Achieving Thoracic Oncology data collection in Europe: a precursor study in 35 Countries.

BACKGROUND: A minority of European countries have participated in international comparisons with high level data on lung cancer. However, the nature and extent of data collection across the continent is simply unknown, and without accurate data collection it is not possible to compare practice and set benchmarks to which lung cancer services can aspire. METHODS: Using an established network of lung cancer specialists in 37 European countries, a survey was distributed in December 2014. The results relate to current practice in each country at the time, early 2015. The results were compiled and then verified with co-authors over the following months. RESULTS: Thirty-five completed surveys were received which describe a range of current practice for lung cancer data collection. Thirty countries have data collection at the national level, but this is not so in Albania, Bosnia-Herzegovina, Italy, Spain and Switzerland. Data collection varied from paper records with no survival analysis, to well-established electronic databases with links to census data and survival analyses. CONCLUSION: Using a network of committed clinicians, we have gathered validated comparative data reporting an observed difference in data collection mechanisms across Europe. We have identified the need to develop a well-designed dataset, whilst acknowledging what is feasible within each country, and aspiring to collect high quality data for clinical research.

Characterization and management of elderly and very elderly patients with non-small cell lung cancer.

OBJECTIVES: Despite non-small cell lung cancer (NSCLC) high prevalence and increasing incidence, evidence specific to the elderly and very elderly is sparse. To retrospectively compare characterization and approach of NSCLC patients (pts) aged 70-79 and ≥80 years. METHODS: We performed a retrospective analysis of 297 adult NSCLC pts who registered and initiated NSCLC management in our Pulmonology Oncology Unit from January 2013 to December 2016 corresponding to 38.2% of all NSCLC patients (n = 778). Demographic data and lung cancer management were analysed. RESULTS: Pts were categorized as elderly (n = 211, 71.0%) and very elderly (n = 86, 29.0%). Very elderly pts had worse Eastern Cooperative Oncology Group performance status (P = 0.047), higher Charlson age comorbidity index (P < 0.001) and the majority had stage IV cancer (66.3%, P = 0.04). The first management option in very elderly pts was chemotherapy (CTX) (30.2%, P = 0.37) and in elderly pts was multimodal therapy (30.3%, P ≤ 0.001). Support therapy and first-line targeted (EGFR or ALK-positive) were more common in the very elderly (23.6%, P = 0.01; 17.4% P = 0.002, respectively). Curative radiation or surgery rates did not differ between groups. Reasons for premature first-line CTX stop, toxicity and hospitalization did not differ. Death rate (69.7% vs 63.5% for very elderly and elderly, respectively) and mean survival since diagnosis (11.5 vs 11.6 months for very elderly and elderly, respectively) did not differ. CONCLUSIONS: There were significant differences in pts characteristics having the very elderly more multimorbidity and advanced state of disease. First management options were significantly different with respect to multimodal, targeted and support therapy.

Treatment of non-small cell lung cancer, advanced disease, with erlotinib in 2(nd) and 3(rd) lines. Two cases report. / Tratamento do carcinoma pulmonar de não pequenas células, doença avançada, com erlotinib em segunda e terceira linhas. A propósito de dois casos clínicos.

Agents that inhibit the activity of cell membrane receptor tyrosine kinases, such as the human epidermal growth factor receptor (EGFR) have been an attractive target because EGFR is expressed by 80% of NSCLC. Erlotinib as monotherapy in the treatment of NSCLC after failure of at least one prior chemotherapy regimen, prolonged survival and improved quality of life, although modest response rate. Women, Asiens, patients with Adenocarcinoma and never smokers, were more likely than other patients to have a response to erlotinib. This is the group of patients that most commonly have an EGFR mutation. The authors describe two cases, with important control of symptoms and increased time to progression, independently o response rate (stable disease or partial response). Rev Port Pneumol 2008; XIV (Supl 3): S53-S60.

[Pulmonary embolism and difficult-to-treat asthma]. / Tromboembolismo pulmonar e asma de difícil controlo.

Asthma control is a key point in patient management. GINA's most recent report emphasises the need to investigate uncontrolled asthma, of which non-compliance with treatment, COPD, smoking, chronic sinusitis, gastroesophageal reflux disease and obesity are the usual causes. The aim of this work is to evaluate the role of pulmonary thromboembolism (PTE) in cases of difficult- -to-treat asthma. We reviewed the case reports of patients with severe persistent asthma followed in our Asthma Outpatients Clinic between 2004 and 2006. We selected the ones that maintained uncontrolled disease despite an optimal therapeutical approach and investigated the causes. In this group (n=254), 28 (11%) had severe persistent asthma and their mean age was 44 +/- SD18 years old. 86% were females. Of these, 57% (n=16) had uncontrolled disease: 35% (n=6) due to non-compliance with treatment; 29% (n=5) pulmonary thrombombolism (scintigraphic confirmation); 12% (n=2) severe rhinosinusitis; 6% (n=1) hypereosinophilic syndrome; 6% (n=1) persistent allergen exposure and 6% (n=1) are still being investigated. Patients with TPE (mean age 56 +/- SD9 years old; 80% females; 80% Caucasians) were diagnosed with asthma as adults (mean age 37 +/- SD14 years old). The mean time until the diagnosis of TPE was 18 +/- SD12 years. Predisposing factors for TPE were venous insufficiency (40%), hypertension (40%) and deficit of functional protein C and S (20%). All these patients received anticoagulant therapy (80% are still medicated). It should be noted that after the beginning of anticoagulants, 40% of the patients achieved control of their asthma and 40% have partially controlled disease. There were no hospital admissions for asthma exacerbations after the beginning of anticoagulation in this group. This study supports the inclusion of TPE in the group of comorbidities to consider while investigating uncontrolled asthma.

[Lung cancer in women: a different entity?]. / Cancro do pulmão na mulher: Uma entidade diferente?

Lung cancer is becoming a real epidemic in developed countries and one of the main causes of cancer death in women. Although it is controversial to state, it is probable that women are more susceptible to lung cancer than men. Molecular and genetic epidemiology studies are underway to prove this statement. Several biological factors, such as family history, histopathology, response to treatment and the prognosis for lung cancer in women have a bearing on the case. While these are not completely clear or consensual, there is a need for wide-ranging prospective studies which compare the differences between males and females. Until now, there has only been one area in which gender could impact on the therapeutic management of lung cancer: the role of gefitinib and erlotinib in inhibiting the epidermal growth factor receptors, since these products are clearly of more benefit to female non-smokers. Given that women have a better lung cancer prognosis, it is recommended that future research protocols include stratification on gender. Prevention of lung cancer in both women and men is a priority public health concern. A mandatory aim of this is the fight against smoking, the largest aetiological factor of lung cancer.

[Historic landmarks in the study of lung cancer]. / Marcos históricos no estudo do cancro do pulmão.

This study reviews the milestones which have been reached in the study of lung cancer, from its first early descriptions up until the end of the twentieth century. The study accompanies the birth of this new clinical entity, underlining the difficulties inherent in its diagnosis, its ever-growing increase and traces the growth of its aetiological factors, placing particular emphasis on smoking. In tandem with this, the study delves into the clinical aspects, along with new discoveries in imaging techniques and endoscopic and bioscopic techniques. It also looks at the histopathological classifications of bronchopulmonary tumours and the various staging systems which have been used over the course of time as well as the importance of mapping the disease and the different treatment weapons which have successively become available in the fight against it. The study also takes a look at the scales used in evaluating patients' physiological condition, the criteria used in evaluating response to oncostatic treatment and the role some international and national scientific societies and medical associations have played in adding to the increasing medical knowledge of lung cancer. The study clearly shows to whom we are indebted for each advance. This is a fascinating sweep of history - as is the story of all medical progress - and one we feel is important to understand, in order for us to see more clearly where we are now.

[Ehlers-Danlos syndrome - a rare cause of spontaneous pneumothorax]. / Síndroma de Ehlers-Danlos - Uma causa rara de pneumotórax espontâneo.

Ehlers-Danlos syndrome (cutis hyperelastica), is a group of connective tissue disorders characterized by abnormalities of the skin, ligaments and internal organs. It is a hereditary syndrome, usually with autossomal dominant inheritance; that primarily affects the collagen synthesis. The skin and blood vessels are extremely fragile and elastic. The skin is soft with rubber consistency and easily bruising. There are hypermobile joints with increased extensibility. We summarize the case of a sixteen year old boy with a history of joint hypermobility since childhood and splenic fracture that was diagnosed with Ehlers-Danlos syndrome after the occurrence of recidivant spontaneous pneumothorax. We present the most common pulmonary complications of Ehlers-Danlos syndrome and discuss the importance of not forgetting the least commons etiologies of pneumothorax, in cases of spontaneous pneumothorax.

Angiosarcoma of the lung.

Angiosarcoma is a rare malignant vascular tumor. Pulmonary involvement is usually attributable to metastasis from other primary sites, primary pulmonary angiosarcoma therefore being quite uncommon. We report a case of angiosarcoma with pulmonary involvement, probably primary to the lung, which had gone untreated for more than two years. We describe this rare neoplasm and its growth, as well as the extensive local invasion and hematogenous metastasis at presentation. We also discuss its poor prognosis.

[Idiophatic mediastinal fibrosis: a case report]. / Fibrose idiopática do mediastino: A propósito de um caso clínico.

We herein report a case of a male patient, who presented a mediastinal mass that had a slow growth over the years. After surgery, that made the diagnosis of idiopathic mediastinal fibrosis, the growth stabilized. Four years latter it was observed a substantial increase which lead to the inevitable compression of vital structures which, in turn, led to pulmonary hypertension. Idiopathic mediastinal fibrosis is an extremely rare pathology. There are same cases in which it is associated with other pathologies but has always a fatal prognosis when surgery is not an option.

Bevacizumab for the treatment of nonsquamous non-small-cell lung cancer in Portugal: a retrospective, multicenter study.

INTRODUCTION: Lung cancer is the leading cause of cancer-related mortality. In patients with nonsquamous non-small-cell lung cancer (NSCLC) stage IIIB/IV treatment with chemotherapy plus bevacizumab led to significant improvements in progression-free and median overall survival (OS). AIM: To report the experience of five Portuguese centers in treating patients with nonsquamous NSCLC in stage IIIB or IV with bevacizumab and chemotherapy regarding survival and toxicity outcomes. MATERIALS AND METHODS: This was a retrospective, multicenter study on patients with nonsquamous stage IIIB/IV NSCLC treated with bevacizumab and chemotherapy from November 2007 to August 2010 through special use permits. We reviewed the medical records, registry of demographic characteristics, treatments provided, treatment responses, adverse events, and dates of death. Statistical analysis was performed with SPSS statistics software. Median OS and event-free survival (EFS) were calculated using the Kaplan-Meier method. RESULTS: From an eligible population of 41 patients, 37 participants were registered. Study participants were predominantly male (78.4%) with a median age of 53 years (29-75 years). In total, 83.8% patients had stage IV disease (TNM, 6th Ed.). The OS was 21.5 months (95% confidence interval [CI]: 12.6-30.5] and median EFS was 9.4 months (95% CI9: 7.1-11.7). Hematologic toxicity grade 3/4 occurred in 35.1% of patients, and nonhematologic toxicity in 24.3% patients. One fatal thromboembolic event was recorded (2.7%). CONCLUSIONS: The results of chemotherapy plus bevacizumab treatment for nonsquamous NSCLC obtained from the daily clinical practice of the centers involved in this study were similar to those of published clinical trials. Collaboration between the different Portuguese centers is crucial for this kind of study.

Angiosarcoma of the lung / Angiossarcoma pulmonar

Angiosarcoma is a rare malignant vascular tumor. Pulmonary involvement is usually attributable to metastasis from other primary sites, primary pulmonary angiosarcoma therefore being quite uncommon. We report a case of angiosarcoma with pulmonary involvement, probably primary to the lung, which had gone untreated for more than two years. We describe this rare neoplasm and its growth, as well as the extensive local invasion and hematogenous metastasis at presentation. We also discuss its poor prognosis.

O angiosarcoma é um tumor vascular maligno. O envolvimento pulmonar é geralmente atribuído à metástase de outros sítios primários, sendo o angiossarcoma pulmonar primário extremamente raro. Relatamos um caso de angiossarcoma com envolvimento pulmonar, provavelmente primário no pulmão com mais de dois anos de evolução. Descrevemos seu crescimento e sua extensa invasão local e hematogênica na apresentação. Documentamos ainda seu mau prognóstico.

Clinical characteristics of patients with lung cancer and metachronous or synchronous tumours with other locations.

Lung cancer is the leading form of cancer death worldwide. Cancer patients are at a high risk of developing a second cancer. The present study attempts to determine the characteristics of a population with lung cancer diagnosed with another cancer. We analysed records of patients from the Department of Lung Oncology of our hospital from 2000 to 2007 who were identified as having two or more tumours. We found 4.2% (n=44) multiple cancers among the registered cases (n=1046), 88.6% males, (high) mean age 70.1+/-10 years old. About 86% (n=38) of the patients were smokers or ex -smokers. From the patients with record of family history, 65.4% (n=17) had relevant family history of cancer. The majority of the first malignancy diagnosed was prostate, colon, head and neck and bladder. Lung cancer was essentially the second malignancy. The mean time lag between the two diagnoses was 62.9+/-64.9 months (max. 240, min. 0), with the second cancer usually detected at an advanced stage. The mean survival of patients who had a second primary lung cancer was 8.6+/-8.24 months (max. 32, min. 1), with four patients still surviving. Our results suggest that extended follow -up is needed in these patients, using screening strategies which follow international recommendations, and with control of carcinogenic risk factors such as smoking. We suggest a tailored risk algorithm and a further study to assess if there are particular molecular markers in these patients.

[Malignant peripheral nerve sheath tumors: A case report]. / Tumor maligno da bainha dos nervos periféricos do pulmão: A propósito de um caso clínico.

Malignant peripheral nerve sheath tumors comprehend a rare group of soft tissue sarcomas that tend to occur in patients with neurofibromatosis type 1 or several years after radiotherapy treatments. Its thoracic localization is a very unusual entity. The typical symptoms are due to nerve roots compression which can persist for several months or years before diagnosis. Due to very few patients with this type of tumor its therapeutic approach is still a matter of permanent debate, being surgery the main treatment. This tumor has a bad prognosis because of high local recurrence and metastasis. The clinical case we describe serves as a glimpse for discussion.