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.

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.

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.

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.

Biphasic malignant mesothelioma cases with osseous differentiation and long survival: a review of the literature.

This report describes two cases of diffuse biphasic malignant mesothelioma with osseous differentiation and long survival. Two male patients aged 66 and 54 years presented with chest pain, bloody pleural effusions, and diffuse and nodular pleural thickening. They both had a history of prolonged asbestos exposure. Sarcomatoid and epithelioid areas were identified on histopathological examination. In addition, there were foci of ossification. Epithelioid tumor cells as well as benign osteoclasts within the ossification reacted positively for cytokeratin 5/6, whereas sarcomatoid areas were negative. Both patients survived 39 and 69 months, respectively. To our knowledge, these two cases with biphasic malignant mesothelioma and osseous differentiation with long survival are the first to be described from Central Anatolia.

Vascular endothelial growth factor immunostaining correlates with postoperative relapse and survival in non-small cell lung cancer.

BACKGROUND: Angiogenesis is an early step in tumor progression, and vascular endothelial growth factor (VEGF) is an important angiogenic factor. In this study, we investigated the prognostic significance of VEGF immunostaining in tumor tissues of non-small cell lung cancer (NSCLC) patients during a 5-year follow-up period. METHODS: The study comprised 50 male patients diagnosed with NSCLC with a mean age of 57.26 +/- 8.64 years (range: 40-74 years). All patients had early stage NSCLC and none of the patients received chemo- or radiation therapy before surgery. VEGF immunostaining was performed in tumor tissues and immunoreactivity was graded as negative (0-10%), weak (10-50%), and strong (>50% tumors are stained). RESULTS: VEGF staining was weak in 20 (40%) specimens and strong in 13 (26%) specimens, whereas VEGF staining was negative in 17 (34%) specimens. Strong VEGF staining showed a significant correlation with both short time of relapse (p = 0.0001) and short survival (p = 0.0005). Multivariate analysis using Cox regression model was performed to determine the independent prognostic factors. Age (p = 0.029, OR: 1.05), tumor stage (p = 0.001, OR: 14.89), and VEGF staining (p = 0.006, OR: 4.65) were all found as independent prognostic factors in NSCLC. CONCLUSIONS: Strong VEGF immunostaining in tumor tissues was found to be an important prognostic factor for time to relapse and survival in patients with early stage disease.

Surgical management of bronchiectasis in childhood.

OBJECTIVE: Though there is a gradual decrease in the prevalence of bronchiectasis, it is still a cause of mortality and morbidity among children in developing countries such as Turkey. We reviewed the morbidity and mortality rates and the outcome of surgical treatment for childhood bronchiectasis. PATIENTS AND METHODS: Age, sex, etiological factors, symptoms, radiological examinations, surgical procedures, postoperative morbidity and mortality in patients aged 16 years and younger, operated for bronchiectasis between January 1991 and April 2006 in the Thoracic Surgery Clinic of Atatürk Training and Research Hospital for Chest Disease and Chest Surgery were reviewed retrospectively. RESULTS: Between January 1991 and April 2006, 176 cases aged 16 and younger were operated for bronchiectasis. There were 95 females (54%) and 81 males (46%), with a mean age of 12.3 years (range: 3.4-16 years). The most common cause of bronchiectasis was lung infection (n: 87, 49.4%). Main symptoms were coughing (n: 167, 94.9%), sputum (n: 139, 79%), hemoptysis (n: 78, 44.3%), and fever (n: 77, 43.7%). Mean duration of the symptoms in patients who were operated was 3.8 years (range: 0-7.6 years). Cases underwent a total of 201 operations: 19 cases had bilateral surgical resection and 6 cases had completion pneumonectomy. Majority of cases had complete resection (n: 165, 93.75%) while only 11 cases (6.25%) had incomplete resection. Mean duration of hospitalization was 8.9 days (range: 5-39 days). The outcome, based on the responses of patients postoperatively, was 'perfect' in 129 cases (73.3%), 'improved' in 41 cases (23.3%), and 'no changes' in 6 cases (3.4%). The mean follow-up after surgery was 4.3 years (range: 14 months to 7.2 years), mortality was 0% and morbidity was 13% (n: 23). CONCLUSION: With acceptable mortality and morbidity rates and high chance of cure after complete resection, surgical treatment is a successful and reliable method of treatment in childhood bronchiectasis that yields marked improvement in the quality of life.

Primary pulmonary mucinous adenocarcinoma in a 15-year-old boy.

Bronchogenic carcinomas are rare in childhood. Furthermore, mucinous (so-called colloid) adenocarcinoma, an unusual variant of pulmonary adenocarcinoma, is extremely rare in the first decade of life. To the best of our knowledge, we report the first case with primary pulmonary mucinous adenocarcinoma at the age of 15. Another interesting aspect of this tumor was its metastasis to thyroid, because metastasis of primary bronchogenic carcinomas to thyroid is uncommon. One can face up with difficulties in the establishment of the definite diagnosis due to its complex and often indistinguishable histopathologic pattern. In this paper we report a patient with pulmonary solid mass and thyroid nodule, initially diagnosed as metastatic thyroid carcinoma in whom postoperative resective surgery confirmed primary pulmonary mucinous adenocarcinoma with synchronous metastasis to thyroid.

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.

One-stage operation for hydatid disease of lung and liver: principles of treatment.

OBJECTIVE: Hydatid disease is endemic in many countries throughout the world. Although we do not have exact figures, the disease is prevalent in Turkey. A considerable number of patients have additional liver cysts as well. In this study we reviewed our experience with hydatid disease of the lung and liver and discussed the principles of treatment. METHODS: From 1971 to 1999, 2509 patients with pulmonary hydatidosis were referred to us, and 485 of them had concomitant liver cysts. Of these, 405 patients had cysts located on the dome of the liver, and they were operated on with phrenotomy through a right thoracotomy. Eighty patients who were found to have concomitant liver cysts in the lower part of the liver were referred to general surgery for a laparotomy. RESULTS: Hydatid cysts located in the lungs were managed by means of cystotomy and capitonnage. For liver cysts, cystotomy and inversion of the cavity with sutures was the surgical method of choice, and a drain was left in place. In case of multiple cysts in the liver, needle aspiration was preferred. Twelve major complications, including excessive biliary drainage and bronchobiliary fistula, occurred in these patients. CONCLUSIONS: We believe that management of pulmonary and hepatic cysts simultaneously through the thoracic route is convenient and should be encouraged in patients because this prevents a second operation. Needle aspiration can be applied only for liver cysts. It is absolutely contraindicated in lung hydatid cysts.

A review of 24 patients with bronchial ruptures: is delay in diagnosis more common in children?

OBJECTIVE: Bronchial ruptures due to blunt chest traumas are rarely encountered injuries. Because they occur seldom in pediatric age groups, even total ruptures of bronchi can be overlooked in the absence of accompanying lesions. This condition may result in a delay of diagnosis that consequently leads to irreversible septic changes in the lungs, which make resections unavoidable. The determination of predisposing factor(s) may be valuable in predicting and prevention of such situations. METHOD: We reviewed the records of 24 patients with bronchial ruptures hospitalized between January 1974 and December 2001 in Atatürk Center for chest disease and chest surgery. We divided the cases as 'early' and 'delayed' and each group consisted of 'adult (age>15 years)' and 'pediatric (age<15 years)' patients. RESULTS: On reviewing the records of these patients, we observed that 75% of the delayed cases were under the age of 11 years when they survived the thoracic trauma. CONCLUSION: The susceptibility of children to delays is emphasized and the possible causes are discussed in the light of world literature. Simple approaches to minimize the number of misdiagnosed pediatric cases are highlighted.

A comprehensive analysis of traumatic rib fractures: morbidity, mortality and management.

OBJECTIVE: A rib fracture secondary to blunt thoracic trauma is an important indicator of the severity of the trauma. In the present study we explored the morbidity and mortality rates and the management following rib fractures. METHODS: Between May 1999 and May 2001, 1417 cases who presented to our clinic for thoracic trauma were reviewed retrospectively. Five hundred and forty-eight (38.7%) of the cases had rib fracture. There were 331 males and 217 females, with an overall mean age of 43 years (range: 5-78 years). These patients were allocated into groups according to their ages, the number of fractured ribs and status, i.e. whether they were stable or unstable (flail chest). RESULTS: The etiology of the trauma included road traffic accidents in 330 cases, falls in 122, assault in 54, and industrial accidents in 42 cases. Pulmonary complications such as pneumothorax (37.2%), hemothorax (26.8%), hemo-pneumothorax (15.3%), pulmonary contusion (17.2%), flail chest (5.8%) and isolated subcutaneous emphysema (2.2%) were noted. 40.1% of the cases with rib fracture were treated in intensive care units. The mean duration of their stay in the intensive care unit was 11.8+/-6.2 days. 42.8% of the cases were treated in the wards whereby their mean duration of hospital stay was 4.5+/-3.4 days, while 17.1% of the cases were followed up in the outpatient clinic. Twenty-seven patients required surgery. Mortality rate was calculated as 5.7% (n=31). CONCLUSIONS: Rib fractures can be interpreted as signs of significant trauma. The greater the number of fractured ribs, the higher the mortality and morbidity rates. Patients with isolated rib fractures should be hospitalized if the number of fractured ribs is three or more. We also advocate that elderly patients with six or more fractured ribs should be treated in intensive care units due to high morbidity and mortality.

Surgery in bronchial carcinoids: experience with 83 patients.

OBJECTIVE: With the changing clinical presentation and histopathological pattern, carcinoids are now considered as a distinct and well-defined group in the neuroendocrine tumour scale. Surgery, especially parenchyma-sparing operations, are the treatment of choice for carcinoids. METHODS: Over a 25-year period, 83 patients with typical carcinoid tumour underwent thoracotomy on in our institution. The records of the patients were reviewed and the results were evaluated. RESULTS: The diagnosis was made with radiological methods and bronchoscopy. Cough and recurrent pneumonia were the most common symptoms. A variety of surgical procedures were performed. Thirty of the 83 patients underwent tissue-saving operations. Twenty patients underwent bronchotomy excision, eight were managed with sleeve or partial sleeve resective procedures, and two underwent segmentectomy. CONCLUSIONS: Conservative surgery is the treatment of choice of carcinoids, which were histologically typical and anatomically endobronchial. Especially for polypoid type carcinoids and for selected cases with sessile type, bronchotomy with simple excision and sleeve resections is a simple and effective method. As these types of operations produce a better functional result, they should be encouraged in these patients.

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.

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.

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.

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.