[Effect of safinamide on morning-off phenomenon in elderly patients with Parkinson's disease].

AIM: Morning-off is a symptom experienced by patients with Parkinson's disease (PD), which markedly reduces patients' quality of life. The present study evaluated the effect of safinamide on morning-off in elderly PD patients. METHODS: This observational study included 30 PD patients treated with 50 or 100 mg/day of safinamide in the evening. Using patient-reported outcomes, we evaluated the effect of safinamide on daily/morning ON-time, daily/morning OFF-time, Unified Parkinson's Disease Rating Scale (UPDRS) Part III score, and non-motor symptoms. Data at baseline (treatment start) and at 4, 8, 12, and 16 weeks after baseline were recorded. RESULTS: The PD patients (75.8±7.5 years old) in this study, who tended to be older than in previous phase 2/3 or 3 studies, may represent real-world Japanese PD patients. Compared with baseline, safinamide significantly increased the daily ON-time at eight weeks and morning ON-time at four weeks. Safinamide significantly reduced the daily OFF-time and morning OFF-time at four weeks. The UPDRS Part III score was significantly reduced by 1 point at 12 weeks. Safinamide showed a tendency to reduce non-motor symptoms, such as anxiety, pain, and depressive feelings. There was no marked difference in these parameters between patients treated with 50 and 100 mg of safinamide. CONCLUSIONS: Our results suggest that safinamide administered in the evening can benefit elderly patients who experience wearing off, especially morning off, and non-motor symptoms.

Tracheal leiomyoma resected with endobronchial electrocautery snare.

Tracheobronchial leiomyoma is a rare benign tumor, which comprises about 2% of benign tumors of the lower respiratory tract. Bronchoscopic intervention is a minimally invasive treatment, but incomplete resection or recurrence has been reported. We report a case of a 76-year-old woman with a tracheal tumor of 14 mm in diameter incidentally detected on chest computed tomography. Bronchoscopic examination revealed a smooth polypoid tumor which pedunculated from the left side of the cartilaginous tracheal wall. An endobronchial electrocautery snare with flexible bronchoscope was successfully used under general anesthesia and with intubation. The resected specimen was pathologically consistent with leiomyoma. Bronchoscopic intervention can yield successful treatment of pedunculated tracheobronchial leiomyomas, although surgical resection should also be considered in cases of broad-based or iceberg-type leiomyomas because of the risk of incomplete resection or recurrence.

Malignant peripheral nerve sheath tumor in the anterior mediastinum: A case report.

Malignant peripheral nerve sheath tumors (MPNSTs) are rare neoplasms, accounting for only 5% of all malignant soft-tissue sarcomas. This is the case report of a 28-year-old male patient with a MPNST located in the anterior mediastinum, originating from the phrenic nerve. The patient presented with an abnormal shadow on chest radiography and a 10-cm mass in the right anterior mediastinum was identified on computed tomography and magnetic resonance imaging. The patient subsequently underwent surgical resection. The tumor originated from the right phrenic nerve and was tightly adherent to the middle and lower lobes of the right lung, the pericardium and the diaphragm. The tumor was completely resected, along with partial resection of the adherent tissues. The histological diagnosis was spindle cell sarcoma and the final diagnosis was MPNST of the anterior mediastinum, based on the characteristic microscopic appearance indicating the nervous origin of the tumor and on the intraoperative findings. The patient remains free of recurrence 1 year after surgery.

Placement of self-expandable metallic stents for tracheal stenosis secondary to thyroid cancer.

The indications and suitable approaches for treating upper airway obstruction secondary to thyroid cancer are controversial. Patients with thyroid cancer generally have a good prognosis, but airway stenosis and vocal cord paralysis are not uncommon. Subglottic airway stenting may be challenging, due to stent migration, granulation tissue formation and supraglottic stenosis. In this study, we evaluated the application of covered self-expandable metallic stents to relieve upper airway obstruction. This was a retrospective study of 5 patients with airway stenosis due to thyroid cancer treated in 2009 and 2010. Immediate airway enlargement was achieved in 3 patients with stenosis at the middle mediastinum. Gradual enlargement over 2 months was observed in the remaining 2 patients with stenosis at the cervical level. The performance status was improved in all 5 patients, including a case with anaplastic carcinoma. The follow-up averaged 13 months (range, 8-27 months). Granulation tissue developed at both ends of the stent in 3 patients, sputum was retained in 2 cases and bacterial colonization was detected in all 5 cases. No stent migration was reported. Additional tracheostomy was required in 2 patients, due to proximal tumor growth or progressive bilateral vocal cord paralysis after 10 and 6 months, respectively. In conclusion, stenting for central airway stenosis secondary to thyroid cancer may be beneficial, even in patients with anaplastic carcinoma. Long-term regular bronchoscopic follow-up is required to monitor complications, as patients with thyroid cancer are at high risk of granulation tissue formation, sputum retention and bacterial colonization.

Isolated nodular thymic amyloidosis associated with diplopia.

An 85-year-old man presented with diplopia and anterior mediastinal tumor that had enlarged during the preceding 4-year period. Computed tomographic chest imaging showed an irregularly shaped mass comprising two nodules (diameter, 4 cm) with calcification. Suspecting thymoma, we performed video-assisted thoracoscopic thymectomy. The resected specimen showed deposition of homogeneous eosinophilic and hyalinized material around the vessel wall in thymic tissue, and it stained positively for anti-λ antibody, indicating localized AL amyloidosis. There was no other organ dysfunction or symptoms and no evidence of systemic amyloidosis. Diplopia resolved immediately after thymectomy; however, the connection of diplopia with amyloidoma and thymic tissue remains uncertain.

Clinical significance of BIM deletion polymorphism in non-small-cell lung cancer with epidermal growth factor receptor mutation.

BACKGROUND: Germline alterations in the proapoptotic protein Bcl-2-like 11 (BIM) can have a crucial role in tumor response to treatment. To determine the clinical utility of detecting BIM deletion polymorphism in non-small-cell lung cancer positive for epidermal growth factor receptor (EGFR) mutation, we examined outcomes of patients with and without BIM alterations. METHODS: We studied 70 patients with EGFR mutation-positive non-small-cell lung cancer who were treated with an EGFR tyrosine kinase inhibitor between January 2008 and January 2013. BIM deletion was analyzed by polymerase chain reaction in 58 samples of peripheral blood and 24 formalin-fixed paraffin-embedded slides of surgical specimens (20 of lung tissue and four of brain tissue); both blood and tissue specimens were available for 12 patients. We retrospectively analyzed clinical characteristics, response rate, toxicity, and outcomes among patients with and without BIM deletion. RESULTS: BIM deletion was present in 13 of 70 patients (18.6%). There were no significant differences between patients with and without BIM deletion in clinical characteristics, rate of response to EGFR tyrosine kinase inhibitor, or incidence of adverse events. Patients with BIM deletion had significantly shorter progression-free survival (PFS) than those without BIM deletion (median, 227 versus 533 days; p < 0.001). Multivariate Cox regression analysis showed that BIM deletion was an independent indicator of shorter PFS (hazard ratio, 3.99; 95% confidence interval, 1.864-8.547; p < 0.001). CONCLUSIONS: Polymerase chain reaction successfully detected BIM deletion in samples of peripheral blood and formalin-fixed paraffin-embedded slides of surgical specimens. BIM deletion was the most important independent prognostic factor in shorter PFS.

An anomalous unilateral single pulmonary vein associated with a bone morphogenetic protein receptor II gene mutation.

Anomalous unilateral single pulmonary vein (AUSPV), a rare congenital anomaly, is associated with an aberrant course but normal drainage, and resembles arteriovenous malformation (AVM). We treated a 26-year-old man with AUSPV in the right lung and an anomalous segmental pulmonary vein in the left lung. CT revealed a tortuous vascular shadow with an enhancement pattern identical to that of the pulmonary vein, suggesting AUSPV. This was confirmed by pulmonary angiography. Although pulmonary AVMs were not detected on angiography, microvascular AVMs could not be excluded because delayed bubbles appeared on contrast echocardiography. A genetic examination revealed a missense mutation of BMPR2.

EBUS-TBNA-related complications in a patient with tuberculous lymphadenopathy.

Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is becoming more common for diagnosing intrathoracic lymphadenopathy, including tuberculous lymphadenopathy. We herein report two cases of complications possibly related to EBUS-TBNA for tuberculous lymphadenopathy. The first patient was a 26-year-old woman who developed intrabronchial polypoid granulomas exclusively at puncture sites two months after undergoing EBUS-TBNA. Although endobronchial extension may occur, the risk of aggravation caused by puncture should be considered. The second patient was a 39-year-old woman with transient smear-positive bloody sputum that developed immediately after EBUS-TBNA and persisted for three days. Temporary isolation following EBUS-TBNA should be considered for possible tuberculous lymphadenopathy.

Therapeutic bronchoscopy followed by lobectomy for pulmonary sarcoma.

Malignant central airway obstruction is a life-threatening presentation requiring emergency palliative procedure. In selected patients, bronchoscopic intervention could be used as a bridge to curative resection. Here we report a 54-year-old male with pulmonary sarcoma of the right upper lobe, presenting with acute respiratory failure because of endobronchial extension. Emergency coring with the rigid bronchoscope and Dumon stent insertion stabilized the patient, and subsequent lobectomy resulted in occurrence-free survival over a 71-month follow-up.

Pathophysiological implication of reversed CT halo sign in invasive pulmonary mucormycosis: a rare case report.

BACKGROUND: It has been accepted that reversed halo sign (RHS) appeared on a computed tomography (CT) image in immunocompromised patients indicates an invasive fungal infection, but its pathophysiology remains obscure as to what this image implies. Therefore, the present report describes detailed radiological and histopathological findings of a case of invasive pulmonary mucormycosis (IPM) presenting RHS with comparison to those from a lesion of discrete nodule caused by invasive pulmonary aspergillosis (IPA), and discusses the pathophysiological implications of this characteristic image. CASE PRESENTATION: RHS had been clinically noted at the time of recovering of bone marrow function of a 64-year-old Japanese man who had chemotherapy for his acute lymphoblastic leukemia. Histological examination of the surgically removed lung revealed a lesion of IPM. This was composed of coagulation necrosis of septa at the center of lesion with preservation of air content which was encompassed outer rim comprising triplet structure; liquefaction, consolidation, and organization from the inner to the outer layer. In addition, Micro-CT examination confirmed reticular structure and monotonous high density at the central coagulation necrosis preserving air content and surrounding consolidation, and organization lesion of the IPM lesion. CONCLUSION: Our investigations suggest that RHS might be understood as a kind of immune reconstitution syndrome and be the initial and prior status of air crescent sign. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/3480054198968132.

Transbronchoscopic oxygen insufflation-induced barotrauma during endobronchial silicon spigot removal.

While transbronchoscopic air insufflation has been described in refractory atelectasis as a therapy without any serious complications, 3 cases of gastric rupture during the same procedure have been reported when it was used to support tracheal intubation by employing the jet of oxygen from the wall pipeline. Here, we report a 66-year-old woman who underwent transbronchoscopic oxygen insufflation using a flexible fiberscope to clear away secretions during an endobronchial silicon spigot removal procedure. She suffered a sudden drop of blood pressure with pneumomediastinum, subpleural and subcutaneous emphysema, and bilateral pneumothorax. Blood pressure recovered rapidly when we stopped the insufflation. Tube thoracostomy was initiated, and she recovered well without systemic air embolism. We conclude that transbronchoscopic oxygen insufflation using the wall pipeline does carry a potential risk of serious barotrauma, and is not to be recommended except with the use of a pressure monitor or pop-off valve.

Complete response of 7 years' duration after chemoradiotherapy followed by gefitinib in a patient with intramedullary spinal cord metastasis from lung adenocarcinoma.

Intramedullary spinal cord metastasis is a rare but serious complication which causes rapid progression of neurological deficits. Here we report a 35-year-old man presenting with increasing leg pain and gait disturbance, 8 months after surgery for lung adenocarcinoma. Spinal magnetic resonance imaging revealed an intramedullary tumor at the Th7/8 level. Radiotherapy at 35 Gy resulted in transient symptomatic improvement, but during chemotherapy with vinorelbine and cisplatin, symptoms worsened again. Gefitinib was then administered; the patient improved after 2 weeks and has now maintained a complete response for 7 years.

Simultaneous bilateral spontaneous pneumothorax with congenital pleuro-pleural communication.

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.

Haemothorax following bullectomy caused by a sharp edge of the Endoloop.

A 22-year old man presented with a massive haemothorax 25 days after bullectomy for a spontaneous pneumothorax. Thoracoscopic surgery revealed ongoing bleeding from the chest wall caused by a sharp edge of the Endoloop Ligature (Ethicon Endo-Surgery) used to resect the remaining small part of the lung at the earlier staple bullectomy. The point where bleeding was occurring was clipped and covered using a collagen patch coated with human fibrinogen and thrombin. The protruding sharp edge of the Endoloop was excised together with the surrounding lung tissue, using a stapler. Although prevention of this type of complication is difficult, awareness of the potential problem may help in managing such extremely rare events.

Video-assisted thoracoscopic surgery for ectopic middle mediastinal thymoma in a patient with myasthenia gravis.

We present a rare case of middle mediastinal thymoma with myasthenia gravis. A 51-year-old man presented with right ptosis and muscle weakness, and received a diagnosis of generalized myasthenia gravis. Computed tomography of the chest showed a 20-mm nodule in the middle mediastinum, suggesting a possible ectopic thymoma. He underwent video-assisted thoracoscopic extended thymectomy and resection of the tumor. Histologic examination revealed an ectopic thymoma and ectopic thymic tissue around the tumor. One year after the operation, his condition remains well controlled solely with tacrolimus. Careful preoperative radiologic examination concerning possible ectopic thymoma outside the dissection area of the extended thymectomy is recommended.

Ciliated muconodular papillary tumor of the lung: a newly defined low-grade malignant tumor with CT findings reminiscent of adenocarcinoma.

A ciliated muconodular papillary tumor has been reported to be a peripheral low-grade malignant tumor, consisting of ciliated columnar cells and goblet cells with basaloid cell proliferation. Although ciliated muconodular papillary tumors have not yet been classified according to the World Health Organization classification, they can pose diagnostic and therapeutic problems. Here we report a resected case of ciliated muconodular papillary tumor with computed tomography findings reminiscent of adenocarcinoma, showing a small irregular nodule adjacent to the intersegment pulmonary vein. There was no uptake of F-18 fluorodeoxyglucose positron emission tomography. The patient underwent surgical resection, and a lobectomy was performed because intraoperative needle biopsy suggested neoplastic proliferation. No EGFR mutations were detected. No recurrence was noted during 24-month follow-up after lobectomy.

Anterior mediastinal cystic seminoma.

Mediastinal cystic seminoma is uncommon; only 17 cases have been reported, most diagnosed postoperatively, without recurrence on follow-up, even without radiotherapy. Here, we report a mediastinal seminoma showing a unilocular cyst with enhancing thickened wall in computed tomography (CT) and septal structures in magnetic resonance imaging (MRI) in a 24-year-old man. Fluorodeoxyglucose (FDG) uptake was not significant. Histopathologically, seminoma cells were found scattered in the cyst wall. Twenty months after resection, the patient's quality of life is good, without chemotherapy or radiation. Cystic seminoma has a good prognosis and complete resection without adjuvant therapy might be sufficient for young patients of reproductive age.

Negative pressure pulmonary edema during tracheal Dumon stent implantation.

Negative pressure pulmonary edema (NPPE), or acute postobstructive pulmonary edema, is caused by negative intrathoracic pressure against an obstructed upper airway. Although NPPE is generally a benign condition, several fatal cases attributed to the delay in diagnosis have been reported. We report a case of NPPE experienced during Dumon stent placement. In a 39-year-old man, unilateral NPPE developed upon relief from a right main bronchial obstruction caused by a blood clot within the Dumon. The incident indicates that the "increased preload theory" is unable by itself to explain the pathophysiology of NPPE. Although we preferred spontaneous assisted ventilation for interventional rigid bronchoscopy, the potential risk of NPPE during Dumon stent placement, especially in spontaneously breathing young, healthy patients, needs to be considered.

A case of chronic expanding hematoma resulting in fatal hemoptysis.

An 80-year-old woman presented with a huge intrathoracic mass which had increased in size over 4 years. Computed tomography showed a thick calcified capsule and early-enhanced streaks inside the mass. Needle biopsy aspirated pure blood and fibrous connective tissue. F-18 fluorodeoxyglucose positron-emission tomography showed moderate FDG uptake at the periphery with central photon defects. Gallium-67 scintigraphy showed no abnormal uptake. On suspicion of chronic expanding hematoma, we recommended surgical resection, but the patient requested only follow-up. One year later, she was hospitalized with cardiac tamponade and subsequent massive hemoptysis. Repeated embolization was ineffective, and the patient soon succumbed.