PENTALOGY OF CANTRELL IN TWINS: A CASE REPORT.

UNLABELLED: INTRODUCTION. Pentalogy of Cantrell is a very rare congenital multiple malformation with the poor outcome. Syndrome included the following pentad: abdominal wall defect, a defect of the lower sternum, anterior diaphragm defect, ectopia cordis, as well as a variety of intracardiac anomalies. CASE REPORT: In the fifteenth week of the twin pregnancy, ultrasound examination showed multiple anomalies in one, as well as the absence of the nose apex in the other twin. The autopsy of the fetuses was performed after the abortion had been approved by the Ethics Committee. The external examination of the fetus number one showed ectopic heart and omphalocele. The internal examination revealed the existence of sternum cleft, ectopic heart and part of the left lung outside the thoracic cavity, the presence of diaphragmatic hernia with protrusion of abdominal organs, heart with only one large vessel and the horseshoe kidney. The full expression of the Pentalogy of Cantrell was confirmed. The external and internal examination of the other twin showed just agenesis of the nose apex. CONCLUSION: Recognition and diagnosis of the syndrome is of the exceptional importance. Proper and timely diagnosis should contribute to good outcome of the pregnancy and delivery.

Mesenchymal hamartoma of the liver in adults--case report.

INTRODUCTION: Mesenchymal hamartoma of the liver is a benign lesion presenting as an enlarging abdominal mass in children less than 2 years of age. Fewer than 5% cases are present in individuals over 5 years of age, and this lesion is extremely rare in adults. It may affect the left or the right lobe of liver as a cystic or solid mass or both components may be present. The pathogenesis remains incompletely understood, but these lesions have generally been considered to represent a development abnormality in the bile duct plate formation. CASE REPORT: In this report, we present a case of a 44-year-old man who was surgically treated at the Department of Abdominal, Endocrine and Transplantation Surgery of the Clinical Center of Vojvodina due to cystic lesion in the liver segment IV that had been verified by computed tomography imaging diagnostics. The patient was sent from a smaller health center with the diagnosis of echinococcosis. After the adequate preparation of the patient, surgical excision ofthe liver cystic lesion was done. Once a thorough histological examination had been performed, the diagnosis of mesenchymal hamartoma was made. CONCLUSION: Mesenchymal hamartoma of the liver is a benign tumor resulting from abnormal, intra-uterine development of bile ducts and has a delayed clinical manifestation, thus this lesion appears to be related to the processes of maturation. It is potentially pre-malignant lesion presenting as a solid and/or cystic neoplasm. Symptoms, laboratory results and radiographic imaging are nonspecific and inconclusive, so surgical excision of the whole lesion is the imperative for the definitive diagnosis.