We report a patient with vascular-type Ehlers-Danlos syndrome (vEDS) who developed pneumothorax and was treated with a total pleural covering technique (TPC). A 24-year-old man developed repeat pneumothorax with intermittent hemo-sputum. Based on unusual radiological manifestations of lung lesions and physical findings, EDS was suspected as an underlying cause of the pneumothorax. Surgical treatment was performed using a mediastinal fat pad and TPC, and no relapse was seen up to 2 years after surgery. TPC is a less invasive surgical approach for selected patients with vEDS. Accurate underlying diagnosis of vEDS and systemic evaluation of vascular complications are necessary before planning surgery.
The HCS approach is suitable for advanced lung cancer, including invasion of mediastinal structures, the apical dome and mediastinal lymph nodes. It provides a wide view of the mediastinum and apex of the chest, and safe access to the thoracic great vessels, resulting in better long-term survival rates.
Adenoid cystic carcinoma (ACC), which is a subtype of the nonpapillary adenocarcinoma of the thymus, is extremely rare. We report a patient with thymic carcinoma with ACC-like features presented with multiple bone and pulmonary metastases that underwent surgery. The present case firstly demonstrated that thymic carcinoma with ACC-like features could have metastatic potential.Keywords: thymus, pathology, surgery which had been ceased 14 years prior. Spirometry revealed a decreased forced expiratory volume (FEV1.0 : 1.29L, FEV1.0% : 52.0%). Computed tomographic (CT) scan demonstrated an anterior mediastinal mass which measured 46 mm in diameter adjacent to the ascending aorta (Fig. 1A). Eight pulmonary nodules up to 8 mm in diameter were also demonstrated in the bilateral lungs. Masses were found in the manubrium and the right fifth rib which measured 25 mm and 15 mm in diameter, respectively ( Fig. 1B and 1C). A study of whole-body Fluorine-18-2-fluoro-D-glucose positron emission tomography/CT (FDG-PET/CT) revealed intense focal FDG uptake in the anterior mediastinal mass, the manubrium mass, and the right fifth rib mass [standard uptake value (SUV) max values were 4.6, 2.4, and 1.3 respectively] and no other abnormal FDG uptake.Based on these radiological findings, a thymic tumor with multiple bone and pulmonary metastases was suspected. A surgical biopsy of the manubrium mass was performed, and the mass was diagnosed to be "non-small cell carcinoma with a cribriform pattern". Immunohistochemically, the tumor cells were positive for AE1/AE3, and negative for CD56, chromogranin A, p63, SMA, and synaptophisin. The MIB-1 index was less than 10%. Taken together, these findings indicated that the thymic tumor was a low-grade malignant tumor despite its metastatic behavior. Surgery was planned.The patient was placed in the supine position under general anesthesia.
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