control group. The survival of non-tumor-bearing rats undergoing left pulmonary artery ligation for 24 hours followed by right pneumonectomy after 2 weeks was also studied. No significant lung damage after a period of left pulmonary artery ligation was seen, as evidenced by both survival after contralateral right pneumonectomy and histology. Balloon occlusion of pulmonary artery, together with regional chemotherapy for patients with lung metastases, may warrant investigation.     

Hepatopulmonary syndrome: the paradigm of liver-induced hypoxaemia

The current chapter deals with the concept, clinical manifestations and diagnostic tools of the hepatopulmonary syndrome (HPS) and highlights its most salient pathophysiological, mechanistic and therapeutic aspects. Defined as a clinical triad, including a chronic liver disorder, pulmonary gas exchange abnormalities and generalized pulmonary vascular dilatations, in the absence of intrinsic cardiopulmonary disease, this entity is currently growing in interest with both clinicians and surgeons. The combination of arterial hypoxaemia, high cardiac output with normal or low pulmonary artery pressure, and finger clubbing in a patient with advanced liver disease should strongly suggest the diagnosis of HPS. Its potential high prevalence together with failure of numerous therapeutic approaches depicts a life-threatening unique clinical condition that may dramatically benefit with an elective indication of liver transplantation (LT). A better orchestration of the concepts of the pathophysiology of this lung-liver interplay may foster our knowledge and improve the clinical management and indications of LT.     

Isolated lung perfusion with doxorubicin prolongs survival in a rodent model of pulmonary metastases

BACKGROUND: We developed a rodent model of unilateral pulmonary metastases to evaluate long-term survival after isolated lung perfusion with doxorubicin. METHODS: In the model development study, on day 0, two groups of F344 rats (n = 15) underwent transient right pulmonary artery occlusion for either 5 or 10 minutes at the time of intravenous injection of methylcholantrene-induced sarcoma cells. On day 14, all animals were sacrificed and lung nodules counted. In the survival study, on day 0, 21 rats received intravenous injection of sarcoma cells with concomitant 10-minute right pulmonary artery occlusion. On day 7, eight rats underwent left isolated lung perfusion with doxorubicin (6.4 mg/kg); five rats underwent perfusion with buffered Hespan; six untreated rats were studied as controls. RESULTS: Ten of fifteen animals (67%) in the model study with 5-minute pulmonary artery occlusion had right-sided tumor nodules. Ten-minute occlusion resulted in a tumor-free right lung in all animals. In the survival study, all animals in the Hespan and control groups died of massive tumor replacement of the left lung, with median survival times of 20 and 18 days, respectively. The median survival time of 36 days for the animals undergoing isolated lung perfusion with doxorubicin was significantly longer (p < 0.00001). The left lung of two of the doxorubicin perfused rats was tumor-free at 6 weeks. CONCLUSIONS: Isolated lung perfusion with doxorubicin results in a durable response and prolongs survival in the treatment of experimental sarcoma pulmonary metastases.     

Pulmonary hypertrophic osteoarthropathy and clubbing of fingers in patients with lung cancer

We examined clinical characteristics of patients with primary lung cancer associated with clubbing of the fingers or pulmonary hypertrophic osteoarthropathy. Clubbing was observed in 12.5% of patients with lung cancer. Squamous cell carcinoma was frequently associated with clubbing. Clubbing was found in all clinical stages. PaO2 and PaCO2 were normal in patients with lung cancer, which suggests that neither hypoxemia nor hypercapnia caused the clubbing in these patients. Pulmonary hypertrophic osteoarthropathy was found in three patients with lung cancer (two men and one woman, mean age 49 years). The incidence was 2.9% among lung cancer patients with clubbing, 0.22% in all lung cancer patients, and was apparently lower than those in reports from outside Japan. One of these patients has stage IIIA squamous cell carcinoma, one had stage IV large cell carcinoma, and one had stage IV adenocarcinoma. In all cases bone scans were useful for diagnosis and for following the clinical course.     

Diagnosis of pulmonary Kaposi's sarcoma by detection of human herpes virus 8 in bronchoalveolar lavage

Human herpes virus 8 (HHV8) DNA has recently been detected in sarcoma tissue of patients with Kaposi's sarcoma. HHV8 DNA could also be found in bronchoalveolar lavage (BAL) fluid of patients with tracheobronchial Kaposi's sarcoma. To determine the specificity, sensitivity and predictive values of HHV8 DNA detection in the BAL for the diagnosis of pulmonary Kaposi's sarcoma, 100 consecutive BAL were prospectively analyzed for the presence of HHV8 DNA using a nested PCR assay. In addition, 19 BAL samples of 14 AIDS patients with cutaneous or visceral Kaposi's sarcoma were retrospectively investigated. The prospective group consisted of 79 BAL performed in immunocompromised and of 21 BAL in nonimmunocompromised patients. Four patients of the prospectively analyzed group undergoing six BAL showed tracheobronchial Kaposi's sarcoma at five bronchoscopies. All of the five BAL samples performed in these patients with endoscopically visible Kaposi's sarcoma were positive for HHV8 DNA. Following chemotherapy and antiretroviral treatment tracheobronchial Kaposi's sarcoma was no longer detectable at a subsequent bronchoscopy and HHV8 DNA in BAL became negative in one patient. One BAL sample of a HIV-positive patient with no evidence of Kaposi's sarcoma was HHV8 DNA-positive. The sensitivity, specificity, positive and negative predictive values of HHV8 detection for the diagnosis of tracheobronchial Kaposi's sarcoma were 100%, 98.9%, 83.3%, and 100%, respectively. Twelve of 19 BAL samples of the retrospective group were HHV8 DNA-positive. In this group, 10 patients undergoing a total of 14 BAL suffered from pulmonary Kaposi's sarcoma. HHV8 DNA was documented in 10 of these 14 BAL samples. In three BAL of this group HHV8 DNA was positive, but pulmonary Kaposi's sarcoma was diagnosed at a later stage. In conclusion, the detection of HHV8 DNA in BAL is restricted to patients with Kaposi's sarcoma and is highly sensitive and specific for pulmonary involvement of Kaposi's sarcoma.     

Hippocratic fingers in Beh?et's disease

Digital clubbing has been a well recognised feature of several distinct clinical conditions, including respiratory, cardiac, and gastrointestinal diseases since the Hippocratic era. However, clubbing associated with Beh?et's disease has not been reported before. We describe a case of clubbing in a young man with Beh?et's disease, in the absence of any other apparent aetiology and propose that clubbing might be an accompanying feature of Beh?et's disease.     

Is clubbing a feature of the anti-phospholipid antibody syndrome?

A patient with the anti-phospholipid antibody syndrome and digital clubbing is described. No recognized cause for the clubbing was found. It is suggested that platelet aggregation and microthrombi formation as a result of anti-phospholipid antibody may be involved in the pathogenesis of the digital clubbing. This may be a new feature of the anti-phospholipid antibody.     

Primary digital clubbing associated with palmoplantar keratoderma

The association of hereditary palmoplantar keratoderma and idiopathic clubbing of the digits in the same patient is uncommon. The differential diagnosis includes the Bureau-Barrière-Thomas syndrome, primary pachydermoperiostosis, Fischer's and Volavsek's syndromes, and palmoplantar keratoderma V?rner. A 30-year-old woman with palmoplantar keratoderma and clubbing of the fingers since the age of 13 years is presented.     

Gastroesophageal reflux with protein-losing enteropathy and finger clubbing

Finger clubbing, protein-losing enteropathy, and iron deficiency were documented in three children with severe gastroesophageal reflux. One patient had Sandifer syndrome and the other two had the rumination syndrome. In each case, surgical repair of the gastroesophageal reflux resulted in immediate clearing of signs of the Sandifer syndrome, gastroesophageal reflux, and anemia and the return of serum protein levels to normal. There was definite regression of the finger clubbing during the ensuing year. It is suggested that finger clubbing, protein-losing enteropathy, Sandifer syndrome, and rumination be viewed as parts of an extended syndrome of unusual presentations of gastroesophageal reflux.     

Variation in the size and distensibility of the pulmonary arteries in d-transposition of the great arteries

The relative size of the main pulmonary artery was determined from cineangiograms of 117 patients with d-transposition of the great arteries by calculating the ration between the diameters of the main pulmonary artery and aorta. The pulmonary artery was largest in patients with ventricular septal defect or patent ductus arteriosus, or both, because of increased pulmonary arterial pressure and flow. In patients with an intact ventricular septum or with left ventricular outflow tract obstruction, or both, the main pulmonary artery was approximately the size of the aorta. Two cases of d-transposition and gross dilatation of the main pulmonary artery and hypoplastic first branch pulmonary arteries are presented. In these cases the ratio between the diameters of the main pulmonary artery and aorta was greater than in any of the other 117 cases studied...     

Pulmonary arterial reconstruction with autologous pulmonary arterial tissue for pulmonary artery sling

One-year and two-month-old boy with pulmonary artery sling underwent pulmonary artery reconstruction with autologous pulmonary arterial tissue. Surgical repair consisted of the transection of the left pulmonary artery at its origin with trimming a button hole and the creation of an oval hole with the same size to the left pulmonary button. An autologous pulmonary patch and the left pulmonary button were exchanged and sutured to the former partner's place. He recovered uneventfully in post-operative course. Postoperative CT revealed a well developed left pulmonary artery from 5 mm to 10 mm in diameter without any distortion or stenosis. This exchange method could allow the natural growth of the anastomotic places without narrowing or distortion.     

Observation of relationship between function and structure of experimental pulmonary artery hypertension in rats and inhibitory effect of garlicin and other herbal medicines

The paper deals with the treatment of the pulmonary artery hypertention in rats caused by monocrotaline with garlicin, liqustrazine and berbamine and studies the relationship between the function and structure of pulmonary artery and pulmonary artery hypertension before and after use of hypotensive agents. The results show that there is a close relationship between the function and structure of pulmonary artery and pulmonary hypertension, and the inhibitory effect of garlicin is the strongest among the three herbal medicines. The pathogenesis is also studied.     

The study on noninvasive evaluation of pulmonary artery pressure by the method of pulmonary artery flow spectrum in chronic pulmonary disease

OBJECTIVE: Pulmonary artery flow spectrum of patients with chronic pulmonary disease were studied in order to find excellent Doppler Indexes reflected pulmonary artery pressure, and establish multiple regression equation for estimating pulmonary artery pressure more accurately. METHODS: During catheterization, 54 patients with chronic pulmonary disease were examined by echocardiography. Fourteen Doppler indexes of spectrum were compared with the pulmonary artery pressure by catheter simultaneously, and multiple regression equations were taken. RESULTS: There were significant correlation between Doppler indexes PEP/AcT, AcVm, VI/(QTxVmax), RVET/QT and pulmonary artery pressure. Two multiple regression equations were deduced: PAPs (kPa) = [9.4 x PEP/AcT-1.4 x AcVm-126.1 x VI/(QT x Vmax) + 75] divided by 7.5 and PAPm (kPa) = [7.5 x PEP/AcT-1.2 x AcVm- 87.7 x VI/(QT X Vmax) + 48] divided by 7.5. CONCLUSIONS: Above two regression equations could be used to noninvasively evaluate pulmonary artery pressure in patients with chronic pulmonary disease.     

Clinical implications and possible association of malposition of the branch pulmonary arteries with DiGeorge syndrome and microdeletion of chromosomal region 22q11

We describe a series of 10 patients with malposition of the branch pulmonary arteries (4 patients with crossing [crossed pulmonary arteries] and 6 patients without crossing), 2 of whom had a short main pulmonary artery segment that resulted in iatrogenic right pulmonary artery stenosis after pulmonary artery band placement. DiGeorge syndrome was seen in 5 patients and 4 had microscopic deletion of chromosomal region 22q11.     

Consequences of a small decrease of air temperature from thermal equilibrium on thermoregulation in sleeping neonates

PURPOSE: To evaluate macromolecular contrast-enhanced MR-angiography for the detection of experimentally induced pulmonary artery embolism and to determine the size of the smallest detectable embolised vessel. METHODS: Pulmonary artery embolism was artificially induced in eight isolated perfused sheep lungs by injecting room air into the main pulmonary artery. The pulmonary vascular system enhanced by macromolecular gadolinium-DTPA-polylysine was imaged with a flow-sensitive gradient-echo technique. RESULTS: Pulmonary artery embolism was demonstrated in all eight lungs by an obvious cut-off phenomenon in the contrast-enhanced arteries proximal to the pulmonary air emboli. The smallest detectable embolised artery measured 1.6 mm in diameter and was located in the 6th generation of the pulmonary vascular system. CONCLUSIONS: In this experimental study MR angiography enhanced by a macromolecular contrast agent allowed noninvasive diagnosis of pulmonary artery embolism.     

Device to limit inflation of a pulmonary artery catheter balloon

OBJECTIVE: The most serious complication seen with pulmonary artery catheters is rupture of the pulmonary artery. The effectiveness of an external safety balloon added to the pulmonary artery balloon inflation port was tested. DESIGN: The external balloon is designed to inflate and absorb excess volume from the inflation syringe after the internal balloon contacts the vessel wall. When the catheter tip is in a small pulmonary artery, expansion of the external balloon indicates that the catheter tip is in a noncompliant or small vessel. SETTING: The external balloon was tested in a bench simulation. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The pulmonary artery balloon was slowly inflated inside 2.6-, 3.0-, 4.7-, 8.6-, and 11.6-mm internal diameter polyvinyl chloride tubes, with and without the external safety device in place. Without the external balloon, the average balloon pressure was 1647 +/- 145 (SD) mm Hg in the 2.6-mm vessel. With the external balloon in use, the maximum pulmonary artery balloon pressure was 473 +/- 7.2 mm Hg in the 2.6-mm vessel. CONCLUSIONS: The external balloon can limit balloon pressures within the pulmonary artery and identify when excessive volumes are being forced into the pulmonary artery balloon.