Thymic carcinoma presenting with overlap polyarthritis and myositis: A rare paraneoplastic syndrome in childhood.

Thymic tumors are very rare neoplasms in children and account for less than 1% of mediastinal tumors in pediatric patients. One-third of the pediatric patients present with symptoms related to the compression of the tumor mass on the surrounding anatomic structures, and paraneoplastic syndromes such as myasthenia gravis, pure red cell aplasia, acquired hypogammaglobulinemia, and connective tissue disorders, which rarely occur in children with thymic tumors. Herein, we report a case of thymic carcinoma mimicking the symptoms of a connective tissue disease with symmetrical polyarthritis accompanying myositis, fever, weight loss, and malaise in a 15-year-old male patient. To our knowledge, this is the first case pediatric thymic carcinoma accompany with severe polyarthritis and myopathy, thus we have reviewed the current literature regarding the cases of thymic malignancies coexisting with paraneoplastic syndromes in children.

Management of Massive Hemoptysis: Analyses of 58 Patients.

OBJECTIVES: The objective was to describe changing patterns of etiological factors and treatment modalities for massive hemoptysis. MATERIAL AND METHODS: From January 2008-December 2012, the medical records of 58 massive hemoptysis patients were reviewed. RESULTS: Fifty-eight patients, 44 were men (75.9%) and 14 were women (24.1%), with a mean age of 51.4 years (range= 19-84 years), were divided into three groups; surgical management (n= 37, 63.8%), conservative management (n= 14, 24.1%) and bronchial artery embolization (n= 6, 10.4%). One case (1.7%) had combined treatment modality; bronchial artery embolization was followed by surgical resection. Anatomical lung resections were the most preferred resection type in the surgical management group (n= 34, 91.9%). The most common etiological factor was bronchiectasis (n= 19, 32.8%); followed by bronchial cancer (n= 14, 24.1%). The duration of hospitalization in the surgical management group was 11.4 days (range= 4-24); whereas in the bronchial artery embolization group, hospitalization was only four days (range= 2-7) (p< 0.01). Prolonged air leak (n= 7; 18.9%) was the most common complication in the surgical management group. CONCLUSION: We emphasize that bronchiectasis was leading cause of massive hemoptysis. Surgical treatment remains the definitive therapy in the management of massive hemoptysis with decreased mortality rates over decades; whereas bronchial artery embolization is an effective therapeutic tool.

Coexistence of Obstructive Sleep Apnea and Superior Vena Cava Syndromes Due to Substernal Goitre in a Patient With Respiratory Failure: A Case Report.

INTRODUCTION: Substernal goiter may rarely cause superior vena cava syndrome (SVCS) owing to venous compression, and cause acute respiratory failure due to tracheal compression. Obstructive sleep apnea syndrome (OSAS) may rarely occur when there is a narrowing of upper airway by edema and vascular congestion resulting from SVCS. CASE PRESENTATION: We presented the clinical course and treatment of acute respiratory failure (ARF) developed in a patient with SVCS and OSAS due to substernal goiter. After treatment of ARF with invasive mechanical ventilation, weaning and total thyroidectomy were successfully performed through collar incision and median sternotomy without complications. CONCLUSIONS: Our case showed that if the respiratory failure occurred due to substernal goiter and SVCS, we would need to investigate the coexistence of OSAS and SVCS.

Surgical management of pulmonary aspergilloma: clinical experience with 77 cases.

BACKGROUND/AIM: This retrospective study evaluated the clinical presentation, underlying lung disease, surgical indications, technique, treatment outcomes, and postoperative complications of pulmonary aspergilloma. MATERIALS AND METHODS: We evaluated 77 patients who underwent pulmonary resection of an aspergilloma at Atatürk Chest Diseases and Thoracic Surgery Research and Training Hospital between January 2000 and December 2013. The initial operations were 4 pneumonectomies, 24 lobectomies, 9 lobectomy plus myoplasties, 10 segmental resections, and 30 wedge resections. Six reoperations were carried out to deal with postoperative complications: 1 myoplasty, 2 completion lobectomies plus myoplasties, 2 myoplasties with rib resections, and 1 completion lobectomy. RESULTS: The subjects comprised 53 males (mean age: 44.26 (range: 10-73) years) and 24 females (mean age: 48.25 (range: 26-70) years). The most common indication for surgery was hemoptysis in 52 patients (67.53%). The most common underlying lung disease was tuberculosis in 37 patients (48.05%). Forty patients (51.94%) had a simple pulmonary aspergilloma and 37 (48.05%) had a complex pulmonary aspergilloma. Major complications occurred in 18 patients (23.37%). The postoperative mortality rate was 3.89%, with 3 patients dying. CONCLUSION: Surgical resection of pulmonary aspergilloma is the best way to prevent recurrent hemoptysis with low morbidity and mortality.

Pulmonary tumor thrombotic microangiopathy from metastatic epithelioid angiosarcoma.

The lung is most common site for metastatic disease via hematogenous route. Tumor emboli of the vessels of the lung induces fibrocellular and fibromuscular intimal proliferation. These histopathological changes may cause pulmonary tumor trombotic microangiopaty. Few cases are diagnosed antemortem. We report a 60 year old woman with by metastatic epithelioid angiosarcoma involving the lung. Tumor cells were positive for VEGF and topoisomerase II. VEGF may be involved in the pathogenesis pulmonary tumor trombotic microangioapy and topoisomerase II positivity showed sensitivity against catalytic topoisomerase II inhibitors.

Pulmonary Langerhans cell histiocytosis; characteristics of 11 cases.

INTRODUCTION: Pulmonary Langerhans cell histiocytosis (PLCH) is a rarely seen disease of younger population. Almost all of the patients were smoker. In this study we aimed to evaluate the characteristics, diagnosis, treatment modalities and prognosis of 11 cases with PLCH. MATERIALS AND METHODS: We retrospectively reviewed our case series of eleven patients who were pathologically diagnosed as PLCH. The median age was 35 years (19-51) and male to female ratio (M/F) was 5/6. All of the patients were symptomatic. The most common symptoms were dyspnea (81.8%) and dry cough (72.7%). Mean duration of the symptoms was 10.8 months. All patients except two of them were smoker (81.8%). All patients were also passive smokers. RESULTS: Bilateral cystic appearance (n= 9, 81.8%), interstitial findings [septal and peribronchovascular thickening (72.7%) and nodular pattern (54.5%)] were common radiological findings. Spontaneous pneumothorax was present in two cases. All patients were diagnosed with surgical biopsies (90.9%) or transbronchial parenchymal biopsy (9.1%). Smoking cessation (81.8%) and immunosupression therapy (methylprednisolone) were the treatment modalities. Mean follow-up period was 5.40 ± 1.78 years. Generally, symptoms were improved with smoking cessation or methylprednisolone therapy. One patient was readmitted to our clinic with recurrent pneumothorax. In conclusion, it should be kept in mind that passive smoking is also responsible in the pathogenesis of PLCH. CONCLUSION: Exact consensus for PLCH treatment was not present except a few recommendations. In the future, with the understanding of the pathogenesis of the disease, new therapeutic agents will be discovered for this rare condition.

The effect of CYP1A1, GSTT1 and GSTM1 polymorphisms on the risk of lung cancer: a case-control study.

Lung cancer, which is mainly affected by environmental factors, is a lethal malignancy. It is also important to investigate the effect of genetic factors on lung cancer aetiology. In this study, we aimed to investigate the distribution of CYP1A1*2C, GSTT1 and GSTM1 polymorphisms in Turkish lung cancer patients to determine whether any promoting effect of polymorphisms could cause development of lung cancer. For this purpose, genomic DNA samples obtained from peripheral blood of 128 patients with lung cancer and 122 healthy subjects were analyzed. Genotyping of polymorphic enzymes were carried out by polymerase chain reaction-restriction fragment length polymorphism methods. Although there were no significant differences between groups in terms of CYP1A1 polymorphism, the carriers of CYP1A1 Ile/Val genotype (odds ratio [OR] = 1.224, 95% confidence interval [CI]: 0.585-2.564) or CYP1A1 Val/Val genotype (OR = 3.058, 95% CI: 0.312-30.303) had an increased risk of lung cancer development. There was no statistical difference between groups in terms of both GSTT1 null genotype (OR = 1.114, 95% CI: 0.590-2.105) and GSTM1 null genotype (OR = 0.776, 95% CI: 0.466-1.290). This is the first case-control study investigating CYP1A1 Ile/Val, GSTT1 and GSTM1 polymorphisms in Turkish lung cancer patients. Although we suggest that other genes in addition to the proposed genes could play a role in lung cancer development, the results of our study will contribute to the possible associations between CYP1A1 Ile/Val, GSTT1 and GSTM1 gene polymorphism on the risk of lung cancer.

Expression of CD44 and MMP-2: possible association with histopathological features of pleuro-pulmonary solitary fibrous tumors.

OBJECTIVE: Recent studies have shown that tumor cell adhesion molecules CD44 and matrix metalloproteinases (MMP-2) are expressed strongly in many tumors and associated closely with invasion and metastasis of these tumors. Although solitary fibrous tumors (SFT) have a good prognosis, a minority behave malignantly. The aim of this study was to analyze the correlation between CD44 and MMP-2 expression with histopathological parameters in SFT. MATERIAL AND METHOD: Haemotaxylin-Eosin stained sections of 10 patients with SFT were reexamined for evaluation of histopathological parameters. Immunostaining of CD44 and MMP-2 was performed by using the streptavidin-biotin method with mouse monoclonal antibody. RESULTS: Our cases consisted of three male and seven female patients with a mean age of 54.5 years. Three patients had a history of asbest exposure. Complete resection was performed in 2 malignant (multiple masses) and 8 benign SFT cases. One intrapulmonary tumor was treated with pneumonectomy. 3 cases originated from the right and 7 from the left hemithorax. Tumor size ranged from 5 to 27cm. All cases expressed strong CD44. Only 2 malignant SFT and intrapulmonary SFT expressed focal MMP-2. CONCLUSION: Although MMP-2 positivity was observed in 2 malignant cases, CD44 positivity was not associated with malignancy criteria in solitary fibrous tumors.

Kergin pneumonectomy: a rare procedure.

We have evaluated our cases of "Kergin" pneumonectomy in which a bronchial flap of the medial part of the right main stem bronchus was created during right pneumonectomy and was turned upward to cover the lateral airway defect at the level of the carina. Five male patients with a mean age of 53.6 underwent "Kergin" pneumonectomy due to nonsmall cell carcinoma arising from right upper lobe entrance, which does not allow a classical pneumonectomy. Postoperative pathology stagings were stage IIB in 1 patient and stage IIIA in 4 patients. Any operative mortality or short-term complication was not observed. Two of the patients died in the second year of follow-up. "Kergin" pneumonectomy is a rarely performed procedure with acceptable morbidity and mortality and good lung cancer resection. Actually, our current report of five cases will be one of the largest series of "Kergin" pneumonectomy.

Long-term outcomes and prognostic factors of patients with surgically treated pulmonary carcinoid: our institutional experience with 104 patients.

OBJECTIVE: Pulmonary carcinoid tumors are rare low-grade malignant neoplasms and constitute 2-5% of all lung tumors. In this study, we aimed to determine the clinical presentations, types of surgery, long-term treatment outcomes and survival rates of patients diagnosed with carcinoid tumor treated surgically in our clinic. METHODS: Patients operated in our clinic between 1992 and 2008 with confirmed or suspected diagnoses of carcinoid tumors were included in our study. Patients' hospital records were retrospectively analyzed. RESULTS: A total of 104 patients (age 19-71 years, mean 44±13 years, 2 SD) with pathologically confirmed carcinoid tumor were analyzed. A total of 84 patients (81%) were diagnosed as typical and the remaining 20 (19%) being atypical carcinoid tumor. As many as 24 patients (23%) were asymptomatic. The most frequent symptom was recurrent respiratory infection (35%). The most used surgical procedures were lobectomy (47%) and bilobectomy (16%). Mean postoperative follow-up period was 72 months (6-190 months). No surgery related mortality was noticed. As many as 15 (14%) patients died during the follow-up period. Overall 5- and 10-year survival rates were 89% and 72%, respectively. For typical carcinoid tumors, the 5- and 10-year survival rates were 92% and 83%, and for atypical carcinoids 73% and 46%, respectively (p<0.001). CONCLUSIONS: In our study, we noticed histological subtype, stage of the disease and the type of surgery performed to be prognostic factors of carcinoid tumors. Atypical carcinoid tumors tend to be more metastatic and had worse prognosis when compared with typical carcinoid tumors. We conclude that surgery is the best treatment of choice for carcinoid tumors, especially parenchyma-sparing procedures, because of their good mid- and long-term survival rates.

Sleeve resections for squamous cell carcinoma of the lung.

BACKGROUND: Sleeve resection is an advanced technique that was developed as an alternative to pneumonectomy. This study evaluated our cases of sleeve resection for squamous cell carcinoma of the lung and compared the outcomes with the literature reports. METHODS: In total, 26 bronchial, 5 bronchovascular, and 3 vascular sleeve lobectomies were performed between January 2000 and July 2005 in our clinic. Age, gender, operations, postoperative diagnosis and staging, and postoperative morbidity and mortality were evaluated. RESULTS: Sleeve resections were performed in 34 patients. All patients were male, with a mean age of 59.4 years. The operations consisted of 16 right upper, 14 left upper, and 1 left lower sleeve lobectomies and 3 superior sleeve bilobectomies. The most common postoperative pathological staging group was stage IIb (32.3%). Operative mortality was 5.9% (n=2). Postoperative morbidity was 20.5% (n=7), including 4 prolonged air leaks plus empyema, 1 prolonged air leak, 1 postoperative bleeding needing revision, and 1 severe bronchostenosis; of these, 6 had persistent atelectasis. The local tumour recurrence rate was 11.7% (n=4). The median survival time and 5-year survival were 36 months and 42%, respectively. CONCLUSIONS: Sleeve resection proved to be good therapy for lung cancer and has a lower morbidity and mortality than standard pneumonectomies and results in better lung function and quality of life. The anastomosis-related complications are experience-related technical complications and training thoracic surgeons to perform SRs at experienced centres will reduce the morbidity associated with SRs.

Synchronous bilateral multiple typical pulmonary carcinoid tumors.

Synchronous bilateral multiple carcinoid tumors of the lung are uncommon. We report an unusual case of synchronous, bilateral, and multiple typical pulmonary carcinoids. A 58-year-old woman with cough and chest pain was admitted to our clinic. A thoracic computed tomographic scan showed multiple bilateral nodular lesions. Bronchoscopic evaluation was normal. We performed sequential bilateral thoracotomies, frozen section examination, wedge resection, and lymphadenectomy. Histopathologic examinations revealed typical carcinoid tumor in both lungs. No pathologic lesions were observed in 18-month postoperative follow-up. We discuss treatment of synchronous bilateral multiple carcinoid tumors.

Evaluation of platelet parameters in patients with pulmonary hydatid cyst.

BACKGROUND: Echinococcosis is a near-cosmopolitan zoonosis caused by adult or larval stages of tapeworms (cestodes) into the genus Echinococcus (family Taeniidae). It was demonstrated that platelets were capable of killing parasites independent from leukocytes. PURPOSE: The aim of our study was to examine mean platelet volume (MPV), platelet mass (PM) and platelet count (PC), which are practical indicators of platelet activity in preoperative and postoperative periods of the patients with hydatid cyst. METHODS: In this retrospective study we evaluated 72 patients admitted to clinic of chest surgery with a diagnosis of pulmonary hydatid cyst in our hospital between January, 2006, and October, 2008. The MPV, PC, and PM were evaluated by complete blood count. PM was calculated by multiplying MPV and PLT. RESULTS: PREOPERATIVE MPV VALUES OF THE PATIENTS WAS FOUND TO BE SIGNIFICANTLY HIGHER THAN POSTOPERATIVE MPV VALUES (MEAN: 8.07±0.83, 7.78±0.87, p= 0.002). Preoperative PM values (median: 2456.75, range: 1013.70-5046.60) was found to be higher when compared to postoperative PM values (median: 2280.80, range: 134.20-4042.60) (p= 0.039). PC values were not significantly different between two periods (Preoperative-postoperative PC mean values: 320.48±98.42, 307.29±96.45, p= 0.286). CONCLUSION: In this study, we demonstrated that there were no statistical difference in PC for both periods but, MPV and PM were found statistical higher than postoperative period in preoperative period. PM and PM can be used as markers of disease activity in patients who undergo surgical resection of hydatid cyst.

Primary tumors of the ribs; experience with 78 patients.

A retrospective study of primary tumors of the ribs (PTR) was conducted to review their clinical, radiological, and pathological features, as well as the early and long-term results of surgical management. Seventy-eight patients (48 male, 30 female, age range: 7-80 years) with PTR were treated in our clinic between January 1998 and July 2008. Forty-nine (63%) of the patients had benign lesions and 29 (37%) had malignant lesions. In the group with malignant tumors, the main symptom was pain, but in the group with benign tumors, the main symptom was swelling. Eight cases in the benign group and four cases in the malignant group were asymptomatic (accidental radiological findings in recruitment centers). Most of the patients with PTR had benign lesions. All PTR should be considered as malignant until proven otherwise. Surgery must consist of wide resection with tumor-free margins in order to provide the best chance for cure in both benign and malignant lesions.

Thoracotomies in children.

Thoracotomies in children have been less extensively studied, as the incidence of diseases necessitating thoracotomies is low in the pediatric age group. This study reviews childhood thoracic diseases, thoracotomy approaches, indications, and complications. Surgical procedures and complications of a total of 196 children below 16 years of age who underwent thoracotomy for various reasons at the Department of Thoracic Surgery, Ataturk Chest Diseases and Chest Surgery Training and Research Hospital, between January 2000 and December 2004, were reviewed in this study. Out of the 196 patients, 77 were female (39%) and 119 (61%) were male. The most commonly encountered indications for surgery were hydatid cyst (35%), bronchiectasis (25%), chronic nonspecific pleuritis (13%), chest wall deformities (10%), and mediastinal cystic formations and masses (10%). The other indications included tuberculosis (3%), aspergilloma (0.5%), fibrohyalinized cyst (0.5%), resection of trachea (0.5%), bronchogenic cyst (0.5%), inflammatory pseudo-tumor (0.5%), sequestration (1%), lipoblastoma (0.5%), and eosinophilic granuloma (1%). Out of the 196 patients, 176 underwent lateral thoracotomy and 20 patients with a chest wall deformity underwent midsternal incision. Complications were seen in 35 patients (18%): atelectasia and secretory retention (54%), wound infection (17%), hemorrhage (3%), chylothorax (3%), intrathoracic space (3%), and postoperative extended air leakage (20%). The mean hospital stay was 15 days and we did not encounter any mortality. The physiology and anatomy of the respiratory system and especially the respiratory control mechanism in pediatric patients vary from those of the adults, resulting in a more morbid course after thoracic surgery in children. Despite severe postoperative pain, posterolateral thoracotomy is the preferred approach in adults because of an advanced intrathoracic exposure and easy manipulation. On the other hand, lower pain threshold and the different types of diseases seen in children make lateral thoracotomy a more appropriate choice for thoracotomy, which, at the same time, spares the serratus anterior muscle decreasing its negative impact on postoperative respiratory function.

Unforeseen scenario in removal of a tracheobronchial foreign body.

Tracheobronchial foreign body aspirations occur most commonly in children but under certain circumstances, are seen in adults. Majority of patients can successfully be managed via bronchoscopy. However, unexpected complications may develop during the removal procedure. We describe an unusual complication encountered during the removal procedure of an inhaled scarf pin in the trachea of a 23-year old woman. Crucial removal procedure is implicated and awareness of this rare complication is emphasized.

Diffuse cystic disease of the lung associated with simultaneous bilateral spontaneous pneumothorax: an unknown component of Caroli disease?

Simultaneous bilateral spontaneous pneumothorax is an uncommon and serious medical problem frequently related to an underlying lung disease such as parenchymal lung disease, inflammatory events or neoplasms. Recurrent pneumothorax associated with persistent air leakage may pose a therapeutic dilemma in patients with underlying lung diseases. Caroli disease (CD) is a very rare congenital disorder characterised by intrahepatic biliary cystic dilatations and frequently associated with polycystic kidney and hepatic fibrosis. To date, no relation between CD and the bilateral diffuse cystic structure of pulmonary parenchyma has been described. In this paper we present a patient with the diagnosis of CD in whom simultaneous bilateral spontaneous pneumothorax was the initial finding due to severe underlying pulmonary disease. Our patient's demographic and clinical characteristics, laboratory findings and course made us exclude the other aetiologies leading to such diffuse multi-bullous pulmonary involvement. The coincidence of recurrent pneumothorax and severe pulmonary disease has led to a big therapeutic dilemma. Open or minimally invasive surgery could be morbid or even mortal because of the nature of the parenchyma and the operative risk due to CD. Therefore, we suggest a unique conservative management including tube thoracostomy by locating the correct air spaces with the aid of high-resolution computed tomography followed by talc pleurodesis in patients with severe multi-bullous lung disease associated with potential risks due to co-morbidities.

Surgical results and prognosis of patients with primary bronchogenic carcinoma aged less than 36 years.

BACKGROUND AND OBJECTIVE: This study reports on the demographic features, clinico-pathological results and prognoses of patients aged less than 36 years diagnosed with non-small cell lung cancer (NSCLC). METHODS: This is an observational study of patients with primary NSCLC who had a surgical procedure at a tertiary thoracic surgery centre in Turkey. Data collected were age, gender, history of smoking, symptoms, postoperative histopathological diagnosis, stage, surgical procedure and survival. RESULTS: Of the 31 patients in the study, 27 were male (87%) and the median age was 32 years (10-35 years). Nineteen patients were smokers (61.2%). The most common presenting symptom was cough (n = 23, 67.7%). Histopathological diagnosis was squamous cell carcinoma (SCC, n = 17), adenocarcinoma (n = 12), lymphoepithelioma-like carcinoma (n = 1) and undifferentiated carcinoma (n = 1). Staging of the 17 patients with SCC (58.8%) was stage I and II (n = 10, 58%), and stage III (n = 7, 41%). Staging of the 13 patients with adenocarcinoma was stage IV (n = 2, 16%) and stage III patients (n = 8, 66%). Follow-up data were available on 22 patients (71%) and showed a median survival of 17.2 months. Two and 5-year survival rates were 54.5% and 45.5%, respectively. CONCLUSIONS: SCC comprised a relatively high proportion of NSCLC in these younger patients. Aggressive multimodality treatment may achieve satisfactory 2- and 5-year survival rates in young patients with NSCLC who usually present with advanced disease.