Childhood undifferentiated embryonal liver sarcoma: clinical features and immunohistochemistry analysis

Abstract

Purpose: The aim of the study was to report on 3 cases of childhood undifferentiated embryonal liver sarcoma (UELS) and to highlight the clinical features, laboratory findings, diagnosis, and management of this rare disease.

Methods: The patients' age, sex, clinical features, laboratory findings, pathologic results, and therapy were reviewed. Immunohistochemistry analysis was performed on the resected mass sections.

Results: In this study, 2 cases were female (aged 8 and 12 years) and 1 was male (aged 9 years). The causes of hospitalization were mainly abdominal pain, mass, or fever. An elevated erythrocyte sedimentation rate was noted in 2 available cases, and alpha fetoprotein (AFP) was within the normal range. Imaging findings indicated a well-defined heterogeneous large mass in the right lobe. Histopathologic evaluation of the mass confirmed the diagnosis of UELS. Immunohistochemical staining showed that vimentin and CD68 antigen were positive in all samples, whereas desmin was positive in one sample. Surgery with chemotherapy was performed in 2 cases.

Conclusion: The diagnosis of UELS depends mainly on the pathologic findings. Undifferentiated embryonal liver sarcoma should be included in the differential diagnosis of mass in the liver, especially with well-defined heterogeneous imaging findings and normal AFP. Diagnosis and management should be made early as UELS is a potentially treatable tumor.