Objective

Esophageal cancer incident cases and deaths in 2009 were retrieved from national database of population based cancer registry to describe esophageal cancer burden in registration areas.

Methods

In 2012, 104 population-based cancer registries reported cancer incidence and mortality data of 2009 to Chinese National Central Cancer Registry. Total 72 registries’ data met the national criteria to be pooled and analyzed. The crude incidence and mortality rates of esophageal cancer were calculated by age, gender and area. China sensus in 1982 and Segi’s world population were applied for age standardized rates.

Results

The crude incidence of esophageal cancer ranked fifth in all cancer sites with rate of 22.14/100,000 (30.44/100,000 for male and 13.64/100,000 for female, 14.21/100,000 in urban and 38.44/100,000 in rural). Age-standardized rates by China population (CASR) and World population (WASR) for incidence were 10.88/100,000 and 14.81/100,000 respectively. The crude mortality of esophageal cancer ranked fourth in all cancer sites with rate of 16.77/100,000 (23.29/100,000 for male and 10.11/100,000 for female, 10.59/100,000 in urban and 29.47/100,000 in rural). The CASR and WASR for mortality were 7.75/100,000 and 10.76/100,000 respectively. For both of incidence and mortality, the rates of esophageal cancer were much higher in males than in females, in rural areas than in urban areas. The overall age-specific incidence and mortality rates showed that both rates were relatively low before 45 years old, and then gradually increased, reaching peak in age group of 80-84.

Conclusions

The burden of esophageal cancer remained high in China, especially for males in rural areas. Effective prevention and control action, such as health education, nutrition intervention and screening should be enhanced in the future.

Cystic lesions can be occasionally be found in the mediastinum, and typically include bronchogenic cysts, esophageal duplication cysts, and neuroenteric cysts. In 2005, Hattori described the first mediastinal cyst with Mullerian differentiation. Since that time, three other authors have described similar cysts occurring in the posterior mediastinum. Here we present two cases of patients with ciliated cysts with Mullerian differentiation with expression of estrogen receptor, progesterone receptor, PAX8 and Wilm’s tumor 1, occurring in the posterior mediastinum and review the related literature.

Chordomas are rare, slow-growing malignant bone tumours arising from cellular remnants of the notochord. These tumours are locally invasive but have also a metastastic potential.

Chordomas are characterized by the presence of physaliferous cells in a myxofibrillary stromal background. In cytological aspirates, these characteristic cells are usually absent, revealing only clusters of cells with varying degrees of vacuolation. This makes definitive diagnosis of chordoma difficult as the tumor can mimic other myxoid neoplasms including renal cell carcinomas and well-differentiated chondrosarcomas. In such situations, a confident diagnosis of chordoma requires comparison with histology of the primary tumor.

We describe the first case of metastatic chordoma diagnosed by endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA).

There are only a few cases of primary mediastinal synovial sarcoma in the literature. Normally, they do not respond well to chemotherapy. In our case, a 30-year-old patient was admitted due to thoracic pain, dyspnea, orthopnea, cough, hoarseness and weight loss over a 3-month period as well as a dramatic worsening a week before the admission. A chest radiography showed a completely white left hemithorax and contralateral mediastinal shift; in addition, a chest tomography revealed a giant heterogeneous mediastinal mass, lung atelectasia and a small pleural effusion. The patient was submitted to Chamberlain procedure (biopsy) under local anesthesia and the diagnosis of a synovial sarcoma was obtained after immunohistochemical analysis. Due to his poor general condition, he received chemotherapy first, with a dramatic response, after what, the mass that had been reduced was removed surgically. After a 5-year- follow-up period there are no signs of disease recurrence.

A 47-year-old man was referred to our hospital because of a 2-month history of dry cough, 2-kg weight loss, and a feeling of abdominal fullness. The PET-CT scan depicts the intense standard uptake values (SUVs) of the anterior and subphrenic lymphnodes, and intraperitoneal cavity, especially in the omentum, while, no uptake was found in the pleural cavity. Based on the pathological findings of the open lung biopsy specimens, he was diagnosed with malignant peritoneal mesothelioma of epithelioid type with thoracic metastasis. The present case demonstrated the some of the limitations of PET-CT in the diagnosis of malignant mesothelioma, which failed to detect pleural involvement despite aggressive invasion by this tumor.

Pulmonary enteric adenocarcinoma, a rare histologic type of primary pulmonary adenocarcinoma with only 16 cases reported to date, has to be differentiated from metastatic colorectal carcinoma. Here we report a case of pulmonary enteric adenocarcinoma which shows villin immunoreactivity in the brush border of tumor cells. As a marker for gastrointestinal adenocarcinoma, villin has been rarely found positive like this pattern in pulmonary adenocarcinomas. This case suggests brush border immunoreactivity of villin is possible in some cases of pulmonary enteric adenocarcinomas. We suggest pathological practitioners pay attention to it.

Background

Recently, driver oncogenes in adenocarcinoma of the lung were identified, and several molecular target agents were introduced in the clinical setting. However, there are few reports on the frequency of gene abnormalities in young patients with lung cancer.

Materials and methods

Twelve patients with lung adenocarcinoma aged 40 or younger at Juntendo University Urayasu Hospital or Juntendo University Hospital from July 2004 to March 2010 were analyzed for driver oncogene status including EGFR activating mutation, EML4-ALK fusion gene, and K-ras mutation.

Results

Four patients showed EGFR gene mutation. Five out of 7 EGFR mutation-negative patients showed positive results for EML4-ALK gene fusion. One case whose EGFR mutation was indeterminate.

Conclusions

Driver oncogene including EGFR mutation and EML4-ALK fusion gene was identified in 9 of 12 cases (75%). Examination of gene abnormalities is essential in young patients with non-small cell lung cancer to provide the best treatment.

Background

Cyclooxygenase-2 (COX-2) has been implicated in tumorigenesis and metastasis, and it presumably mediates the proliferation of endothelial cells and promotes vascular permeability. However, the prognostic value of COX-2 overexpression in patients with non-small cell lung cancer (NSCLC) remains controversial.

Methods

A systematic review of eligible studies with meta-analysis was performed to quantitatively review the correlation of COX-2 overexpression with survival in patients with NSCLC.

Results

We conducted a final analysis of 1,892 patients from 16 studies. The studies were categorized by histology, disease stage, patient race and laboratory techniques used. Combined hazard ratios (HR) suggested that COX-2 overexpression was not associated with a significant impact on survival, the HR (95% CI) was 0.90 (95% CI: 0.76-1.04) overall, 0.99 (0.71-1.26) in Asian patients, 0.87 (0.71-1.03) in non-Asian patients, 0.63 (0.33-0.93) in adenocarcinoma, 1.42 (1.02-1.81) in stage I NSCLC, 0.83 (0.72-1.08) in NSCLC by IHC, 3.28 (1.48-5.13) in NSCLC by RT-PCR.

Conclusions

COX-2 overexpression seems to have no significant impact on survival of NSCLC patients. However, the statistically significant was found in stage I NSCLC, suggesting that COX-2 expression could be useful at early stages to distinguish those with a worse prognosis.

Lymphangioleiomyomatosis is a rare slowly progressive lung disease that affects almost exclusively young women of reproductive age. It occurs sporadically or in association with Tuberous Sclerosis Complex. LAM is characterized by cystic remodeling of the lung parenchyma, due to proliferation of abnormal smooth muscle-like LAM cells and presence of extra pulmonary manifestations such as lymphadenopathy, angiomyolipomas and abdominal lymphangioleiomyomas. The most common clinical manifestations are progressive dyspnea on exertion, pneumothorax and chylous effusions. Currently there is no curative treatment for the disease, but the ongoing study of the genetic and molecular pathways implicated in the pathogenesis of the disease could lead to targeted therapy.

Background

Maximal oxygen consumption (VO2max) is considered a decisive test for risk prediction in patients with borderline cardiopulmonary reserve. Guidelines have adopted decreasing VO2max cut-off values to define operability within acceptable mortality and morbidity limits. We wanted to investigate how the adoption of decreasing VO2max cut-off-values assessment contributed to better select lung surgery candidates.

Methods

One hundred and nineteen consecutive surgical candidates have been prospectively analyzed as a sample population. Preoperative work-up included spirometry and transfer factor (DLco); irrespective of the spirometric values, these patients were subjected to VO2max assessment. Surgical eligibility was decided by the same surgeon throughout the series. In the postoperative period, overall mortality and the occurrence of any, major or minor complications was recorded and graded according to the Common Terminology Criteria for Adverse Events v.4.3.

Results

Three arbitrary cut-offs were introduced at 15, 14 and 12 mL.kg-1.min-1. Notably, 15 and 12 mL.kg-1.min-1 correlated with percentage VO2max values of 50% and 35% of predicted (P<0.0001 and 0.0079), respectively. Accordingly, the patients were subdivided into groups in which the prevalence of postoperative morbidity was recorded. The groups were homogeneous as to age, BMI, preoperative absolute and percentage FEV1 and DLco. In the Cox proportionate-hazards multivariate analysis, VO2max less than 35% (P=0.0017) and CTCAE >2 (P=0.0457) emerged as significant predictors of survival after surgery. Conversely on logistic regression analysis, age over 70 years (P=0.03) and pneumonectomy (P=0.001), but not VO2max cut-off values, were significant predictors of major (CTCAE >2) morbidity.

Conclusions

Since VO2max is increasingly used to contribute to risk prediction for the individual patient, surgeons need to be advised that the concept of a definitive, generalized cut-off value for VO2max is probably a contradiction in terms. Patient-specific VO2max values are more likely to contribute to risk assessment since they may reflect the primarily affected component among the determinants of maximal oxygen consumption. Whether patient-specific VO2max should be routinely used by surgeons to define operability for borderline patients needs further evaluation.

Objective

To analyze the characteristics and technical difficulties of complete video-assisted thoracoscopic surgery (c-VATS) for treatment of pulmonary sequestration operation.

Methods

25 cases of c-VATS lobectomy for intrapulmonary sequestration performed between January 2009 and May 2012 were reviewed. The 25 patients included 13 (52%) males and 12 (48%) females, with a mean age of 34.7 years (range, 16-62 years). Preoperative imaging by CT scan and three-dimensional reconstruction of abnormal blood vessels diagnosed 19 cases as pulmonary sequestration, misdiagnosed 1 case as pulmonary cyst syndrome, 4 cases as bronchiectasis and 1 case as benign tumor.

Results

All the patients underwent c-VATS excision, 16 in the left lower lobe, 7 in the right lower lobe, 1 in right middle lobe and 1 extralobar pulmonary sequestration. Vascular abnormality was observed intraoperative including the thoracic aorta in 20 cases, abdominal aorta in 2 cases, phrenic arteries and intercostal artery in 1 cases and thoracic aorta combined with abdominal aorta in 1 case. No conversion to open was achieved in all cases. The mean operating time was 114.2 mins (range, 78-156 mins), the mean blood loss was 228 mL (range, 50-3,000 mL), the mean duration of chest drainage was 3.2 days (range, 2-7 days) and the mean length of post-operative hospital stay was 6.6 days (range, 3-13 days). There was no mortality, without significant postoperative complications, were cured and discharged. Patients were followed up for 2-32 months, mean 21.4 months, with no recurrence.

Conclusions

c-VATS is feasible, effective, and safe in treatment of pulmonary sequestration. It is worthy of clinical application.

The use of non-invasive mechanical ventilation (NIV) during an asthma attack is controversial. We report a case of a 28-year-old female patient in her 16th week of pregnancy with community-acquired pneumonia who presented during an asthma attack, which led to hypoxic respiratory failure. She was successfully treated using NIV. This case is worth discussing as it includes two clinical conditions in which NIV is often considered contraindicated.

A single pleural space can lead to serious simultaneous bilateral pneumothorax in cases of congenital or acquired pleuro-pleural communication. Here we report a 35-year-old man with bilateral pneumothorax. Chest computed tomography scans revealed a small air space between the esophagus and aorta, suggesting pleuro-pleural communication. Bilateral thoracoscopic bullectomy was performed. Repeated inspection revealed a 2-cm-long pleural window between the aorta and esophagus, which was closed with a collagen patch. Although congenital pleuro-pleural communication has been regarded as rare, as many as nine patients have been reported (if suspected cases are included). The lower middle mediastinum should be carefully inspected.

Fibrosing mediastinitis is a rare but benign disorder characterized by an excessive fibrotic reaction in the mediastinum which can result in compromise of airways, great vessels, and other mediastinal structures. In this paper we presented a patient with fibrosing mediastinitis mimicking bronchogenic carcinoma. The patient was a 32-year-old diabetic male admitting with cough and hemoptysis. There was a right hilar mass and multiple mediastinal conglomerated lymph nodes on chest computed tomography. Positron emission tomography with computed tomography (PET/CT) scan demonstrated increased fluorodeoxyglucose (FDG) uptake at the right hilar mass lesion and mediastinal lymph nodes. Fiberoptic bronchoscopy showed mucosal distortion of right upper lobe. Pathologic examination of the mucosal biopsy revealed inflammation. Endobronchial ultrasound guided transbronchial needle and cervical mediastinoscopic lymph node biopsies were undiagnostic. Diagnostic thoracotomy confirmed the diagnosis fibrosing mediastinitis. Administration of six months of systemic corticosteroid and antituberculous therapy was not beneficial. In conclusion, despite being a rare clinical entity, fibrosing mediastinitis should be kept in mind in the differential diagnosis of mediastinal mass lesions of unknown etiology. The diagnosis is exceptionally difficult in the presence of atypical radiological findings. The treatment is particularly challenging without any proven effective therapy.

Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) has been widely used for sampling of the mediastinum and hilar lesions for diagnosis or for staging of lung cancer. The viewing fields of EBUS Scope are 35 or 45 degrees forward oblique. This makes the manipulation of the scope very difficult. Plus its bigger external diameter, it is unusable for routine airway examination and biopsy. It is uncomfortable for the patient and often need general anesthesia and usually is preceded by a standard bronchoscopic examination. We tested a Fuji EBUS scope (EB-530 US) extensively on a bronchial cast and EBUS teaching phantom, then applied to the patient. This case report describes the unique quality of this EBUS scope and its potential. It has a 10 degrees forward oblique view and smaller external diameter. It might be able to eliminate the need of a second scope and makes the TBNA with or without EBUS simpler to do and easier to learn.

Idiopathic pulmonary fibrosis (IPF) is a progressively fibrotic interstitial lung disease that is associated with a median survival of 2-3 years from initial diagnosis. To date, there is no treatment approved for IPF in the United States, and only one pharmacological agent has been approved outside of the United States. Nevertheless, research over the past 10 years has provided us with a wealth of information on its histopathology, diagnostic work-up, and a greater understanding of its pathophysiology. Specifically, IPF is no longer thought to be a predominantly pro-inflammatory disorder. Rather, the fibrosis in IPF is increasingly understood to be the result of a fibroproliferative and aberrant wound healing cascade. The development of therapeutic targets has shifted in accord with this paradigm change. This review highlights the current understanding of IPF, and the recent as well as novel therapeutics being explored in clinical trials for the treatment of this devastating disease.

Diffuse pulmonary ossification (DPO) is a rare entity which is characterized by metaplastic bone formation in the lung parenchyma. It is an uncommon condition without significant symptoms, which is usually diagnosed on autopsy. Diffuse pulmonary ossification can be easily misdiagnosed as one of interstitial lung diseases due to diffuse pulmonary lesions. Two types of diffuse ossification are described in medicine: dendriform and nodular. In this article, the authors present a patient with persistent pneumothorax who underwent investigation of the cause of his disease and a diagnosis of DPO was revealed.

Idiopathic pulmonary fibrosis (IPF) is a chronic diffuse interstitial disease characterized by a predominant reticular pattern of involvement of the lung parenchyma which can be well documented by High Resolution Computed Tomography (HRCT). While almost half of the patients with IPF may develop pulmonary arterial hypertension, the occurrence of superimposed acute thrombo-embolic disease is rare.

We describe a case of an 87 yrs old female who was found to have IPF complicated by acute pulmonary thrombo-embolism during the clinical and radiological investigation of a rapidly worsening dyspnea. While chest x-ray findings were initially considered consistent with a congestive heart failure, a bed side echocardiography revealed findings suggestive of pulmonary arterial hypertension and right ventricular failure with enlargement of both right cavities and associated valvular regurgitations. An acute thrombo-embolic disease was initially ruled out by a perfusion lung scintigraphy and subsequently confirmed by contrast-enhanced multi-detector CT which showed an embolus at the emergency of the right inter-lobar artery with associated signs of chronic pulmonary hypertension. However, unenhanced scans performed with both conventional and high resolution techniques also depicted a reticular pattern of involvement of lung parenchyma considered suggestive of IPF despite a atypical upper lobe predominance. IPF was later confirmed by further clinical, serological and instrumental follow-up.