Extra-axial sacral soft tissue giant cell ependymoma affecting a child: Case report and review of the literature.

An otherwise healthy eight-year-old girl presented with a mass in the soft tissue of the sacral region. The lesion was diagnosed as a vascular malformation on imaging studies, for which percutaneous sclerotherapy was attempted. The mass continued to grow and a complete resection was performed after four years. The pathological diagnosis was giant cell ependymoma (GCE). GCE is a term used to describe a rare histologic variant of ependymoma characterized by malignancy-like morphologic phenotype and indolent behavior. To the best of our knowledge, this is the first case of extra-axial soft tissue sacral GCE reported in a child.

Prominent EMA 'dots' in tumour-induced Bergmann gliosis.

AIMS: To describe an unusual pattern of epithelial membrane antigen (EMA) immunoreactivity in highly proliferative human Bergmann glia. METHODS AND RESULTS: An immunohistochemical study was performed of postmortem cerebellar tissue from 18 adult patients with cerebellar damage of various aetiologies and 15 biopsies of diverse adult and paediatric cerebellar tumours. We observed marked proliferation of Bergmann glia with unusual prominent dot-like cytoplasmic EMA immunoreactivity in a case with extensive leptomeningeal sarcomatosis. Similar staining was not observed in association with other types of cerebellar pathology, except for other neoplastic conditions, such as leptomeningeal carcinomatosis, adult medulloblastoma, and pilocytic astrocytoma in children. At an ultrastructural level, the index case showed prominent endoplasmic reticulum with some intermediate filaments and lipofuscin granules, but no structures related to cilia or microvilli were observed. CONCLUSIONS: We consider that prominent EMA dots in Bergmann glia might represent excessive activation induced by an overlying leptomeningeal tumour that stimulates the expression of early developmental antigens. This observation suggests modulation of the glial phenotype when exposed to a neoplastic microenvironment that, in turn, might influence the regenerative potential of Bergmann glia.

[Incidence and characteristics of pancreatic cystic neoplasms]. / Incidencia y características de las neoplasias quísticas pancreáticas.

INTRODUCTION: Cystic neoplasms (CN) of the pancreas represent 10% of cystic lesions and 1% of pancreatic tumors. Mucinous cystic neoplasm (MCN), serous cystadenoma (SC) and intraductal papillary mucinous neoplasm (IPMN) are cystic neoplasms and represent more than 90% of these types of lesion. Few series have been published on these lesions, especially in Spain. AIM: To evaluate the incidence, characteristics and survival of patients with cystic neoplasms attended in our hospital in the last 12 years. PATIENTS AND METHOD: A retrospective analysis was carried out in all patients diagnosed with CN between January 1997 and December 2008. Diagnosis was made by abdominal computed tomography, pancreatic-magnetic resonance imaging and/or endoscopic ultrasonography. Sex, age, year of diagnosis, symptoms, tumoral location and size, type of surgery, pathology, and survival were evaluated. RESULTS: A total of 117 patients were analyzed. The mean age was 63±14 years and 56% were women. Eighty-eight patients had IPMN, 21 had SC and eight had MCN. Fifty-six per cent were diagnosed in the last 4 years, 42.7% were diagnosed as an incidental finding and 19% had a history of acute pancreatitis. The most frequent location was the pancreatic head (53%). The mean imaging size was 32mm. Surgical resection was performed in 69.2% of the patients. Twenty-three percent of the tumors were malignant, 30% were carcinoma in situ and 70% were invasive. Thirteen percent of the patients died; of these 93.3% had invasive carcinoma. Five-year survival was 94.7% in SC, 76% in IPMN and 60% in MCN. CONCLUSIONS: CN were mainly identified as incidental findings, although acute pancreatitis is another possible cause. The most frequent tumor in our environment is IPMN. Surgical treatment of IPMN and MCN, at the right moment, may be useful to prevent the development of pancreatic carcinoma.

Hepatic preconditioning after prolonged warm ischemia by means of S-adenosyl-L-methionine administration in pig liver transplantation from non-heart-beating donors.

BACKGROUND: This study ascertained the effect of S-adenosyl-L-methionine (SAMe) administration on the ischemia-reperfusion injury associated with pig liver transplantation from non-heart-beating donors (NHBDs) after prolonged warm ischemia. METHOD: Twenty-five animals underwent transplantation with an allograft from an NHBD. After donor cardiac arrest, cardiopulmonary bypass and normothermic recirculation (NR) were performed for 30 min. Ten animals were given SAMe during NR. Donors were cooled to 15 degrees C, and liver procurement was performed. RESULTS: SAMe reduced histologic liver damage 5 days after transplantation. The necrotic area affected 15.9%+/-14.5% of the liver biopsies in controls and 7.4%+/-9% in SAMe livers. Six of eight controls and only one of eight survivors in the SAMe group developed ischemic cholangitis. SAMe reduced apoptosis of hepatocytes 5 days after transplantation and apoptosis of sinusoidal endothelial cells at reperfusion and at 5 days. SAMe increased energy charge at the end of NR and favored the balance between adenosine and xanthine. It was also associated with higher portal blood flow (740+/-59.2 vs. 475.2+/-65.0 mL/min-1/m-2), hepatic hyaluronic acid extraction (132+/-72.2 vs. -205.8+/-64.6 microg/L), and lower levels of alpha-glutathione-S-transferase after reperfusion (2,601%+/-581% with respect to baseline vs. 6,488%+/-5,612%). CONCLUSION: SAMe administration during liver procurement from NHBDs prevents liver endothelial, parenchymal, and biliary tract damage. The protective role of SAMe may be partially mediated by the effect of adenosine during liver procurement.

Impact of adjuvant chemotherapy in the long-term outcome of patients with resected gastric cancer.

BACKGROUND AND OBJECTIVES: We analyzed in a retrospective analysis whether adjuvant chemotherapy with mitomycin (MMC) alone or with Tegafur (TG) is associated with long-term survival benefit in resected gastric cancer. Other prognostic factors are compared. METHODS: From 1977 to 1998, 314 consecutive totally resected gastric adenocarcinoma patients have been included in a survival study. In 151 patients no adjuvant therapy was given. In 163 patients, four courses of adjuvant chemotherapy was given, 109 of them with MMC, 10-20 mg/m(2) i.v. every 6 weeks and the other 54 with MMC plus TG, 500 mg/m(2) p.o. day for 42 consecutive days. Univariate and multivariate survival analyses were performed. RESULTS: Survival benefit was seen in patients who had received adjuvant chemotherapy compared with the controls (52% vs. 30% alive at the end of the study, relative risk = 0.46, 95% CI: 0.33-0.62, P < 0.0001), women (52% vs. 35%, RR = 0.83, 95% CI: 0.71-0.98, P = 0.0342) and increment of staging IB, II, IIIA, or IIIB (80, 56, 24 vs. 13%, respectively, RR = 1.83, 95% CI: 1.42-2.35, P < 0.0001). Better prognosis of node-negative patients was observed only in univariate analysis. CONCLUSIONS: After curative surgery, adjuvant chemotherapy with mitomycin C, both alone or combined with Tegafur, improved the long-term cure rate over no postoperative chemotherapy in locally advanced gastric cancer patients. This benefit was not influenced by other prognostic factors.