Domenico Attinà

Pulmonary artery intimal sarcoma. Problems in the differential diagnosis

PurposePulmonary 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 methodsBetween 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.ResultsCT 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.ConclusionsThe 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.RiassuntoObiettivoI sarcomi dell’arteria polmonare (SAP) sono rari tumori maligni che originano dallo strato intimale dell’arteria polmonare, insorgono nell’età media e hanno una prognosi sfavorevole. Per impostare l’approccio terapeutico adeguato è opportuno sospettare la patologia tumorale qualora si presentino peculiari manifestazioni radiologiche e cliniche, al fine di porre la diagnosi differenziale con il quadro di embolia polmonare acuta piuttosto che di ipertensione polmonare cronica tromboembolica, con le quali tale patologia neoplastica è più comunemente confusa.Materiali e metodiTra il 2008 ed il 2012 sono giunti alla nostra osservazione 4 pazienti adulti, con quadro clinico non specifico che al termine dell’iter diagnostico sono risultati affetti da SAP. Tutti i pazienti, per il sospetto iniziale di embolia polmonare, hanno eseguito una radiografia del torace, una scintigrafia polmonare perfusionale, un’ecocardiografia trans-esofagea, e una angio-tomografia computerizzata (TC) torace. In seguito alle peculiari caratteristiche TC associate alla mancata risposta alla terapia anticoagulante, nel sospetto clinico di SAP, è stata effettuata una tomografia ad emissione di positroni (PET)-TC ed in un caso anche una risonanza magnetica (RM) toracica. In seguito tutti i pazienti sono stati sottoposti a tromboendoarteriectomia con indagine istologica del pezzo operatorio che ha confermato il sospetto clinico-radiologico di SAP.RisultatiLa TC è la metodica che ha permesso di effettuare il primo step nella diagnosi differenziale tra SAP ed embolia polmonare. Le caratteristiche suggestive per SAP includevano il particolare difetto di riempimento occupante l’intero lume del tronco polmonare con aumento di diametro del vaso coinvolto e il contrast enhancement disomogeneo e ritardato alla angio-TC, ancora più evidente in fase venosa. La PET-TC è stata impiegata per confermare il sospetto di SAP e distinguere tra SAP ed embolia polmonare sulla base dell’ipercaptazione del radiofarmaco della componente neoplastica. La RM è stata utilizzata in un caso con PET-TC dubbia per una migliore caratterizzazione tissutale della lesione e per meglio differenziare la componente trombotica da quella neoplastica.ConclusioniIl radiologo è in genere il primo a potere porre il sospetto di SAP in pazienti con grave dispnea e difetto di riempimento nell’arteria polmonare non responsivi alla terapia anticoagulante. La combinazione TC e PET-TC si è rivelata di estrema utilità nella valutazione dei pazienti con sospetto SAP. La diagnosi precoce mediante imaging integrato rimane ancora al giorno d’oggi la via principale da perseguire per ottenere miglioramenti in termini prognostici.

Densitometric CT evaluation of acute and chronic thromboembolic filling defects of the pulmonary arteries before and after contrast injection.

PurposeThe aim of this study was to assess the baseline computed tomography (CT) attenuation of acute and chronic pulmonary thromboemboli, their contrast enhancement (CE), correlation with haematocrit (Ht) levels and the presence of hypertrophic bronchial arteries.Materials and methodsFrom January 2006 to October 2009, we measured the baseline and postcontrast attenuation values of acute pulmonary thrombi emboli on CT angiograms of 86 patients with acute pulmonary embolism (PE) and those of chronic thrombi in 29 patients with pulmonary hypertension of various origins. The attenuation of acute thrombi was correlated with Ht and CE of chronic thrombi with the presence of hypertrophic bronchial arteries.ResultsAcute emboli had a mean baseline attenuation of 54.9 Hounsfield units (HU) and showed no CE. The attenuation of acute thrombi was not dependent on Ht. Chronic thrombi had a mean baseline attenuation of 33.8 HU, and 54% of thrombi showed significant CE. In 57% of cases, a collateral circulation had developed. In 76.5% of cases, CE and hypertrophic bronchial arteries coexisted (p=0.026). Neither thrombotic CE nor bronchial artery hypertrophy predominated in any one of the diseases associated with chronic thrombosis.ConclusionsBefore contrast administration, acute emboli coare prevalently hyperattenuating and therefore more conspicuous. Only chronic thrombi exhibit CE, and CE is significantly associated with the development of collateral circulation, which may be involved in the process of thrombotic recanalisation.RiassuntoObiettivoScopo del presente lavoro è stato valutare la densità basale dei trombi polmonari acuti e cronici, il loro contrast enhancement (CE), la correlazione con i valori di ematocrito (Ht) e la presenza di arterie bronchiali ipertrofiche (IB).Materiali e metodiDa gennaio 2006 a ottobre 2009 sono stati calcolati i valori densitometrici basali e postcontrastografici dei trombi acuti ricavati dalle angiotomografie computerizzate (TC) di 86 pazienti con embolia polmonare acuta e dei trombi cronici di 29 pazienti con ipertensione polmonare di diversa eziologia. La densità dei trombi acuti è stata correlata all’Ht e il CE dei trombi cronici alla presenza di IB.RisultatiGli emboli acuti hanno mostrato densità basale media di 54,9 UH e non hanno mostrato CE. La densità dei trombi acuti non dipendeva dall’Ht. I trombi cronici hanno mostrato densità basale media di 33,8 UH; nel 54% dei casi mostravano significativo CE. Il 57% dei casi sviluppa circoli collaterali. Nel 76,5% dei casi CE e IB erano concomitanti (p=0,026). Né il CE trombotico né l’ipertrofia delle arterie bronchiali prevalevano in una delle patologie associate a trombosi cronica.ConclusioniIn fase pre-contrastografica i trombi polmonari acuti sono prevalentemente iperdensi e quindi meglio identificabili. Solo i trombi cronici mostrano CE; il CE si associa significativamente allo sviluppo di circoli collaterali che potrebbero essere implicati nel processo di ricanalizzazione trombotica.

Evolution of the subsolid pulmonary nodule: a retrospective study in patients with different neoplastic diseases in a nonscreening clinical context

PurposeThe aims of this study were to evaluate the high-resolution computed tomography (HRCT) features of subsolid pulmonary nodules (SSN) detected in cancer patients to differentiate between benign and malignant lesions, to assess their evolution during the follow-up, and to determine which neoplastic diseases are most frequently associated with the growth in size and/or density of SSN.Materials and methodsNinety-seven patients with a total of 146 subsolid nodules [140 pure ground-glass opacities (pGGOs) and six mixed ground-glass opacities (mGGOs)] were retrospectively recruited. Two chest radiologists independently reviewed the HRCT features of the nodules (location, shape, size, density) and the patients’ clinical characteristics (sex, age, smoking and cancer history). Mean duration of follow-up was more than 2 years.ResultsDuring follow-up, 58% of SSN remained stable, 10% disappeared. An increase in size and/or density was seen in 32% of SSN, and in particular in partly solid (mGGOs), large (≥10 mm) and irregular nodules. The majority of small-size (<5 mm) rounded SSN remained stable. SSN growth was more frequent in patients with advanced age and a history of smoking, and occurred even after a long period of stability (39% of pGGOs “changed” over 3 years). The neoplastic diseases most frequently associated with SSN growth were cancers of lung (34%), breast (15%), colon (15%) and bladder (10%).ConclusionsThe observation of a sample of cancer patients has shown that SSN may frequently grow in size and/or density in these patients, especially if associated powith cancers of lung, breast, colon and bladder. As the majority of SSN showed a very slow development time, a follow-up period longer than 3 years is warranted even in cancer patients.RiassuntoObiettivoScopo del presente lavoro è valutare le caratteristiche alla tomogracfia computerizzata ad alta risoluzione (HRCT) dei noduli polmonari subsolidi (NSS) in pazienti oncologici, per differenziare NSS benigni e maligni, valutare la loro evoluzione durante il follow-up e determinare quali patologie neoplastiche più frequentemente si associano a crescita dimensionale e/o densitometrica delle lesioni.Materiali e metodiSono stati reclutati in maniera retrospettiva 97 pazienti, per un totale di 146 NSS (140 ground glass “puri” — pGGO, e 6 ground glass “misti” — mGGO). Le caratteristiche HRCT dei noduli (localizzazione, forma, dimensioni, densità) e quelle cliniche dei pazienti (sesso, età, abitudine al fumo e anamnesi oncologica) sono state valutate indipendentemente da due radiologi toracici. La durata media del follow-up è stata superiore ai 2 anni. Risultati. Durante il follow-up il 58% dei NSS è rimasto invariato, il 10% è scomparso, mentre in circa un terzo dei casi (32%) si è verificato un aumento di dimensioni e/o densità, specialmente in caso di noduli parzialmente solidi (mGGO), di grandi dimensioni (≥10 mm) e irregolari. La maggior parte dei NSS rotondeggianti e di piccole dimensioni (<5 mm) è rimasto invariato. La crescita è stata più frequente in soggetti di età avanzata e con storia di fumo e si è verificata anche dopo un lungo periodo di stabilità (39% di pGGO “modificati” oltre i 3 anni). Le patologie oncologiche più frequentemente associate a crescita dei NSS sono risultate i tumori del polmone (34%), della mammella (15%), del colon (15%) e della vescica (10%).ConclusioniL’osservazione di un campione di pazienti oncologici ha dimostrato come i NSS frequentemente possono crescere di dimensioni e/o densità in questi pazienti, soprattutto se associati a tumori del polmone, della mammella, del colon e della vescica. La maggioranza di essi ha mostrato un tempo di sviluppo estremamente lento, perciò, un follow-up superiore ai 3 anni appare giustificato anche nei pazienti oncologici.

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.

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.

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.

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.

Thromboembolic Disease and Pulmonary Hypertension

Venous thromboembolism (VTE) is a common medical condition in the elderly that includes both deep vein thrombosis and pulmonary embolism (PE).