Sarcoidosis-associated pulmonary hypertension due to pulmonary arteries stenosis - a case report.

BACKGROUND: Sarcoidosis-associated pulmonary hypertension (SAPH) is listed in Group 5 of the clinical classification of pulmonary hypertension, due to its complex and multifactorial pathophysiology. The most common cause of SAPH development is advanced lung fibrosis with the associated destruction of the vascular bed, and/or alveolar hypoxia. However, a substantial proportion of SAPH patients (up to 30%) do not have significant fibrosis on chest imaging. In such cases, the development of pulmonary hypertension may be due to the lesions directly affecting the pulmonary vasculature, such as granulomatous angiitis, pulmonary veno-occlusive disease, chronic thromboembolism or external compression of vessels by enlarged lymph nodes. Based on the case of a 69-year-old female who developed SAPH due to pulmonary arteries stenosis, diagnostic difficulties and therapeutic management are discussed. CASE PRESENTATION: The patient, non-smoking female, diagnosed with stage II sarcoidosis twelve years earlier, presented with progressive dyspnoea on exertion, dry cough, minor haemoptysis and increasing oedema of the lower limbs. Computed tomography pulmonary angiography (CTPA) showed complete occlusion of the right upper lobe artery and narrowing of the left lower lobe artery, with post-stenotic dilatation of the arteries of the basal segments. The vascular pathology was caused by adjacent, enlarged lymph nodes with calcifications and fibrotic tissue surrounding the vessels. Pulmonary artery thrombi were not found. The patient was treated with systemic corticosteroid therapy and subsequently with balloon pulmonary angioplasty. Partial improvement in clinical status and hemodynamic parameters has been achieved. CONCLUSIONS: An appropriate screening strategy is required for early detection of pulmonary hypertension in sarcoidosis patients. Once SAPH diagnosis is confirmed, it is crucial to determine the appropriate phenotype of pulmonary hypertension and provide the most effective treatment plan. Although determining SAPH phenotype is challenging, one should remember about the possibility of pulmonary arteries occlusion.

Predictive value of chest HRCT for survival in idiopathic pulmonary arterial hypertension.

BACKGROUND: Little attention has been paid to chest high resolution computed tomography (HRCT) findings in idiopathic pulmonary arterial hypertension (IPAH) patients so far, while a couple of small studies suggested that presence of centrilobular ground-glass opacifications (GGO) on lung scans could have a significant negative prognostic value. Therefore, the aims of the present study were: to assess frequency and clinical significance of GGO in IPAH, and to verify if it carries an add-on prognostic value in reference to multidimensional risk assessment tool recommended by the 2015 European pulmonary hypertension guidelines. METHODS: Chest HRCT scans of 110 IPAH patients were retrospectively analysed. Patients were divided into three groups: with panlobular (p)GGO, centrilobular (c)GGO, and normal lung pattern. Association of different GGO patterns with demographic, functional, haemodynamic, and biochemical parameters was tested. Survival analysis was also performed. RESULTS: GGO were found in 46% of the IPAH patients: pGGO in 24% and cGGO in 22%. Independent predictors of pGGO were: positive history of haemoptysis, higher number of low-risk factors, and lower cardiac output. Independent predictors of cGGO were: positive history of haemoptysis, younger age, higher right atrial pressure, and higher mixed venous blood oxygen saturation. CGGO had a negative prognostic value for outcome in a 2-year perspective. This effect was not seen in the longer term, probably due to short survival of cGGO patients. CONCLUSIONS: Lung HRCT carries a significant independent prognostic information in IPAH, and in patients with cGGO present on the scans an early referral to lung transplantation centres should be considered.

Morphology and morphometry of pulmonary veins and the left atrium in multi-slice computed tomography.

PURPOSE: Pulmonary veins are involved in pathogenesis and treatment of atrial fibrillation and structures at risk during thoracic surgeries. There is lack of data regarding pulmonary vein morphology and morphometry in normal population. METHODS: The study was conducted using 135 chest computed tomography studies with intra-venous iodine contrast injection. The study population contained 86 females and 49 males, mean age was 60. 13 had atrial fibrillation.The studies were analyzed using radiological workstation. RESULTS: Mean dimensions of the left atrium: transverse 52 mm, coronal 49 mm, and sagittal 35 mm. The mean volume of the left atrium was 93 cm3. The mean volume of the left atrium in patients with atrial fibrillation was 176 cm3. The sagittal dimension and the volume of the left atrium were correlated with age, r = 0.43 and r = 0.42, respectively. Surface area of the left inferior pulmonary vein ostium was 136 mm2, significantly less than the surface area of other ostia of pulmonary veins. The mean distance between two pulmonary veins was 5.42 mm on the right and 4.02 mm on the left side. 13 types of pulmonary veins outflow patterns were described on the right side and 5 types on the left side. 66.7% of right pulmonary veins and 82% of the left pulmonary veins emptied into the left atrium with two venous trunks on each side (the typical pattern). CONCLUSIONS: Morphological features of pulmonary veins and morphometry of the left atrium and pulmonary veins are important for clinical purposes and are in accordance with previous papers.

Centrilobular nodules in high resolution computed tomography of the lung in IPAH patients - preliminary data concerning clinico-radiological correlates.

INTRODUCTION: Inhomogeneity of lung attenuation pattern is observed in high resolution chest computed tomography (HRCT) in some IPAH patients despite lack of interstitial lung disease. Such radiological changes are described either as ill-defined centrilobular nodules (CN) or as focal ground glass opacities (FGGO). There is no consensus in the literature, whether they indicate the distinct type of IPAH, or pulmonary venoocclusive disease (PVOD) with subtle radiological changes. Thus the aim of the present pilot study was to assess the frequency and clinical significance of inhomogenic lung attenuation pattern in IPAH. MATERIAL AND METHODS: 52 IPAH patients (38 females, 14 males, mean age 41 years ± 15 years), entered the study. All available chest CT scans were reviewed retrospectively by the experienced radiologist, not aware about the clinical data of the patients. RESULTS: CN were found in 10 patients (19%), FGGO - in 12 patients (23%). No lymphadenopathy or interlobular septal thickening suggestive of PVOD were found. The significant differences between CN and the remaining patients included: lower mean age - 31 and 43.5 years, (p = 0.02), lack of persistent foramen ovale (PFO) - 0% and 43% (p = 0.03), and higher mean right atrial pressure (mRAP) - 12.5 mm Hg and 7.94 mm Hg (p = 0.01). No significant survival differences were observed between the groups of CN, FGGO and the remaining patients. CONCLUSION: Centrilobular nodules in IPAH were combined with lack of PFO, higher mRAP and younger age of patients.

Low DLCO in idiopathic pulmonary arterial hypertension - clinical correlates and prognostic significance.

INTRODUCTION: Decreased diffusing capacity of the lung for carbon monoxide (DLCO) is observed in some idiopathic pulmonary arterial hypertension (IPAH) patients, but its clinical significance is uncertain. We aimed to assess clinical correlates and prognostic significance of low DLCO in IPAH patients. MATERIAL AND METHODS: In the group of 65 IPAH patients the cut off value for low DLCO was set up based on histogram as < 55% of predicted value. Demographic data, exercise capacity, lung function tests, hemodynamic parameters and survival of the patients were compared depending on DLCO value. RESULTS: Low DLCO was found in 18% of the patients, and it was associated with male sex, older age, worse functional status and exercise capacity, and higher prevalence of coronary artery disease. Low DLCO carried a 4-fold increase of death risk in 5-year perspective. CONCLUSIONS: Low DLCO was a marker of worse functional capacity and increased risk of death in studied IPAH patients.

Non-high risk PE in the patients with acute or exacerbated respiratory disease: the value of the algorithm based on D-dimer evaluation and Revised Geneva Score.

INTRODUCTION: The diagnostic algorithm of non-high risk pulmonary embolism (PE) is based on probability scoring systems and plasma D-dimer (DD) assessment. The aim of the present study was to investigate the efficacy of Revised Geneva Scoring (RGS) and DD testing for the excluding of non-high risk PE, in the patients admitted to the hospital due to acute respiratory diseases. MATERIAL AND METHODS: The consecutive patients, above 18 years of age, referred to the department of lung diseases, entered the study. The exclusion criteria were: the pregnancy and the suspicion of high risk PE. Plasma DD was measured with quick ELISA test, VIDAS D-dimer New, bioMerieux, France. Multislice computed tomography angiography was performed in all of the patients. RESULTS: 153 patients, median age 65 (19-88) years entered the study. The probability of PE was: low - in 58 patients (38%), intermediate - in 90 (59%), high - in 5 (3%). DD < 500 ng/ml was found in 12% of patients with low and intermediate probability of PE. PE was recognized in 10 out of 153 patients (7%). None of the patients with DD < 500 ng/ml was diagnosed with PE (NPV 100%). Median DD value was significantly higher in PE patients comparing to non-PE (4500 ng/ml and 1356 ng/ml respectively, p = 0.006). CONCLUSION: In the group of the patients with acute respiratory symptoms, low or intermediate clinical probability scoring combined with normal DD had a high NPV in excluding PE. Nevertheless, such approach was not very effective, as the increased DD was noted in 88% of the examined population.

[Disseminated pulmonary actinomycosis - an unusual presentation]. / Rzadka manifestacja plucna promienicy w postaci zmian rozsianych.

Actinomycosis is a rare, chronic infectious disease caused by anaerobic Gram-positive bacteria Actinomyces spp. They induces suppurative inflammation in tissues. They live as commensals in the oropharynx, interstitial tract and genital mucosa, causing almost exclusively endogenic infections. Beacause variable clinical course, its chronicity, quite often actinomycosis mimics rather neoplasmatic disease than infection. We present the case of 56-year old male with unusual pulmonary actinomycosis manifestation as bilateral disseminated lung nodules with systemic symptoms, after initial antitubercular treatment. Diagnosis definitely was made of histologic evaluation of lung specimen from surgical biopsy. After 7-month antibacterial treatment we have achived clinical and radiological improvement.

Accuracy of FDG PET/CT in the evaluation of solitary pulmonary lesions - own experience.

INTRODUCTION: In recent years, positron emission tomography (PET) has been increasingly applied in the diagnosis of neoplastic lung diseases. In contrast to conventional imaging studies, PET-CT enables the visualisation of not only the morphology of the suspicious lesion, but also its metabolism. The aim of the present study was to investigate the role of PET-CT in the initial assessment of patients with indeterminate solitary pulmonary lesions. MATERIAL AND METHODS: The study was conducted on a group of 82 patients with indeterminate lung nodule diagnosed at the National Institute of Tuberculosis and Lung Diseases in the period from January 2008 to May 2011. CT and PET-CT were performed in all of the patients. Histological or cytological examination of the biopsy specimens obtained from bronchoscopy, mediastinoscopy and intraoperatively were the reference tests. RESULTS: Malignancy was documented in 40 patients (48.8%). Histopathological analysis of all tumours revealed 12 cases of squamous cell carcinoma, 18 cases of adenocarcinoma and 1 case of carcinoid, whereas in 9 patients the diagnosis of "non-small cell cancer not otherwise specified" was made. All lesions except one were of solid character on chest CT. SUV(max) values exceeding 2.5 were found in 38 cancer patients (true positives, TP). The mean value of SUV(max) was 9.1 (1-26.8). Forty-two lesions were documented as benign (51.2%). SUV(max) values equal to or less than 2.5 were found in 37 patients (true negatives, TN). The mean value of SUV(max) in this group was 1.9 (0.5-8.6). The diagnostic value of PET-CT SUV(max) exceeding 2.5 in the prediction of neoplastic origin of solitary pulmonary lesions was: sensitivity - 95% (95% CI 84-99%), specificity - 88% (95% CI 75-95%) and accuracy - 91.5% (95% CI 83-96%). Positive predictive value (PPV) was 88.4% (95% CI 76-95%), and negative predictive value (NPV) was 94.8% (95% CI 83-99%). False negative results concerned two patients, with final diagnosis of carcinoid and adenocarcinoma; false positive results were obtained in 5 patients with various inflammatory lesions. CONCLUSIONS: In the present study, PET-CT appeared to have high sensitivity (95%), but lower specificity (88%) for predicting the malignant character of solitary pulmonary lesions. Overall diagnostic value of PET-CT SUV(max) > 2.5 was high - PPV was 88.4%, NPV was 94.8%. In the authors' opinion, the PET-CT value may increase when clinical data as well as other radiological documentation (with retrospective assessment) are taken into consideration.

Methotrexate as a single agent for treating pulmonary sarcoidosis: a single centre real-life prospective study.

INTRODUCTION: The first-line therapy in chronic sarcoidosis, according to WASOG/ATS/ERS recommendations, is GCS. This therapy is associated with significant adverse effects and finally does not alter the natural history of the disease. The objective of our study was to evaluate the efficacy and safety of monotherapy with MTX, as an alternative to GCS, in progressive pulmonary sarcoidosis. MATERIAL AND METHODS: An open prospective real-life, single-centre trial was performed on 50 patients with biopsy proven sarcoidosis, 28M and 22F, mean age 45.55 ± 8.9 years. The average duration of disease before MTX therapy was 12.34 ± 20.49 years, GCS therapy in the past was applied in 41 patients. All patients received MTX (10 mg or 15 mg weekly) between 2004 and 2013 because of chronic progressive pulmonary sarcoidosis. Therapy was planned for 24 months. Patients underwent regular clinical evaluation, pulmonary function assessment, exercise ability testing (6MWT), and chest radiography for therapy effectiveness every six months and side effects monitoring every 4-6 weeks. Forty-nine patients were included for statistical analysis of treatment efficacy. They were retrospectively allocated to "MTX responder" group if an improvement of 10% of FEV1, FVC, TLC, or 15% of DLCO from the initial value was documented for at least one parameter or "non-responders" if the patient did not meet the above-mentioned criteria. RESULTS: Duration of treatment ranged from 6 to 24 months, mean time 60.75 ± 34.1 weeks. For the whole cohort significant improvement after MTX therapy was observed for minimal SaO2 (%) (p = 0.043) and for decrease of DSaO2 (%) (p = 0.048) in six-minute walk test. The results were significantly better for patients treated with 15 mg than for those treated with 10 mg weekly and for those who obtained a greater total amount of MTX during therapy. Significant difference of DLCO%pred was observed after six months of MTX therapy between groups treated 15 mg vs 10 mg weekly (73.27 ± 12.7% vs. 63.15 ± 16.4%, p = 0.03). Twenty-five patients (55%) met the criteria of "MTX responders" group. Patients who responded well to treatment had significantly lower TLC and FVC initial values comparing to "MTX non-responders". After treatment the only significant difference in PFT between groups was noted for DLCO%pred. Eleven patients (22%) stopped the treatment due to adverse events of MTX, mild hepatic abnormalities were observed in ten patients (20%), and concomitant infection was found in four patients. There were no patients with a fatal outcome. CONCLUSIONS: MTX as a single agent in the treatment of sarcoidosis has proved to be a safe and effective steroid alternative. Selected patients with chronic pulmonary sarcoidosis experience definite PFT improvements after MTX treatment. There is need to search for predictors of MTX treatment effectiveness.

[Staging of non-small cell lung cancer using CT and integrated PET-CT]. / Ocena zaawansowania niedrobnokomórkowego raka pluca metodatomografii komputerowej i pozytonowej tomografii emisyjnejskojarzonej z tomografia komputerowa

INTRODUCTION: Lung cancer is the leading cause of death from cancer in developed countries. Radiological imaging methods are the basic methods in early diagnosis of this disease. TNM classification is a very important tool for optimal treatment in non-small lung cancer (NSCLC). Conventional radiological techniques allow the evaluation of the stage on the basis of anatomical changes only, while PET-CT provides information about the biochemical processes that may precede anatomical changes. The aim of this study was to compare the accuracy and sensitivity of CT and PET-CT in the staging of NSCLC. MATERIAL AND METHODS: The study was conducted on a group of 99 patients with NSCLC diagnosed at the Institute of Tuberculosis and Lung Diseases in the period from January 2008 to May 2010. CT and PET-CT were performed in all patients. Histological or cytological examination of the material obtained from biopsy, bronchoscopy, mediastinoscopy, and intraoperatively was the reference test. TNM classification was performed independently after CT and PET-CT. RESULTS AND CONCLUSIONS: It has been shown that PET-CT is a more accurate and sensitive method than CT in the staging process in NSCLC. PET-CT allowed the correct classification of the T, N, M, and total TNM in, respectively, 97%, 95%, 99%, and 89% of cases, while for CT it was, respectively, 95%, 84%, 84%, and 68% (p = 0.0002).

[Severe hypoxemic respiratory insufficiency in a patient with hepato-pulmonary syndrome coexisting with interstitial lung disease of unknown etiology]. / Ciezka hipoksemiczna niewydolnosc oddechowa u chorego z zespolem watrobowo-plucnym i choroba sródmiazszowa pluc o nieznanej etiologii.

The coexistence of the interstitial lung disease and respiratory failure is rarely associated with extrapulmonary pathology. In patients with liver cirrhosis, hypoxemia may develop in the course of hepato-pulmonary syndrome (HPS), but radiological pathology seen in the course of HPS is of vascular origin, and thus typically not classified as interstitial lung disease. We present a patient with severe hypoxemic respiratory insufficiency in whom hepato-pulmonary syndrome coexisted with interstitial lung disease of unknown etiology. The mechanisms of hypoxemia in the course of hepatic diseases and reasons of possible coincidence of lung and hepatic pathology are discussed.

[Virtual bronchoscopy and bronchofiberoscopy--a comparison of diagnostic value in assessment of centrally localized lung tumor]. / Porównanie wartosci diagnostycznej bronchoskopii wirtualneji bronchofiberoskopii w diagnostyce centralnego guza pluca.

INTRODUCTION: Virtual bronchoscopy (VB) is a new, noninvasive diagnostic technique which allows visualizing trachea and bronchi. Virtual images are created on the basis of data derived from helical CT scans using special protocol. A reconstructed, virtual image of the bronchial tree is very similar to that seen during conventional bronchofiberoscopy (FOB). The aim of the study was to compare VB images of the bronchi with those coming from FOB and evaluate diagnostic value of VB in centrally localized lung tumor. MATERIAL AND METHODS: The studied group consisted of 40 patients with suspicion of centrally localized lung cancer. Primary diagnosis was based on the chest X-ray. Each patient underwent CT and bronchofiberoscopy and after those routine procedures the VB analysis was performed. The results of the FOB were not known for radiologist performing VB. In both used methods, FOB and VB, the evaluation and comparison of the features of tumor presence, bronchial stenosis and widening of the carina were performed. In all 40 patients lung cancer was confirmed. The diagnosis was established by histopathologic examination of the tissue biopsy: 32 patients (80%)--non small cell lung cancer, 2 patients (5%)--small cell lung cancer, 5 patients (12.5%)--squamous cell carcinoma, 1 patient (2.5%)--carcinoid. RESULTS: Diagnostic value of VB in assessment of the for presence of the tumor in bronchus was: sensitivity 79.5%, specificity 95.5%, for bronchial stenosis: sensitivity 58.6%, specificity 98.1% and for widening of carina: sensitivity 60.7%, specificity 97.7%. CONCLUSION: The results indicate that virtual bronchoscopy is highly sensitive and specific diagnostic method, a clinically valuable for the evaluation of lung tumor with a central location.

[The bleeding into the emphysematosus bulla imitating lung tumor]. / Krwawienie do pecherza rozedmowego imitujace guz pluca.

Bleeding into the lung parenchyma is a rare phenomenon that usually occurs as a result of chest trauma, other causes are anticoagulant therapy, and infections. The following case presents a patient admitted to the hospital due to haemoptysis, which was a symptom of bleeding into the emphysematosus bulla caused by anticoagulation therapy. The decisive diagnostic examination was chest magnetic resonance. This imaging method allows the precise differentiation of tissues. Using modern imaging techniques can often dispense with invasive diagnostic methods.

Sarcoidosis in coexistence with chronic granulomatous disease.

Granulomas formations are present in many lung diseases. Coexistence of one or more of these diseases is very rare. Diagnostics of such cases always poses a challenge. We present a case of coexistence of chronic granulomatous disease (CGD) and sarcoidosis.

[Pulmonary neuroendocrine tumors. The spectrum of histologic subtypes and current concept on diagnosis and treatment]. / Rozrosty neuroendokrynne pluc. Histologiczne spektrum podtypów, aktualne pglady dotyczace rozpoznawania i leczenia.

Neuroendocrine tumors of the lung represent a broad spectrum of morphologic types that share specific morphologic, immunohistochemical, ultrastructural, and molecular characteristics. The classification of neuroendocrine lung tumors has changed over the last decades and currently four categories are distinguished: typical carcinoid tumor, atypical carcinoid tumor, large cell neuroendocrine carcinoma and small cell carcinoma. Neuroendocrine tumors of the lung comprise approximately 20% of all primary lung cancers. Among them, the most frequent is small cell carcinoma (13-17%). Because of differences in clinical behavior, therapy, and prognosis, a reliable histological diagnosis, as well as clinical and pathological staging system are essential for an appropriate medical proceedings. The most effective treatment of bronchial carcinoids and large cell neuroendocrine carcinoma in an early stage is complete surgical resection, whereas chemotherapy remains the primary treatment for small cell carcinoma. All carcinoids are malignant tumors with the potential to metastasize. The majority of patients with pulmonary carcinoid have an excellent survival, even if they present with lymph node metastases. Large cell neuroendocrine and small cell carcinoma progress rapidly and are generally widespread at the moment of diagnosis. Their overall prognosis is poor. Increased knowledge about pulmonary neuroendocrine tumors biology and the genetic characteristics, imply that carcinoid tumors appear to have a different etiology and pathogenesis than large cell neuroendocrine and small cell carcinoma. In practice, it could be easiest to conceptualize this group of pulmonary tumors as a spectrum of malignancy ranging from the low grade typical carcinoid to the highly malignant large cell neuroendocrine and small cell carcinoma. Typical carcinoid tumors associated with a fairly benign behavior should be classified as low-grade neuroendocrine tumor/carcinoma (G1) and atypical carcinoid tumors as intermediate-grade tumor/carcinoma (G2). Whereas, large cell neuroendocrine and small cell carcinoma should be grouped together under the designation of high-grade neuroendocrine tumor/carcinoma (G3).

[Abnormalities in high-resolution computed tomography of the lungs in patients with idiopathic pulmonary arterial hypertension -- correlation with hemodynamic parameters and prognostic significance]. / [Zmiany w tomografii komputerowej pluc o wysokiej rozdzielczosci u chroych na idiopatyczne tetnicze nadcisnienie plucne--powiazanie z parametrami hemodynamicznymi i znaczenie rokownicze.

INTRODUCTION: The risk stratification in idiopathic pulmonary arterial hypertension (IPAH) patients is currently based on haemodynamic and functional parameters as well as serum biomarker concentrations. Until now the importance of changes appearing in high-resolution computed tomography (HRCT) of the lungs of patients with IPAH has not been investigated. MATERIAL AND METHODS: Lung HRCT scans were analysed retrospectively in 48 IPAH patients (patients): 37 women, 11 men, mean age 41 +/- 15 years. RESULTS: Focal ground-glass opacifications (FGG) were found in 12 patients (25%), and centrilobular nodules (CN) were found in 8 patients (17%). In the remaining 58% of patients HRCT revealed no changes (N). Significantly lower stroke volume was found in the CN group (41.0 +/- 8.5 ml) compared to 60.8 +/- 15.1 ml in the FGG group and 58.1 +/- 18.0 ml in the N group (p = 0.03). Right atrial pressure was significantly higher in the CN group (12.2 +/- 4.86 mm Hg) than in the FGG group (6.9 +/- 3.9 mm Hg) and the N group (7.6 +/- 5.3 mm Hg), p = 0.047. The presence of nodules was combined with considerably increased risk of death, both in univariate analysis (HR 5.35, 95% CI: 1.16-24.7, p = 0.03) and in multivariate analysis (HR 6.98, 95% CI: 1.41-34.59, p = 0.02). Ground-glass opacifications correlated neither with haemodynamic nor functional indexes, and were of no prognostic significance. CONCLUSIONS: The presence of centrilobular nodules in lung HRCT scans of IPAH patients was combined with more severe haemodynamic compromise and was an independent negative prognostic indicator.

Primary mediastinal neoplasms: a report of 1,005 cases from a single institution.

BACKGROUND: This study was an epidemiological analysis of all primary mediastinal neoplasms (PMNs) diagnosed between 2000 and 2016 at the National Tuberculosis and Lung Diseases Research Institute, Poland. METHODS: All patients with any mediastinal abnormality were included in the analysis. The patients' age and gender were obtained from the institutional database. RESULTS: From a cohort of 5,108 patients, 3,691 primary mediastinal lesions were found, including 1,005 (19%) PMNs: lymphomas (533, 53% of PMNs), thymomas (215, 21%), neurogenic tumors (NTs) (100, 10%), germ cell tumors (GCTs) (62, 6%), soft tissue tumors (STTs) (47, 5%) and thymic carcinomas/thymic neuroendocrine tumors (TCs/TNETs) (37 in total, 4%). The most frequent lymphomas were classical Hodgkin lymphomas [256] and primary mediastinal large B-cell lymphomas [163]. Type AB [73] predominated in thymomas and squamous cell carcinomas [9] and carcinoids [10] in TCs/TNETs. NTs encompassed mainly schwannomas [49], ganglioneuromas [21] and neurofibromas [10]. The most frequent STTs were hemangiomas [13] and lymphangiomas [11]. Lymphomas, thymomas and NT were more often in women, TCs/TNETs in men (P<0.001). Lymphomas predominated between the 2nd and 4th decade of life, NTs under the 3rd decade and thymic epithelial tumors between the 6th and 8th decade (P<0.001). There was no correlation between the subtypes of thymomas and the patients' gender (P=0.389) but it was found between histology and patients' age: in patients <30 years of age type B2 and B3 thymomas and >70 years of age AB type and micronodular thymomas with lymphoid stroma (P<0.001) predominated. In the group of GCTs half of them were malignant and these were noted exclusively in men. No correlation between subtypes of NTs or TCs/TNETs and patients' age and gender was found (P>0.05). CONCLUSIONS: PMNs are rare conditions thus awareness of basic epidemiology may be very helpful for final diagnosis.

[Interstitial pneumonitis in a patient treated with amiodarone -- case report]. / Srodmiazszowe zapalenie pluc u chorego leczonego amiodaronem -- opis przypadku.

Amiodarone, antiarrhythmic drug of III class is used in patients with supraventricular and ventricular arrhythmias, often with coexisting congestive heart failure. Side effects of amiodarone treatment are observed in approximately 75% of patients. Most dangerous are the symptoms of amiodarone pulmonary toxicity occuring in 2-17% of patients. We present a patient with COPD, in whom interstitial pneumonitis with radiologic features of organizing pneumonia developed after one year of amiodaron treatment due to supraventricular and ventricular arrhythmias. The drug was stopped and steroids were introduced due to marked respiratory insufficiency. Regression of pulmonary symptoms and improvement of ventilatory parameters were observed after 3 months of treatment. Pathogenesis, diagnostic procedures and current methods of treatment of this jatrogenic disease are discussed.

Pulmonary veno-occlusive disease: pathogenesis, risk factors, clinical features and diagnostic algorithm - state of the art.

Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary haemangiomatosis (PCH) are rare disorders, with the estimated prevalence of less than 1 case per million inhabitants. The vascular pathology in PVOD/PCH involves pre-septal and septal veins, alveolar capillaries and small pulmonary arteries. According to the ERS/ESC classification of pulmonary hypertension (PH) from 2015, PVOD/PCH have been included in the subgroup 1' of pulmonary arterial hypertension (PAH). Recent data indicate, however, the possibility of PVOD/PCH pathology in the patients diagnosed in the group 1. The problem may concern PAH associated with scleroderma, drug- induced PAH, PAH due to HIV infection and up to 10% of patients with idiopathic PAH (IPAH). Recently, bi-allelic EIF2AK4 mutations were found in the cases with heritable form of PVOD/PCH and in about 9% of sporadic cases. Moreover, an association between occupational exposure to organic solvents and PVOD/PCH was proved. The present review is an attempt to summarise the current data on pathogenesis, risk factors, clinical features and diagnostic algorithm for PVOD/PCH.

Intrapericardial cisplatin combined with oral colchicine resulted in long term control of malignant pericardial effusion in the course of metastatic renal cancer.

Background Neoplastic pericardial effusion (NPE) represents a common cause of morbidity and mortality in patient with cancer. NPE presents frequently as cardiac tamponade, requiring urgent pericardiocentesis or pericardiotomy, with subsequent pericardial fluid drainage. Despite high effectiveness of such procedures, the recurrence of effusion is noted in 30- 60% of patients. Intrapericardial therapy with cisplatin was found to be effective in NPE due to lung and breast cancer. Its role in cardiac tamponade due to renal cancer is unknown. Case presentation We presented 82-year-old man with renal cancer who was admitted to the Intensive Care Unit because of threatening pericardial tamponade due to NPE . Urgent subxiphoid pericardiotomy was performed with subsequent evacuation of 1000ml of bloody fluid. On the inner surface of the pericardium several pink nodules were found. Histological examination revealed carcinoma clarocellulare. In view of the persistent high drainage of the pericardium, intrapericardial cisplatin therapy was performed. The first day after surgery colchicine 0.5 mg/day/po was also introduced. No side effects of this treatment were observed. The patient died 12- month later due to cancer progression and cachexia. No recurrence of pericardial effusion was observed. Conclusion This is the first case study demonstrating long-term efficacy and safety of intrapericardial cisplatin combined with oral colchicine in NPE due to metastatic renal cell carcinoma.