Iraqi Postgraduate Medical Journal

Abstract

ABSTRACT:
Undifferentiated (embryonal) sarcoma of the liver
was first defined as a separate clinicopathological
entity in 1978. it is uncommon hepatic tumor of
mesenchymal origin, usually observed in children
and teenage, but also infrequently encounter in
adult. It represent about 9-15% of all hepatic tumor
in children. We report a case of undifferentiated
embryonal sarcoma of the liver in a 6 year old girl
who presented with non specific right
hypochondrial pain and mass with fever.
Laboratory studies of the liver function were
normal and the other tests were non specific.
Ultrasonography and CT scan showed a large
heterogeneous mass ranging from cystic tissue with
multiple septa to more predominant solid
component in the right lobe of the liver.
Exploratory laparotomy was performed and
revealed a large mass in the right lobe and part of
the left lobe of the liver. Tumor resection was
performed and about 70% of the total liver was
resected. Macroscopically, tumor was large
solitary globular firm predominant solid mass
weighing 1100gm and measuring 18 x 12 x 8 cm,
with variegated cut surface of solid, cystic,
necrotic and hemorrhagic areas. Microscopically,
the tumor has a variable but distinctive
sarcomatous appearance, composed of spindle,
oval and stellate-shaped sarcomatous cells, with
marked nuclear pleomorphism, closely packed in
whorls or scattered loosely in a myxoid ground
substance. Numerous bizarre multinucleated giant
cells, containing large intracytoplasmic hyaline
globules with ample mitosis many of which are
atypical. Tumor cells entrapped hyperplastic bile
ducts. Accordingly the diagnosis of