Cardiac Magnetic Resonance to Predict Cardiac Mass Malignancy: The CMR Mass Score.

BACKGROUND: Multimodality imaging is currently suggested for the noninvasive diagnosis of cardiac masses. The identification of cardiac masses' malignant nature is essential to guide proper treatment. We aimed to develop a cardiac magnetic resonance (CMR)-derived model including mass localization, morphology, and tissue characterization to predict malignancy (with histology as gold standard), to compare its accuracy versus the diagnostic echocardiographic mass score, and to evaluate its prognostic ability. METHODS: Observational cohort study of 167 consecutive patients undergoing comprehensive echocardiogram and CMR within 1-month time interval for suspected cardiac mass. A definitive diagnosis was achieved by histological examination or, in the case of cardiac thrombi, by histology or radiological resolution after adequate anticoagulation treatment. Logistic regression was performed to assess CMR-derived independent predictors of malignancy, which were included in a predictive model to derive the CMR mass score. Kaplan-Meier curves and Cox regression were used to investigate the prognostic ability of predictors. RESULTS: In CMR, mass morphological features (non-left localization, sessile, polylobate, inhomogeneity, infiltration, and pericardial effusion) and mass tissue characterization features (first-pass perfusion and heterogeneity enhancement) were independent predictors of malignancy. The CMR mass score (range, 0-8 and cutoff, ≥5), including sessile appearance, polylobate shape, infiltration, pericardial effusion, first-pass contrast perfusion, and heterogeneity enhancement, showed excellent accuracy in predicting malignancy (areas under the curve, 0.976 [95% CI, 0.96-0.99]), significantly higher than diagnostic echocardiographic mass score (areas under the curve, 0.932; P=0.040). The agreement between the diagnostic echocardiographic mass and CMR mass scores was good (κ=0.66). A CMR mass score of ≥5 predicted a higher risk of all-cause death (P<0.001; hazard ratio, 5.70) at follow-up. CONCLUSIONS: A CMR-derived model, including mass morphology and tissue characterization, showed excellent accuracy, superior to echocardiography, in predicting cardiac masses malignancy, with prognostic implications.

[Management of acute type B aortic dissection: treatment and follow-up]. / Gestione della dissezione aortica acuta di tipo B: trattamento e follow-up.

Aortic dissection is a life-threatening condition caused by a tear in the tunica intima which creates a false lumen into the aortic wall. Acute type B aortic dissection (TBAD) is defined by the presence of the entry tear in the aorta distal to the left subclavian artery, without ascending aorta and arch involvement, and accounts for 25-40% of all aortic dissections. Optimal medical therapy (OMT), focused on blood pressure and heart rate control, remains the gold standard treatment, especially for patients with uncomplicated TBAD, while complicated dissections require surgical therapy. Recent studies have shown that a considerable number of patients treated only with OMT develop late aorta-related complications that increase morbidity and mortality, as well as the need for surgical intervention. During the last decades, emerging evidence indicates that thoracic endovascular aortic repair (TEVAR) is safe and effective in the treatment of TBAD, both complicated and uncomplicated, with improved long-term survival outcomes and aortic remodeling in combination with OMT compared to OMT alone. However, in cases of acute uncomplicated TBAD the optimal timing for TEVAR is not entirely clarified and there is lack of long-term evidence. Therefore, the role of pre-emptive TEVAR for these patients is still uncertain and the management of acute uncomplicated TBAD remains challenging.

Update of penetrance estimates in Birt-Hogg-Dubé syndrome.

BACKGROUND: Birt-Hogg-Dubé (BHD) syndrome is a rare genetic syndrome caused by pathogenic or likely pathogenic germline variants in the FLCN gene. Patients with BHD syndrome have an increased risk of fibrofolliculomas, pulmonary cysts, pneumothorax and renal cell carcinoma. There is debate regarding whether colonic polyps should be added to the criteria. Previous risk estimates have mostly been based on small clinical case series. METHODS: A comprehensive review was conducted to identify studies that had recruited families carrying pathogenic or likely pathogenic variants in FLCN. Pedigree data were requested from these studies and pooled. Segregation analysis was used to estimate the cumulative risk of each manifestation for carriers of FLCN pathogenic variants. RESULTS: Our final dataset contained 204 families that were informative for at least one manifestation of BHD (67 families informative for skin manifestations, 63 for lung, 88 for renal carcinoma and 29 for polyps). By age 70 years, male carriers of the FLCN variant have an estimated 19% (95% CI 12% to 31%) risk of renal tumours, 87% (95% CI 80% to 92%) of lung involvement and 87% (95% CI 78% to 93%) of skin lesions, while female carriers had an estimated 21% (95% CI 13% to 32%) risk of renal tumours, 82% (95% CI 73% to 88%) of lung involvement and 78% (95% CI 67% to 85%) of skin lesions. The cumulative risk of colonic polyps by age 70 years old was 21% (95% CI 8% to 45%) for male carriers and 32% (95% CI 16% to 53%) for female carriers. CONCLUSIONS: These updated penetrance estimates, based on a large number of families, are important for the genetic counselling and clinical management of BHD syndrome.

Endovascular Solutions for Aortic Arch Diseases: Total and Hybrid.

Although the endovascular approach is the therapeutic option of choice for thoracic and abdominal aortic diseases, open surgery is still the treatment of choice for aortic arch diseases. While open surgical repair remains the gold standard treatment for complete aortic arch replacement, it continues to be burdened by high mortality and neurologic complications, especially for patients who require redo surgery. Therefore, in the era of endovascular surgery, it is not surprising that hybrid operating rooms, new technologies, and new approaches are strongly challenging open surgery. Less-invasive endovascular procedures, when used to treat aortic arch diseases, when feasible and indicated, have clear advantages over open surgery, primarily because there is no need for cardiopulmonary bypass, hypothermic circulatory arrest, or cerebral protection. Moreover, patients who have already been treated for acute type A aortic dissection continue to have a considerable risk for future aortic reintervention, which is associated with increased risk for short- and long-term mortality. In light of these advantages, it is clear how selected high-risk patients with aortic arch disease could benefit from the endovascular approach. However, the hemodynamic and anatomic characteristics of the aortic arch make the endovascular approach in this region challenging. In fact, uncorrected stent-graft placement can have fatal consequences for the patient and increase the risk of endoleaks and stroke. To minimize these potential risks, precise and accurate preoperative planning to achieve optimal stent-graft dimensions and implantation is essential together with careful patient selection. Endovascular options for the treatment of aortic arch disease include both hybrid procedures and total endovascular solutions. This manuscript provides an overview of the current strategies for endovascular aortic arch treatment, including the most recent available series on this topic. In addition, a literature search offers insight into the current state of the art.

Hybrid transcatheter left ventricular reconstruction for the treatment of ischemic cardiomyopathy.

Left ventricular (LV) enlargement is a mechanical adaptation to accommodate LV systolic inefficiency following an acute damage or a progressive functional deterioration, which fails to correct the decline of stroke volume in the long term, leading to progressive heart failure (HF). Surgical ventricular reconstruction (SVR) is a treatment for patients with severe ischemic HF aiming to restore LV efficiency by volume reduction and LV re-shaping. Recently, a new minimally-invasive hybrid technique for ventricular reconstruction has been developed by means of the Revivent™ system (BioVentrix Inc., San Ramon, CA, USA). The device for ventricular reconstruction consists of anchor pairs that enable plication of the anterior and free wall LV scar against the right ventricular (RV) septal scar of anteroseptal infarctions to decrease cardiac volume without ventriculotomy in a beating-heart minimally-invasive procedure, consisting of a transjugular and left thoracotomy approach. Patients with severe (Grade 4) functional mitral regurgitation (FMR) or with previous cardiac surgery procedures were excluded. Outcome of the reconstruction procedure: from 2012 until 2019, it has been applied to 203 patients, with 5 (2.5%) in-hospital deaths. LV volume reduction varied according to experience gained along years: LV end-systolic volume index decreased from baseline 43% (post-market registry) vs. 27% (CE-mark study); left ventricular ejection fraction (LVEF) increased from baseline 25% (post-market registry) vs. 16% (CE-mark study). Clinical status (NYHA class, HF questionnaire, 6-minute walking test) improved significantly compared to baseline, and re-hospitalization rate was only 13% at 6-month follow-up (60% of patients in NYHA =3). FMR grade decreased at follow-up in 63%, while it was unchanged in 37% of patients. The hybrid ventricular reconstruction (HVR) seems a promising treatment for HF patients who may benefit from LV volume reduction, with reasonable mortality and good results at follow-up. A baseline less severe clinical profile was not associated to better outcome at follow-up, which makes the procedure feasible in patients with very large ventricles and depressed ejection fraction (EF). LV reshaping has no detrimental effect on FMR, that may, on the contrary, benefit owing to less papillary muscle displacement, partial recovery of torsion dynamics and of myofibers re-orientation. A controlled study on top of optimal medical treatment is warranted to confirm its role in the management of HF patients.

The triple rule out CT in acute chest pain: a challenge for emergency radiologists?

PURPOSE: To evaluate the feasibility of triple rule out computed tomography (TRO-CT) in an emergency radiology workflow by comparing the diagnostic performance of cardiovascular and general radiologists in the interpretation of emergency TRO-CT studies in patients with acute and atypical chest pain. METHODS: Between July 2017 and December 2019, 350 adult patients underwent TRO-CT studies for the assessment of atypical chest pain. Three radiologists with different fields and years of expertise (a cardioradiologist-CR, an emergency senior radiologist-SER, and an emergency junior radiologist-JER) retrospectively and independently reviewed all TRO-CT studies, by trans-axial and multiplanar reconstruction only. Concordance rates were then calculated using as reference blinded results from a different senior cardioradiologist, who previously evaluated studies using all available analysis software. RESULTS: Concordance rate was 100% for acute aortic syndrome (AAS) and pulmonary embolism (PE). About coronary stenosis (CS) for non-obstructive (<50%), CS concordance rates were 97.98%, 90.91%, and 97.18%, respectively, for CR, SER, and JER; for obstructive CS (>50%), concordance rates were respectively 88%, 85.7%, and 71.43%. Moreover, it was globally observed a better performance in the evaluation of last half of examinations compared with the first one. CONCLUSIONS: Our study confirm the feasibility of the TRO-CT even in an Emergency Radiology department that cannot rely on a 24/7 availability of a dedicated skilled cardiovascular radiologist. The "undedicated" radiologists could exclude with good diagnostic accuracy the presence of obstructive stenosis, those with a clinical impact on patient management, without needing time-consuming software and/or reconstructions.

Diagnostic Accuracy of Cardiac Computed Tomography and 18-F Fluorodeoxyglucose Positron Emission Tomography in Cardiac Masses.

OBJECTIVES: This study sought to assess the diagnostic accuracy of cardiac computed tomography (CT) and 18F-fluorodeoxyglucose (18F-FDG) with positron emission tomography/computed tomography (PET/CT) in defining the nature of cardiac masses. BACKGROUND: The diagnostic accuracy of cardiac CT and 18F-FDG PET/CT in identifying the nature of cardiac masses has been analyzed to date only in small samples. METHODS: Of 223 patients with echocardiographically diagnosed cardiac masses, a cohort of 60 cases who underwent cardiac CT and 18F-FDG PET/CT was selected. All masses had histological confirmation, except for a minority of thrombotic formations. For each mass, 8 morphological CT signs, standardized uptake value (SUVmax, SUVmean), metabolic tumor volume, and total lesion glycolysis in 18F-FDG PET were used as diagnostic markers. RESULTS: Irregular tumor margins, pericardial effusion, invasion, solid nature, mass diameter, CT contrast uptake, and pre-contrast characteristics were strongly associated with the malignant nature of masses. The coexistence of at least 5 CT signs perfectly identified malignant masses, whereas the detection of 3 or 4 CT signs did not accurately discriminate the masses' nature. The mean SUVmax, SUVmean, metabolic tumor volume, and total lesion glycolysis values were significantly higher in malignant than in benign masses. The diagnostic accuracy of SUV, metabolic tumor volume, and total lesion glycolysis 18F-FDG PET/CT parameters was excellent in detecting malignant masses. Among patients with 3 or 4 pathological CT signs, the presence of at least 1 abnormal 18F-FDG PET/CT parameter significantly increased the identification of malignancies. CONCLUSIONS: Cardiac CT is a powerful tool to diagnose cardiac masses as the number of abnormal signs was found to correlate with the lesions' nature. Similarly, 18F-FDG PET/CT accurately identified malignant masses and contributed with additional valuable information in diagnostic uncertainties after cardiac CT. These imaging tools should be performed in specific clinical settings such as involvement of great vessels or for disease-staging purposes.

Successful multidisciplinary clinical approach and molecular characterization by whole transcriptome sequencing of a cardiac myxofibrosarcoma: A case report.

BACKGROUND: Cardiac tumors are rare and complex entities. Surgery represents the cornerstone of therapy, while the role of adjuvant treatment remains unclear and, in case of relapse or metastatic disease, the prognosis is very poor. Lack of prospective, randomized clinical trials hinders the generation of high level evidence for the optimal diagnostic workup and multimodal treatment of cardiac sarcomas. Herein, we describe the multidisciplinary clinical management and molecular characterization of a rare case of cardiac myxofibrosarcoma in an elderly woman. CASE SUMMARY: A 73-year-old woman presented signs and symptoms of acute left-sided heart failure. Imaging examination revealed a large, left atrial mass. With suspicion of a myxoma, she underwent surgery, and symptoms were promptly relieved. Histology showed a cardiac myxofibrosarcoma, a rare histotype of cardiac sarcoma. Eight months later, disease unfortunately relapsed, and after a multidisciplinary discussion, a chemotherapy with doxorubicin and then gemcitabine was started, achieving partial radiologic and complete metabolic response, which was maintained up to 2 years and is still present. This report is focused on the entire clinical path of our patient from diagnosis to follow-up, through surgery and strategies adopted at relapse. Moreover, due to their rarity, very little is known about the molecular landscape of myxofibrosarcomas. Thus, we also performed and described preliminary genome analysis of the tumor tissue to get further insight on mechanisms involved in tumor growth, and to possibly unveil new clinically actionable targets. CONCLUSION: We report a case of cardiac myxofibrosarcoma that achieved a very good prognosis due to an integrated surgical, cardiac and oncologic treatment strategy.

Bronchial artery embolization for the treatment of haemoptysis in pulmonary hypertension.

PURPOSE: To test the efficacy of bronchial artery embolization (BAE) to treat haemoptysis in pulmonary hypertension (PH). METHODS: 33 patients were treated by BAE for haemoptysis associated with PH (PH group = 21) or non-associated with PH (control group = 12). The details of procedure, outcome, and rate of relapse were compared between the two groups. Within the PH group, the comparison was operated between subjects with congenital heart disease-associated pulmonary artery hypertension (CHD-APAH subgroup = 12) and non-CHD (non-CHD-APAH subgroup = 9). RESULTS: The rate of relapse at 30 and 90-days was similar between the PH group and control group. BAE in the PH group was more challenging (median 2 arteries embolized per procedure) compared to the control group (median 1 artery embolized per procedure; p = 0.001). Bleeding arteries were more heterogeneous in the PH group, while a single right bronchial artery was the only clinical finding in 66.7% of controls (p = 0.001). Within the PH group, the CHD subgroup showed higher survival rate compared to the non-CHD-APAH group (p = 0.007). CONCLUSION: BAE is effective and safe for the treatment of haemoptysis in PH, yet more challenging than other conditions. In PH-associated haemoptysis, BAE provides higher survival rate for subjects with PH associated with CHD.

Combined pulmonary fibrosis and emphysema (CPFE): what radiologist should know.

Combined pulmonary fibrosis and emphysema is a relatively newly defined entity, which has been deeply studied in the recent years. Despite the wide numbers of papers on this topic, there are still several open questions about pathogenesis, epidemiology, natural history and prognosis. The diagnosis could be assessed only after HRCT scan as functional tests often result in an underestimation of this syndrome. What radiologists need to know about this syndrome consists in the heterogeneity of appearances: emphysema is mainly paraseptal and fibrotic pattern could be variable, including the variant of airspace enlargement with fibrosis which needs to be differentiated from honeycombing. A special attention must be paid on complications which could worsen the prognosis, such as pulmonary hypertension and lung cancer. Further studies are needed to address if the type of fibrotic pattern as well as fibrosis CT index could be considered as prognostic factors. Thus, the role of radiologists in the management of these patients is crucial as it involves diagnosis, detection of complications and could possible concerns the identification of patients at higher risk.

128-slice CT angiography of the aorta without ECG-gating: efficacy of faster gantry rotation time and iterative reconstruction in terms of image quality and radiation dose.

OBJECTIVE: To evaluate image quality and radiation dose of non ECG-gated 128-slice CT angiography of the aorta (CTAA) with fast gantry rotation time and iterative reconstruction. METHODS: Four hundred and eighty patients underwent non ECG-gated CTAA. Qualitative and quantitative image quality assessments were performed. Radiation dose was assessed and compared with the dose of patients who underwent ECG-gated CTAA (n = 126) and the dose of previous CTAA performed with another CT (n = 339). RESULTS: Image quality (aortic root-ascending portion) was average-to-excellent in more than 94% of cases, without any non-diagnostic scan. For proximal coronaries, image quality was average-to-excellent in more than 50%, with only 21.5% of non-diagnostic cases. Quantitative analysis results were also good. Mean radiation dose for thoracic CTAA was 5.6 mSv versus 20.6 mSv of ECG-gated protocol and 20.6 mSv of 16-slice CTAA scans, with an average dose reduction of 72.8% (p < 0.001). Mean radiation dose for thoracic-abdominal CTAA was 9.7 mSv, versus 20.9 mSv of 16-slice CTAA scans, with an average dose reduction of 53.6% (p < 0.001). CONCLUSIONS: Non ECG-gated 128-slice CTAA is feasible and able to provide high quality visualization of the entire aorta without significant motion artefacts, together with a considerable dose and contrast media volume reduction. KEY POINTS: • CT image quality of aortic root-ascending aorta is challenging. • Non ECG-gated scans are often limited by pulsatility artefacts. • ECG-gated examinations are usually limited by high radiation doses. • Non ECG-gated 128-slice low dose CTAA provides high quality images. • 128-slice CTAA low dose protocol could frequently replace ECG-gated CTAA.

Endovascular treatment of an aortic traumatic double rupture.

Traumatic thoracic aortic rupture is a life-threatening condition; aortic isthmus is the most common site of rupture, but in rare cases traumatic injury can localize elsewhere, such as at aortic arch or at the level of the diaphragm. In the past few years, endovascular treatment of traumatic aortic injury became a safe procedure, with lower mortality and complication, if compared with open surgery. We report a case of a 40-year-old-man admitted to emergency department after a violent car crash in which an aortic traumatic double rupture was successfully treated with two endovascular stent-grafts coverage.

Thoracic manifestation of Eisenmenger's syndrome in adult patients: a MDCT review.

Eisenmenger's syndrome (ES) is the most advanced form of pulmonary arterial hypertension (PAH) associated with congenital heart diseases (CHD). It is caused by simple or complex CHD with a large systemic-to-pulmonary shunt. When the pulmonary pressure exceeds the systemic pressure, the shunt reverses and central cyanosis appears. ES is a progressive and fatal condition, and it is accompanied by an increased risk of a range of potentially life-threatening complications. Patients with ES are both at risk for bleeding, due to damaged capillaries and high pressure, and for in situ pulmonary thrombosis, related to hyper-viscosity and slow blood flow in dilated pulmonary arteries. Moreover, the enlarged main pulmonary arteries and cardiac chambers may determine displacement or extrinsic compression on airways, pulmonary veins, coronary arteries, and other mediastinal structures. The clinical effects may be diverse, such as respiratory difficulties, localized pulmonary edema, cardiac dysfunction, or sudden death. Multidetector computed tomography (MDCT) allows to accurately assess in a single examination, pulmonary parenchyma and vessels, coronary artery origin, and heart chambers. The aim of this review was to illustrate the thoracic MDCT angiography findings and complications of adult patients with PAH-CHD and in particular of those with ES.

Lung cancer associated with cystic airspaces.

OBJECTIVE: This study aimed to define computed tomographic morphologic features of lung cancer associated with cystic airspaces, their modifications in serial computed tomographic scans, and 18F-fluoro-2-deoxy-D-glucose (FDG) positron emission tomography uptake. METHODS: Computed tomographic scans and 18F-FDG positron emission tomography in 24 patients with lung cancer (17 adenocarcinomas, 7 squamous cell carcinomas, 12 stage I and 12 stage II to IV) associated with cystic airspaces were reviewed. RESULTS: Mean diameter of airspace was initially 17.6 mm (range, 5-30 mm), and 4 morphologic patterns were recognized: solid nodule protruding externally (type I, n = 5) or internally (type II, n = 4) from the cyst wall; circumferential thickening of the cyst wall (type III, n = 8); and tissue intermixed within clusters of cysts (type IV, n = 7). With tumor growth, airspace size decreased in 9, increased in 6, and was unchanged in 9 cases. Five cases evolved from type III to type I, and 5 lesions became completely solid. 18F-fluoro-2-deoxy-D-glucose uptake was initially absent to mild in 7 and moderate to marked in 14 lesions. CONCLUSIONS: Progressive wall thickening or appearance/increase of a nodule inside or outside a cystic airspace should raise suspicion of lung cancer irrespective of FDG uptake.

Two uncommon cases of partial anomalous pulmonary vein connection.

Pulmonary venous developmental anomalies encompass a wide spectrum of congenital diseases, including partial anomalous pulmonary venous connection. We present two cases of uncommon partial anomalous pulmonary venous connection depicted well by magnetic resonance (MR) and computed tomography (CT) which could play an important role as noninvasive imaging techniques, providing high-quality and wide field of view imaging.

Saber-sheath trachea as a marker of severe airflow obstruction in chronic obstructive pulmonary disease.

PURPOSE: Saber-sheath trachea is a specific radiographic parameter for chronic obstructive pulmonary disease (COPD), which consists of marked coronal narrowing associated with sagittal widening (tracheal index <2/3-0.67). The aim of this study was to investigate the correlation between saber-sheath trachea and clinical-radiological findings in a group of patients with COPD of varying severity. MATERIALS AND METHODS: We evaluated the chest radiographs of 71 patients with COPD distributed as follows: GOLD class I, 8/71 (11.3 %); class II, 34/71 (47.9 %); class III, 16/71(22.5 %); class IV, 13/71 (18.3 %). In 52/71 (73.2 %) patients we also evaluated chest computed tomography (CT) scans. We analyzed the prevalence of saber-sheath trachea and its correlation with the Tiffenau index, GOLD stage and radiological signs of COPD. Moreover, we evaluated the sensitivity, specificity and accuracy of chest radiography as compared to CT taken as the gold standard, and the correlation between the radiographic and CT tracheal index. RESULTS: Saber-sheath trachea was found in 18/71 (25.4 %) patients, with a greater prevalence in patients with lower Tiffenau Index (p = 0.02), GOLD stages III-IV and visual severity score 3 (severe) on chest CT. Saber-sheath trachea was not found to be related to other radiological signs of COPD. The sensitivity, specificity and accuracy values of radiography were 72.2, 97.0 and 88.5 %, with perfect concordance between the radiographic and CT tracheal index (p < 0.00001). CONCLUSION: Saber-sheath trachea is linked to the functional severity of airway obstruction, but not to other radiological signs of COPD. Thus, evaluation of the trachea at chest radiography is strongly recommended.

Pulmonary veno-occlusive disease: the role of CT.

OBJECTIVE: The authors sought to evaluate the diagnostic accuracy of high-resolution computed tomography (HRCT) in the detection of pulmonary veno-occlusive disease (PVOD) in patients with pre-capillary pulmonary arterial hypertension (PAH) of unknown aetiology, and to identify the role of CT in diagnosis and therapy. MATERIALS AND METHODS: The CT scans of 96 patients were retrospectively reviewed and assessed for specific HRCT findings: ground-glass opacities, septal lines and mediastinal lymph nodal enlargement (short diameter ≥1 cm). According to the HRCT findings, patients were divided into PVOD-suspicious and not PVOD-suspicious. Subsequently, a clinical-instrumental evaluation was performed, and the response to therapy and histopathological reports were evaluated. RESULTS: Radiological evaluation based on HRCT findings revealed 29 patients as PVOD-suspicious and 67 as not PVOD-suspicious. The final diagnosis was PVOD in 22 patients and idiopathic PAH in 74 patients. The CT scan showed 95.5 % sensitivity, 89% specificity, 72.5% positive predictive value, and 98.5% negative predictive value, with a diagnostic accuracy of 90.5% in identifying patients with PVOD. CONCLUSIONS: Chest CT can be considered a screening test in the assessment of patients with PAH of unknown aetiology, and the radiologist can help the clinician to identify patients with CT findings that make PVOD highly probable.

Pulmonary artery intimal sarcoma. Problems in the differential diagnosis.

PURPOSE: Pulmonary artery sarcomas (PAS) are rare malignant tumours that originate from the intimal layer of the pulmonary artery, occur in middle age and have a poor prognosis. In planning appropriate treatment, malignant disease should be suspected whenever there are specific clinical and radiological manifestations, in order to establish the differential diagnosis with acute pulmonary embolism or chronic thromboembolic pulmonary hypertension, with which this malignancy is most commonly confused. MATERIALS AND METHODS: Between 2008 and 2012, we managed four adult patients with a nonspecific clinical presentation who, at the conclusion of the diagnostic process, were found to be affected by PAS. Because of the initial suspicion of pulmonary embolism, all patients underwent chest radiograph, lung perfusion scintigraphy, trans-oesophageal echocardiography, and computed tomography (CT) angiography of the chest. Then, because of the peculiar CT findings and lack of response to anticoagulation therapy, a clinical suspicion of PAS was considered and all patients underwent positron-emission tomography (PET)-CT, and one patient also magnetic resonance imaging (MRI) of the chest. Subsequently, all patients underwent thromboendoarterectomy with histological investigation of the surgical specimen, which confirmed the clinical and radiological suspicion of PAS. RESULTS: CT is the technique that enabled the first step in the differential diagnosis between PAS and pulmonary embolism. The CT characteristics suggestive of PAS included the particular filling defect occupying the entire lumen of the pulmonary trunk with increase in diameter of the involved vessel and patchy and delayed contrast enhancement at CT angiography, more evident in the venous phase. PET-CT was used to differentiate between PAS and pulmonary embolism on the basis of the intensity of increased radiopharmaceutical uptake. MRI was used in one case of equivocal results on PET-CT, to improve tissue characterisation of the lesions and differentiation between the thrombotic and neoplastic components. CONCLUSIONS: The radiologist is usually the first to raise a suspicion of PAS in patients with severe dyspnoea and filling defect in the pulmonary artery, unresponsive to anticoagulation therapy. Combining CT and PET-CT proved to be extremely useful in assessing patients with suspected PAS. Early diagnosis with the help of integrated imaging remains today the main direction to pursue in order to obtain improvements in prognosis.