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Birt–Hogg–Dube syndrome (BHD) is a rare condition which was first described in 1977. The incidence of BHD syndrome is unknown. However, approximately 600 families have been identified worldwide. The inheritance is autosomal dominant and caused by different mutations in the folliculin (FLCN) gene on chromosome 17 which encodes FLCN protein. These mutations are small insertion/deletions, splice-site, and nonsense mutations, which lead in most cases to premature truncation and loss of function of the folliculin protein. The condition is clinically characterized by diffuse pulmonary cysts which lead to spontaneous pneumothorax (SP), increased risk of renal cancer, and benign skin hamartomas mostly in the head and neck. However, the disease shows a considerable clinical heterogeneity. Pulmonary manifestations of BHD are being detected in more than 80% of the patients and occur at a median age of 30–40 years. Nevertheless, pulmonary function is generally normal or show only mild airway obstruction. Patients present typically with recurrent SP. In this review, we describe a female patient who presented with recurrent (SP). BHD was suspected and later on genetically confirmed. The patient was managed with VATS pleurectomy as definitive treatment of the pneumothorax.

Objective: The surgical resection of pulmonary metastases is associated with a survival benefit in selected patients. The use of laser devices for pulmonary metastasectomy (PM) is believed to facilitate the complete resection of metastases while preserving a maximum of healthy parenchyma. This is a prospective study to evaluate surgical outcome including the changes of lung function after laser–assisted surgery (LAS).Methods: A total of 77 operations in 61 patients in which PM was carried out in a curative intent were analyzed. A 1.320 nm diode-pumped Nd: YAG-Laser was used for resection of the metastases. Surgical and clinical data were collected using a standardized form and postoperative lung function changes 3 and 6 months after surgery were assessed using whole body plethysmography and diffusion capacity for carbon monoxide (DLCO). Size and distance of metastases to the pleural surface were measured radiologically.Results: A median of two metastases were resected per operation (range 1–13). The median duration of postoperative air leak was 1 day (range 0–11). LAS associated postoperative minor and major complications were observed in 4 (5%) cases and 1 (1%) case, respectively; there were no mortalities. The analysis of perioperative lung function showed that mean VC 3 months after surgery was reduced by 11 %, FEV1 by 11% and median DLCO by 11% (all p < 0.0001). There was almost no recovery of lung function between 3 and 6 months in the whole cohort. Patients with two or less metastases showed a recovery of lung function after 3 months regarding DLCO (p = 0.003). Decline of DLCO in the whole cohort correlated with the number of resected metastases at 3 months (r = 0.45, p = 0.006) and at 6 months (r = 0.42, p = 0.02) as well as depth of metastases in the parenchyma at 6 months (r = 0.48, p = 0.001).Conclusions: LAS is a safe and effective method for PM even for higher numbers of metastases, with short duration of postoperative air leak and little morbidity. Number and depth, but not size of metastases affect lung function changes after resection.

Thymic hyperplasia (TH) with lymphoepithelial sialadenitis (LESA)-like features (LESA-like TH) has been described as a tumor-like, benign proliferation of thymic epithelial cells and lymphoid follicles. We aimed to determine the frequency of lymphoma and autoimmunity in LESA-like TH and performed retrospective analysis of cases with LESA-like TH and/or thymic MALT-lymphoma. Among 36 patients (21 males) with LESA-like TH (age 52 years, 32&ndash, 80, lesion diameter 7.0 cm, 1&ndash, 14.5, median, range), five (14%) showed associated lymphomas, including four (11%) thymic MALT lymphomas and one (3%) diffuse large B-cell lymphoma. One additional case showed a clonal B-cell-receptor rearrangement without evidence of lymphoma. Twelve (33%) patients (7 women) suffered from partially overlapping autoimmune diseases: systemic lupus erythematosus (n = 4, 11%), rheumatoid arthritis (n = 3, 8%), myasthenia gravis (n = 2, 6%), asthma (n = 2, 6%), scleroderma, Sj&ouml, gren syndrome, pure red cell aplasia, Grave&rsquo, s disease and anti-IgLON5 syndrome (each n = 1, 3%). Among 11 primary thymic MALT lymphomas, remnants of LESA-like TH were found in two cases (18%). In summary, LESA-like TH shows a striking association with autoimmunity and predisposes to lymphomas. Thus, a hematologic and rheumatologic workup should become standard in patients diagnosed with LESA-like TH. Radiologists and clinicians should be aware of LESA-like TH as a differential diagnosis for mediastinal mass lesions in patients with autoimmune diseases.

Pulmonary metastasectomy (PM) is an accepted treatment modality in colorectal cancer (CRC) patients with pulmonary tumor spread. Positive intrathoracic lymph nodes at the time of PM are associated with a poor prognosis and 5-year survival rates of

Das therapeutische Vorgehen bei NSCLC und Infiltration der Vena cava superior (VCS) wird haufig kontrovers diskutiert. Der Ersatz oder die Rekonstruktion der VCS ist unter Einbeziehung multimodaler Therapieverfahren heute eine therapeutische, aber technisch anspruchsvolle Option. Unsere heutige Vorgehensweise, deren Anderung im Lauf der Zeit und die jeweiligen Ergebnisse werden prasentiert. Zwischen 1998 und 2013 erfolgte bei 17 Patienten ein alloplastischer Ersatz oder die Rekonstruktion (n = 5) der VCS bei NSCLC im Rahmen einer Lobektomie (n = 8) oder Pneumonektomie (n = 14). Eine adjuvante Strahlentherapie erhielten 16, eine induktive Chemotherapie 3 Patienten. Retrospektiv ausgewertet wurden der klinische Verlauf und das Uberleben. Therapierelevante postoperative Komplikationen traten bei 13 Patienten auf. Ein Verschluss des Interponats fand sich bei keinem Patienten. Die 30-Tage-Letalitat nach Pneumonektomie betrug 7 %, nach Lobektomie 0 %. Die Lokalrezidivrate betrug 4,5 %, das Auftreten von Fernmetastasen lag bei 54,5 % ( p = 0,0008). Die Uberlebenskurve fur das Gesamtkollektiv zeigte ein 1-Jahres-Uberleben von 63,6 %, ein 5-Jahres-Uberleben von 27,9 %. Patienten mit VCS-Rekonstruktion haben eine etwas bessere Prognose als Patienten mit VCS-Ersatz im 5-Jahres-Vergleich (33 % vs. 25 %, p = 0,22). Patienten mit einer Lobektomie zeigten ein 5-Jahres-Uberleben von 37,5 % (n = 8), nach Pneumonektomie lag dieses bei 21,4 % (n = 14, p = 0,18). Bei NSCLC und Infiltration der Vena cava superior kann man mit Resektion und adjuvanter Strahlentherapie eine gute lokale Kontrolle erzielen. Aufgrund der hohen Rate an Fernmetastasen sollten induktive und adjuvante chemotherapeutische Moglichkeiten sorgfaltig erwogen werden.

Background Vocal cord injuries, postoperative hoarseness, and sore throat are common complications after general anesthesia. One-lung ventilation can be achieved via two techniques: double-lumen endotracheal tube or endobronchial blocker such as the Arndt blocker. The current study was designed to assess the impact of these techniques for one-lung ventilation on the incidence and severity of postoperative hoarseness, vocal cord lesions, and sore throat. Methods In this prospective trial, 60 patients were randomly assigned to two groups. One-lung ventilation was achieved with either an endobronchial blocker (blocker group) or a double-lumen-tube (double-lumen group). Postoperative hoarseness and sore throat were assessed at 24, 48, and 72 h after surgery. Bronchial injuries and vocal cord lesions were examined by bronchoscopy immediately after surgery. Results In 56 included patients, postoperative hoarseness occurred significantly more frequently in the double-lumen group compared with the blocker group: 44% versus 17%, respectively (P = 0.046). Similar findings were observed for vocal cord lesions: 44% versus 17%, respectively (P = 0.046). The incidence of bronchial injuries was comparable between groups (P = 0.540). Cumulative number of days with hoarseness and sore throat were significantly increased in the double-lumen group compared with the blocker group (P < 0.01). No major complications such as bronchial ruptures were observed. Conclusions Clinicians should be aware of an increased incidence of minor airway injuries that may impair patient satisfaction when using a double-lumen tube instead of an endobronchial blocker for one-lung ventilation.

A cystic lesion in the lung is defined as a parenchymal region that has a well-defined wall and is filled with contents of variable density. This definition includes a variety of diseases, such as bronchogenic cyst, abscess formation, lymphangiomyomatosis, Langerhans cell histiocytosis, emph

BackgroundAdjuvant chemotherapy is beneficial in non-small-cell lung cancer (NSCLC). However, balancing toxicity and efficacy mandates improvement.Patients and methodsPatients with completely resected stages IB-pT3N1 NSCLC were randomly assigned to either four cycles cisplatin (C: 50 mg/m(2) day (d)1 + 8) and vinorelbine (V: 25 mg/m(2) d1, 8, 15, 22) q4 weeks or four cycles cisplatin (75 mg/m(2) d1) and pemetrexed (Px: 500 mg/m(2) d1) q3 weeks. Primary objective was the clinical feasibility rate (no grade (G)4 neutropenia/thrombocytopenia or thrombocytopenia with bleeding, no G3/4 febrile neutropenia or non-hematological toxicity; no premature withdrawal/death). Secondary objectives were drug delivery and efficacy.ResultsOne hundred and thirty two patients were randomized (stages: 38% IB, 10% IIA, 47% IIB, 5% pT3pN1; histology: 43% squamous, 57% non-squamous). The feasibility rates were 95.5% (cisplatin and pemetrexed, CPx) and 75.4% (cisplatin and vinorelbine, CVb) (P = 0.001); hematological G3/4 toxic effects were 10% (CPx) and 74% (CVb) (P

Donor lungs contain large amounts of passenger leukocytes which are transferred to the recipient by organ transplantation. In this study we have analysed the fate of these cells and have studied the population of donor leucocytes detectable in the blood circulation of ten lung transplanted patients during the first postoperative weeks. To this aim we have applied immunocytological as well as flow cytometric analyses using monoclonal antibodies against polymorphic HLA class I antigens that differed between donor and recipient as well as antibodies against cell differentiation markers. The results demonstrate that donor cells can be detected in the circulation of all lung transplanted patients but there is a considerable interindividual variability between 0.9% and 17.5% (mean 5.1%) on postoperative day 3. Cells were usually detectable for 2–4 weeks and had disappeared in all patients after 1 month. The circulating donor cells consisted exclusively of lymphocytes. T cells were the predominant population, most of which seemed to be CD45R0+, but B and NK (natural killer) cells were also present. Probably due to the small numbers of patients studied no correlation between clinical parameters and the extent of donor lymphocyte persistence; there were no clinical graft-versus-host reactions. The findings demonstrate the regular existence of a transient (macro)chimerism due to passenger lymphocytes in the early phase after lung transplantation. The immunological function and the relation between this phenomenon and the long-term microchimerism which frequently develops after solid organ transplantation remain unclear.

7002 Background: Adjuvant chemotherapy is beneficial in early stage NSCLC, but toxicity and dose delivery are an issue in many patients. Therapy with CPx showed clear activity and good tolerability...