Undifferentiated (embryonal) sarcoma of the liver: report of 31 cases.
Thirty-one cases of undifferentiated (embryonal) sarcoma of the liver are presented. The tumor is found predominantly in the pediatric age group, the majority of patients (51.6%) being between 6 and 10 years of age. An abdominal mass and pain are the usual presenting symptoms. Radiographic examination is nonspecific except to demonstrate a space-occupying lesion of the liver. The tumors are large, single, usually globular and well demarcated, and have multiple cystic areas of hemorrhage, necrosis, and gelatinous degeneration. Histologic examination shows a pseudocapsule partially separating the normal liver from undifferentiated sarcomatous cells that, near the periphery of the tumor, surround entrapped hyperplastic or degenerating bile duct-like structures. Eosinophilic globules that are PAS positive are usually found within and adjacent to tumor cells. Areas of necrosis and hemorrhage are prominent. The prognosis is poor, with a median survival of less than 1 year following diagnosis.
D000293:Adolescent; D000328:Adult; D001650:Bile Duct Neoplasms / Q000175:diagnosis; D001653:Bile Ducts, Intrahepatic / Q000473:pathology; D006528:Carcinoma, Hepatocellular / Q000175:diagnosis; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008113:Liver Neoplasms / Q000175:diagnosis / Q000473:pathology*; D008297:Male; D008637:Mesenchymoma / Q000175:diagnosis / Q000473:pathology*; D009362:Neoplasm Metastasis / Q000473:pathology; D009940:Organoids / Q000473:pathology; D011379:Prognosis; D012208:Rhabdomyosarcoma / Q000175:diagnosis