Undifferentiated embryonal sarcoma of the liver: Clinical characteristics and outcomes

Pediatric Primary Hepatic Tumors: Diagnostic Considerations

“…The presentation is nonspecific, with abdominal pain, hepatomegaly, nausea, anorexia, and fever, and is rarely associated with jaundice [ 30 , 159 , 160 , 161 , 162 , 163 ]. The majority of patients who present are asymptomatic; however, patients may present acutely due to tumor rupture and tumor wall dehiscence secondary to rapid growth [ 163 , 164 , 165 ]. Metastatic disease, commonly to the lungs, peritoneum, and pleura, is reported in approximately 5–13% of patients, although it has been rarely reported to be present at the time of diagnosis [ 160 , 162 , 166 ].…”

“…Patients may present with mild leukocytosis or leukopenia, anemia, hypoalbuminemia, mildly elevated transaminases, and inflammatory markers [ 160 , 163 , 167 ]. AFP and carcinoembryonic antigen are typically normal, although cases with elevated levels have been reported [ 159 , 160 , 161 , 163 , 164 , 165 , 168 ]. Interestingly, Letherer et al reported a case of a UESL in a 15-year-old male that caused a false positive ELISA result for Echinococcus , Entameoba histolytica , and histoplasmosis secondary to molecular mimicry [ 168 ].…”

“…Interestingly, Letherer et al reported a case of a UESL in a 15-year-old male that caused a false positive ELISA result for Echinococcus , Entameoba histolytica , and histoplasmosis secondary to molecular mimicry [ 168 ]. Zhang et al reported a case of a nine-year-old female with elevated IgE and transient peripheral eosinophilia, initially misdiagnosed as Echinococcus [ 165 ]. Paraneoplastic syndromes and erythropoeitin-secreting capacity have been reported in rare adult cases [ 169 , 170 ].…”

“…Cytology is notable for medium to large spindle or stellate-shaped cells with ill-defined borders and inconspicuous nucleoli within a myxoid matrix or fibrous stroma [ 30 , 160 , 164 ]. Multinucleated giant cells, bizarre cells with aberrant nuclei, and eosinophilic hyaline globules (which are PAS-positive) may also be identified, which are important diagnostic clues [ 30 , 160 , 163 , 164 , 165 ]. At the periphery of the tumor, trapped hepatocytes and bile duct cells may be visible [ 160 , 165 ].…”

“…Multinucleated giant cells, bizarre cells with aberrant nuclei, and eosinophilic hyaline globules (which are PAS-positive) may also be identified, which are important diagnostic clues [ 30 , 160 , 163 , 164 , 165 ]. At the periphery of the tumor, trapped hepatocytes and bile duct cells may be visible [ 160 , 165 ].…”