Undifferentiated embryonal sarcoma of the liver: a case report of a rare case in an adult patient.

Undifferentiated embryonal sarcoma of the liver is a rare primary mesenchymal hepatic tumor that usually occurs in pediatric patients. In adulthood, this aggressive neoplasm represents only 7% of the liver sarcomas. This case reports a liver sarcoma occurring in a 49-year-old female patient. The patient was admitted in the emergency room with abdominal pain. Computerized tomography scan and magnetic resonance imaging showed a giant lobulated cystic mass in the right hepatic lobe, suggesting an atypical hemangioma. Right hepatectomy was performed. This rare case promotes a review of the differential diagnosis of liver primary neoplasms including sarcoma. The histological examination revealed an undifferentiated embryonal sarcoma. The patient underwent adjuvant chemotherapy. Currently, our patient is in complete sustained remission 4 years after chemotherapy.

Diaphragmatic hernia as a rare cause of bowel obstruction.

Mechanical intestinal obstruction is a common cause of acute abdominal pain that brings patients to the emergency department. One of the main causes is adhesion in the abdomen after abdominal surgery, but rarer causes exist and are a diagnostic challenge due to the similarity of the presenting symptoms. Here, we present a case of intestinal obstruction caused by diaphragmatic hernia.

Duodenal neuroendocrine tumour in a young patient with von Recklinghausen disease.

Von Recklinghausen disease (neurofibromatosis type 1-NFT1) is a genetic disorder with autosomal dominant inheritance pattern, caused by mutation of a tumour suppressor gene. Its main features include multiple cutaneous café-au-lait spots and neurofibromas. It is associated with an increased risk of developing neuroendocrine tumours, for instance, in the duodenum. The authors present a case of a 23-year-old male patient admitted to the emergency department due to persistent vomiting. Imaging and biopsy studies revealed an obstructive and large duodenal neuroendocrine tumour; hence the patient underwent a pancreaticoduodenectomy.

Boerhaave's syndrome: A case report of damage control approach.

INTRODUCTION: Boerhaave's syndrome is a life-threatening oesophageal perforation that carries a high mortality rate (20-50%). Diagnosis is difficult by its rarity and the absence of typical symptoms. Treatment of this condition usually requires surgical intervention. PRESENTATION OF CASE: We report the case of a 77-year-old man that resorted to the emergency room with dyspnoea and thoracic pain after vomiting. CT scan revealed pneumomediastinum, left collapse lung and loculated pleural effusion. A left intercostal chest tube was inserted with food drainage. Hence, Boerhaave's syndrome was suspected. Thoracotomy with mediastinum debridement, pleural drainage and oesophageal T-tube drainage was performed. Patient was admitted on the Intensive Care Unit with septic shock, with need for ventilatory support and vasopressor therapy. Two days later, a second look thoracotomy was done with definitive oesophageal repair and pleural patch. The post-operative course was complicated by pneumonia and stroke. Patient was discharged home on the 38th day and remains well at 3 month of follow-up. DISCUSSION: Delayed diagnosis and treatment are the principal causes of high mortality in Boerhaave's syndrome. The classic Mackler's triad (vomiting, lower thoracic pain and subcutaneous emphysema) is present in less then 50% of cases. A thoracic drainage may be useful to confirm diagnosis promptly. There is no standard treatment option. In this case report, the authors used a damage control approach to control sepsis, allowing for a delayed definitive oesophageal repair. CONCLUSION: Prompt diagnosis with thoracic drainage and a damage control treatment plan might lead to good prognosis for patients with this rare and potentially fatal condition.

There is no difference in nitric oxide metabolites and neonatal outcome between premature infants born to pre-eclamptic and those born to normotensive women.

BACKGROUND: The pathophysiology of pre-eclampsia (PE) is complex, and nitric oxide (NO) may be a factor. The neonatal outcome in pregnancies complicated by PE is controversial, and the PE/NO/neonatal diseases relationship has not been well established. AIMS: To measure nitrate and nitrite levels in the placenta, umbilical cord blood, blood and urine of preterm neonates born to pre-eclamptic and normotensive women and to investigate the relationship between placental, fetal and neonatal NO metabolites and neonatal outcome. METHODS: A prospective study was undertaken of 30 preterm infants <34 weeks of gestation, born to pre-eclamptic mothers and matched by gestational age with 30 infants born to normotensive mothers. Samples from the placental tissue, venous cord blood and the newborns' blood on day 4 and urine on days 1 and 4 were assayed for NO metabolites (nitrate and nitrite). Clinical variables and NO metabolites were compared between the groups. Generalised linear models were fitted to associate NO metabolites levels with adverse neonatal outcomes. RESULTS: There were no differences in NO metabolites and neonatal outcomes between the two groups. Increased levels of NO metabolites were found in the placenta and cord blood of small-for-gestational-age infants, and in the cord blood of newborns with necrotising enterocolitis and those who died. CONCLUSION: NO metabolite levels in the placenta and fetal and neonatal circulation were not associated with PE; however, cord blood levels of NO metabolites differed according to fetal growth and neonatal outcome.