Persistent fifth arch anomalies – broadening the spectrum to include a variation of double aortic arch vascular ring

Abstract

Fifth arch anomalies are rare and complex and frequently misdiagnosed or mistaken for other entities. We report a double arch vascular ring that is thought to consist of right fourth arch and left fifth arch components, a previously undescribed persistent fifth arch variant. The currently recognized spectrum and classification of fifth arch vascular anomalies are expanded along with illustrative images to justify the proposed changes. Reviewing and expanding the classification of fifth arch anomalies to include a double arch ring variant will promote recognition, correct diagnosis and appropriate management of these anomalies.

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Hermes

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Fat-containing soft-tissue masses in children

Abstract

The diagnosis of soft-tissue masses in children can be difficult because of the frequently nonspecific clinical and imaging characteristics of these lesions. However key findings on imaging can aid in diagnosis. The identification of macroscopic fat within a soft-tissue mass narrows the differential diagnosis considerably and suggests a high likelihood of a benign etiology in children. Fat can be difficult to detect with sonography because of the variable appearance of fat using this modality. Fat is easier to recognize using MRI, particularly with the aid of fat-suppression techniques. Although a large portion of fat-containing masses in children are adipocytic tumors, a variety of other tumors and mass-like conditions that contain fat should be considered by the radiologist confronted with a fat-containing mass in a child. In this article we review the sonographic and MRI findings in the most relevant fat-containing soft-tissue masses in the pediatric age group, including adipocytic tumors (lipoma, angiolipoma, lipomatosis, lipoblastoma, lipomatosis of nerve, and liposarcoma); fibroblastic/myofibroblastic tumors (fibrous hamartoma of infancy and lipofibromatosis); vascular anomalies (involuting hemangioma, intramuscular capillary hemangioma, phosphate and tensin homologue (PTEN) hamartoma of soft tissue, fibro-adipose vascular anomaly), and other miscellaneous entities, such as fat necrosis and epigastric hernia.

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Transanal Total Mesorectal Excision (TaTME) for Rectal Cancer: Step by step description of the surgical technique for a two-teams approach

Publication date: Available online 20 November 2016
Source:European Journal of Surgical Oncology (EJSO)
Author(s): María Clara Arroyave, F. Borja DeLacy, Antonio M. Lacy
Interest in transanal total mesorectal excision (taTME) is growing worldwide due to the application of minimally invasive techniques to rectal cancer surgery while maintaining adequate oncologic outcomes.This article presents the standardised and refined technique after performing more than 300 operations at Hospital Clinic of Barcelona.



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Mentored experience of establishing a national peritoneal malignancy programme – experience of first 50 operative cases

Publication date: Available online 19 November 2016
Source:European Journal of Surgical Oncology (EJSO)
Author(s): K.H. Chang, M. Kazanowski, O. Staunton, R.A. Cahil, B.J. Moran, C. Shields, J. Mulsow
BackgroundCytoreductive Surgery (CRS) and Hyperthermic Intraperitoneal Chemotherapy (HIPEC) are considered standard of care for pseudomyxoma peritonei (PMP) and selected patients with colorectal peritoneal metastases (CPM) or peritoneal mesothelioma. A National Peritoneal Malignancy programme was established in Ireland (population of 4.5 million) in May 2013 with mentoring and support from the Peritoneal Malignancy Institute, Basingstoke UK. This study reviews the operative and oncological outcomes for the first 50 patients who underwent CRS and HIPEC in Ireland.MethodsThis is a retrospective review of all patients referred, and of the subset who underwent CRS and HIPEC, for peritoneal malignancy in Ireland between May 2013 and November 2015.ResultsDuring the study period, 130 patients were referred and 50 patients were selected for CRS and HIPEC. Three patients were found to have unresectable disease at laparotomy. Of the remaining 47 patients, eight had major tumour debulking. In total, 39 underwent complete cytoreduction and 45 received HIPEC. After a median follow up of 12.7 months, 12 patients had developed further metastatic disease. The rates of complete cytoreduction, complication and operative mortality were 83%, 0% and 0% respectively. There were no major Clavien-Dindo grade III/IV morbidity.ConclusionsWe report the successful establishment of a national peritoneal malignancy programme. Mentoring from an experienced centre may have shortened the known learning curve evident by our encouraging outcomes. The follow-up period is short, however our early results are comparable with internationally reported figures.



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Vascular reconstruction after retroperitoneal and lower extremity sarcoma resection

Publication date: Available online 20 November 2016
Source:European Journal of Surgical Oncology (EJSO)
Author(s): M. Wortmann, I. Alldinger, D. Böckler, A. Ulrich, A. Hyhlik-Dürr
PurposeSoft tissue sarcomas (STS) of the retroperitoneum and the lower limb with invasion of major blood vessels are very rare malignancies. This study analyses the outcome of patients with vascular replacement during resection of STS of the retroperitoneum and the lower extremity with either arterial or concomitant arterial and venous infiltration.MethodsPatients with vascular replacement during resection of sarcoma of the retroperitoneum and the lower extremity between 1990 and 2014 were included in this retrospective single center study. Patients with a sole infiltration of a major vein were excluded. The follow up was obtained from medical records, the general practitioner and a clinical examination whenever possible. The main endpoints were survival, graft patency and the rate of major amputations.Results47 patients were included in this study. 20 patients have received an operation for a retroperitoneal STS, 27 for a STS of the lower extremity. The median follow-up was 24.5 months. The median survival was 113 months with a median tumor-free survival of 25 months. The two-year patency for arterial bypasses in the retroperitoneum and the lower extremity was 88 % and 72 %, respectively. Limb salvage rate was 89 %.ConclusionsInvasion of major blood vessels is no contraindication for a resection of a STS in the retroperitoneum and the lower extremity, but it is accompanied by a high postoperative morbidity. Since surgical resection is the only curative therapy in these patients, it should also be offered to patients with infiltration of major blood vessels.



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Elevated regulatory T cells, surface and intracellular CTLA-4 expression and interleukin-17 in the lung cancer microenvironment in humans

Abstract

Regulatory T cells (Tregs) play an important role in the suppression of the immune response in lung cancer. Cytotoxic T-lymphocyte antigen 4 (CTLA-4) expressed on T lymphocytes is capable of downregulating cytotoxic T cells and is constitutively expressed on Tregs. Little is known about the population of Tregs with two forms of CTLA-4: surface (s) and intracellular (in) in the lung cancer environment. Th17 cells defined by production of IL-17 have pleiotropic functions in anticancer immune response. Our aim was to detect the elements of immune response regulation in lung cancer in three compartments: by analysis of bronchoalveolar lavage fluid (BALF) from the lung affected by cancer (clBALF), healthy symmetrical lung (hlBALF) and peripheral blood (PB) from the same patient. A total of 54 samples were collected. Tregs, (s)CTLA-4, (in)CTLA-4 were detected by flow cytometry with antibodies against CD4, CD25, Foxp3, CD127, CTLA-4, and concentration of IL-17 was estimated by ELISA. We observed a significantly higher proportion of Tregs in clBALF than in hlBALF or PB (8.5 vs. 5.0 vs. 5.1%, respectively, p < 0.05). The median proportion of (in)CTLA-4+ Tregs was higher in clBALF than in hlBALF or PB (89.0, 81.5, 56.0%, p < 0.05). IL-17 concentration was the highest in clBALF—6.6 pg/ml. We observed a significant correlation between the proportion of Tregs and (in)CTLA-4+ Tregs with IL-17A concentration in clBALF. We confirmed significant differences in the proportion of regulatory elements between cancerous lung and healthy lung and PB and the usefulness of BALF analysis in evaluation of immune response regulation in local lung cancer environment.

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