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Gorham disease is a rare disorder with progressive osteolysis which leads to the vanishing of bones. Its etiology and ideal management strategy are still an enigma. A case of Gorham disease involving the maxillofacial region in a 25-year-old male with an emphasis on etiology and diagnosis is discussed.

Take home figureAdapted from Bärtsch and Gibbs2 Physiological response to hypoxia. Life-sustaining oxygen delivery, in spite of a reduction in the partial pressure of inhaled oxygen between 25% and 60% (respectively at 2500 m and 8000 m), is ensured by an increase in pulmonary ventilation, an increase in cardiac output by increasing heart rate, changes in vascular tone, as well as an increase in haemoglobin concentration. BP, blood pressure; HR, heart rate; PaCO2, partial pressure of arterial carbon dioxide.

Objectives: Over the past two decades, video-assisted thoracoscopic blebectomy and pleurodesis have been used as a safe and reliable option for treatment of spontaneous pneumothorax. The aim of this study is to evaluate the long-term outcome of video-assisted thoracoscopic surgery (VATS) treatment of spontaneous pneumothorax in young patients, and to identify risk factors for postoperative recurrence., Methods: We retrospectively analysed the outcome of VATS treatment of spontaneous pneumothorax in our institution in 150 consecutive young patients (age ≤ 40 years) in the years 1997-2010. Treatment consisted of stapling blebectomy and partial parietal pleurectomy. After excluding 16 patients lost to follow-up, in 134 cases [110 men, 24 women; mean age, 25 ± 7 standard deviation years; median follow-up, 79 months (range: 36-187 months)], we evaluated postoperative complications, focusing on pneumothorax recurrence, thoracic dysaesthesia and chronic chest pain. Risk factors for postoperative pneumothorax recurrence were analysed by logistic regression., Results: Of 134 treated patients, 3 (2.2%) required early reoperation (2 for bleeding; 1 for persistent air leaks). Postoperative (90-day) mortality was nil. Ipsilateral pneumothorax recurred in 8 cases (6.0%) [median time of recurrence, 43 months (range: 1-71 months)]. At univariate analysis, the recurrence rate was significantly higher in women (4/24) than in men (4/110; P = 0.026) and in patients with >7-day postoperative air leaks (P = 0.021). Multivariate analysis confirmed that pneumothorax recurrence correlated independently with prolonged air leaks (P = 0.037) and with female gender (P = 0.045). Chronic chest wall dysaesthesia was reported by 13 patients (9.7%). In 3 patients, (2.2%) chronic thoracic pain (analogical score >4) was recorded, but only 1 patient required analgesics more than once a month., Conclusions: VATS blebectomy and parietal pleurectomy is a safe procedure for treatment of spontaneous pneumothorax in young patients, with a 6% long-term recurrence rate in our experience. Postoperative recurrence significantly correlates with female gender and with prolonged air leakage after surgery., (© The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: Despite the best and most aggressive, often integrated, standard therapeutic approaches for mesothelioma, overall survival remains very poor. The actual failure points out clearly the need for the development of novel therapy. One of the promising paths of experimentation is artificial induction of apoptosis. A therapeutic strategy that relies on the down-regulation of BCL-XL inhibition nuclear factor kappaB (NF-kappaB) with a combination of SN38 and tumor necrosis factor (TNF) was studied in human mesothelioma cell lines (MSTO-221H, IST-MES1, IST-MES2, MPP89, H28, H513, H2052, and H290)., Methods and Results: Cell proliferation (clonogenic assay) was inhibited strongly by the combination of TNF and SN38. Examining the persistence of the NF-kappaB complexes using an electrophoretic mobility-shift assay, it appeared that they still were present at 24 hours in TNF-treated cells. In SN38-treated cells, NF-kappaB complexes persisted for 6 hours. In cells that were treated with combined SN38 and TNF, NF-kappaB complexes disappeared quickly and became undetectable at 6 hours. In flow cytometry analysis, only cells that were treated with combined SN38 and TNF demonstrated significant cellular accumulation in the sub-G0-G1 phase, suggesting a specific induction of apoptosis. Morphologic examination (4,6-diamidino-2-phenylindole staining and electron microscopy) and internucleosomal DNA fragmentation (gel ladder) confirmed rigorously the induction of apoptosis., Conclusions: Because of NF-kappaB inhibition with the combination of SN38 and TNF, the expression of BCL-XL (both the protein [Western blot analysis] and the mRNA [reverse transcriptase-polymerase chain reaction analysis]) was down-regulated, cytochrome c was released into the cytoplasm, caspase 3 was activated (Western blot analysis), and, consequently, apoptosis was triggered. The authors hope that the results of the current study may contribute to the design and implementation of a novel therapeutic approach that improves patients' responses to treatment for mesothelioma., (Copyright 2005 American Cancer Society.)

It is known that the potential clinical use of farnesyltransferase inhibitors (FTI) could be expanded to include cancers harboring activated receptor tyrosine kinases. Approximately 70% of malignant pleural mesotheliomas (MPM) overexpress epidermal growth factor receptors (EGFR) and a subset express both EGFR and transforming growth factor alpha (TGF-alpha), suggesting an autocrine role for EGFR in MPM. We checked on MPM cells (10 human cell lines, 11 primary cultures obtained by human biopsies, and 7 short-term normal mesothelial cell cultures) concerning the following: (a) the relative overexpression of EGFR (Western blotting, flow cytometry, immunohistochemistry), (b) the relative expression of EGFR ligands (EGF, amphiregulin, TGF-alpha, ELISA), (c) the relative increase of the activated form of Ras (Ras-bound GTP) after EGF stimulation (Ras activation assay), (d) the efficacy of five different FTIs (HDJ2 prenylation, cell cytotoxicity, and apoptosis using ApopTag and gel ladder). EGFR was overexpressed in MPM cells compared with normal pleural mesothelial cells in equivalent levels as in non-small cell lung cancer cells A459. MPM cells constitutively expressed EGFR ligands; however, Ras activation was attenuated at high EGF concentrations (100 ng/mL). Growth of MPM cells was substantially not affected by treatment with different FTIs (SCH66336, BMS-214662, R115777, RPR-115135, and Manumycin). Among these, BMS-214662 was the only one moderately active. BMS-214662 triggered apoptosis in a small fraction of cells (not higher than 30%) that was paralleled by a slight decrease in the levels of TGF-alpha secreted by treated MPM cells. Our data highlighted the concept that the same signaling pathway can be regulated in different ways and these regulations can differ between different cells of different origin.

Surgery is the only method of cure in lung cancer. Seldom its application with radical intent is possible. Despite the efforts aimed at integrating all the therapeutic strategies, the overall outcome of the management of this disease remains disappointing. For this reason, in the last three decades, thousands of preclinical and clinical attempts have been realised in order to investigate any possible way to cure this disease and significant steps forward have been made on the basis of the increasing "molecular knowledge" in the so called "post-genomic era". Particularly the impressive step forward in the biological characterization of cancer as a result of genetic/epigenetic multistep process has brought in a multitude of variables with staggering classification potentialities. "Benchside" and "bedside" scientists have assembled in functional teams to move the common efforts "translationally" to bridge basic and clinical research for a mutual synergistic enhancement. This paper represents the effort of a lung cancer focused translational research team made up of molecular biologists, medical oncologists and thoracic surgeons to achieve a comprehensive, but simple, review of the current status of the shift from cytotoxic to molecularly targeted therapy in lung cancer treatment potentially useful in the planning of translational research trials.

Background/aims: Sixteen patients with bacteriologically proven severe infected pancreatic necrosis (IPN) undergoing sequential surgical treatment were studied prospectively., Methodology: The severity of IPN was documented pre-operatively using the following scores: 1) degree of necrosis by CT scan [< 30% in three patients (19%); 30-50% in nine patients (56%); > 50% in four patients (25%)]; 2) Elebute and Stoner's sepsis score (16 +/- 4 points); 3) Goris' score of multiple organ failure (MOF) (5 +/- 2 points). Sequential surgical treatment was carried out by the same surgical team, as follows: 1) abdominal re-explorations through a zipper for the first 7-10 days; 2) open abdomen and repeated peritoneal debridements for the following 7-10 days; 3) continuous closed peritoneal lavage with multiple drainage, until resolution of infection (range: 15-85 days). No patient required further re-exploration., Results: Mortality occurred in 3/16 patients (19%), due to MOF in all 3 cases. The 13 survivors (81%) were discharged convalescent with closed abdominal wound, feeding orally, after 73 +/- 33 days, without fistulae. These results indicate that by treating severe IPN with the technique of sequential abdominal re-explorations, open drainage and continuous closed lavage, a low 19% mortality can be achieved., Conclusion: This study provides an assessment of the pre-operative severity of sepsis and of MOF in each patient with IPN: these data could facilitate future comparison of results obtained with other treatment modalities.

With the aim of preventing deep vein thrombosis after radical or modified mastectomy, 100 patients were randomly assigned to one of two different groups: the first group was treated with defibrotide (400 mg b.i.d. e.v.) starting from the day before the operation and continuing for the following seven days. The second group was given calcium heparin (5,000 IU b.i.d. by s.c. route) from day 0 to the 7th post-operative day. Neither side effects nor DVT or PE were observed. The quantity of fluids from the drainages rapidly decreased in both groups from the first day to the third one, while the quantity of blood cells was negligible starting from the second post-operative day. On this basis defibrotide may be considered an effective and well tolerated drug for the prevention of DVT.