An undifferentiated sarcoma of the liver in one sibling

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Klin Onkol 1997; 10(5): 152-154.

Undifferentiated (embryonal) liver sarcoma is a rare, highly malignant tumor occuring in older children and younger adults. The case report of a 17-years old boy is presented, who had not been seriously ill since his birth till the 17th year of his life (except small accidents). He comes from uniovular twins. His brother also didn´t suffer from serious diseases in his childhood. He was admitted to the Department of Surgery Regional Institution of National Health in a febrile state with abdominal pain. An evident focus in the right (dextral) liver lobe with unhomogenous structure has been diagnosed by USG (Ultrasonic examination). He was transfered to the First Surgical Clinic in Košice, where CT and punctural aspirational biopsy has been made. He has been operated on - (dextral hemihepatectomy). The weight of preparation was 2000g, the tumor was capsuled 20cm in diameter, and it was bloody and friable in some places. Definitive histological examination has shown signs of undifferentiated embryonal sarcoma. The postoperative course has been without complications. Chemotherapy has been applicated. At present, he is 5 years after operation and he is without any difficulties. His brother has been ewamined, too. He doesn´t have symptoms of similar disease.