Perivascular epitheloid cell tumour (PEComa) of the retroperitoneum – a rare tumor with uncertain malignant behaviour: a case report

<p>Abstract</p> <p>Introduction</p> <p>Perivascular epitheloid cell tumours are rare mesenchymal neoplasms characterized by a proliferation of perivascular cells with an epitheloid phenotype and expression of myomelanocytic markers.</p> <p>Case presentation</p> <p>Here we present the case of a cystic perivascular epitheloid cell tumour of the retroperitoneum associated with multifocal lung lesions. A 27-year-old woman underwent laparotomy to remove a 10 × 6 × 4 cm sized retroperitoneal mass. The resected specimen was subjected to frozen and permanent histological sections with conventional and immunohistochemical stains, including antibodies against HMB45. The tumour displayed the typical morphological and immunohistochemical features of a perivascular epitheloid cell tumour. Focal necrosis and a proliferative index of 10% suggested a malignant potential. Moreover, postoperative computed tomography scans demonstrated multiple lung lesions, which were radiologically interpreted as being most likely compatible with lymphangioleiomyomatosis.</p> <p>Conclusion</p> <p>Since lymphangioleiomyomatosis, an otherwise benign condition, belongs to the family of perivascular epitheloid cell tumours, it cannot be excluded that the lung lesions in this case in fact represent metastases from the retroperitoneal perivascular epitheloid cell tumour rather than independent neoplasms. More experience with this new and unusual tumour entity is clearly needed in order to define reliable criteria for benign or malignant behaviour.</p

Consequences of Cold-Ischemia Time on Primary Nonfunction and Patient and Graft Survival in Liver Transplantation: A Meta-Analysis

Introduction: The ability to preserve organs prior to transplant is essential to the organ allocation process. Objective: The purpose of this study is to describe the functional relationship between cold-ischemia time (CIT) and primary nonfunction (PNF), patient and graft survival in liver transplant. Methods: To identify relevant articles Medline, EMBASE and the Cochrane database, including the non-English literature identified in these databases, was searched from 1966 to April 2008. Two independent reviewers screened and extracted the data. CIT was analyzed both as a continuous variable and stratified by clinically relevant intervals. Nondichotomous variables were weighted by sample size. Percent variables were weighted by the inverse of the binomial variance. Results: Twenty-six studies met criteria. Functionally, PNF%=-6.678281+0.9134701*CIT Mean+0.1250879*(CIT Mean-9.89535) 2 - 0.0067663*(CIT Mean-9.89535) 3, r2=.625, p<.0001. Mean patient survival: 93 % (1 month), 88 % (3 months), 83 % (6 months) and 83 % (12 months). Mean graft survival: 85.9 % (1 month), 80.5 % (3 months), 78.1 % (6 months) and 76.8 % (12 months). Maximum patient and graft survival occurred with CITs between 7.5-12.5 hrs at each survival interval. PNF was also significantly correlated with ICU time, % first time grafts and % immunologic mismatches. Conclusion: The results of this work imply that CIT may be the most important pre-transplant information needed in the decision to accept an organ. © 2008 Stahl et al

Caustic esophagitis in children

La prise en charge des enfants souffrant d'oesophagite caustique reste un sujet de discussion en raison du manque d'études prospectives. L'approche diagnostique et thérapeutique est fondée sur l'expérience clinique individuelle. L'analyse rétrospective de 73 dossiers d'enfants avec commémoratif d'ingestion de caustique a permis l'analyse des résultats de l'endoscopie du tractus digestif supérieur. Les lésions de degré IV s'observent uniquement après ingestion de silicates, de bases, d'acides organiques et d'acides. Les mesures thérapeutiques relatives aux sténoses oesophagiennes concernent uniquement les enfants qui ont absorbé des bases et des acides forts. L'ensemble de ces données impose de façon formelle l'endoscopie diagnostique chez un enfant présentant des symptômes cliniques et/ou une anamnèse d'ingestion de bases ou d'acides forts. Une endoscopie de contrôle est souhaitable après trois à quatre semaines. Les sténoses simples peuvent être traitées par dilatations; en revanche, les sténoses étendues ou multiples doivent benéficier d'une aesophagectomie. Le rôle des stéroïdes au cours de la phase initiale n'est pas clairement établi; toutefois, l'antibiothérapie demeure essentiell