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A 10-year-old boy presented to the emergency department with fever, abdominal pain, and vomiting that had lasted for over a week. Physical examination revealed a palpable mass in the right upper quadrant. Sonographic examination revealed a large heterogeneous mass, predominantly solid, hyperechogenic and with sparse cystic areas, located in the right hepatic lobe (Fig. 1A), which we suspect to be a neoplastic liver lesion. A subsequent laboratory panel revealed mild inflammation (high C-reactive protein levels, 47.2 mg/dL), discrete elevation of liver function tests (aspartate aminotransferase, 53 U/L; alanine aminotransferase, 49 U/L; and gamma-glutamyl transferase, 52 U/L), and elevated lactate dehydrogenase levels (619 U/L). However, alpha-fetoprotein levels (AFP) were normal (<130 ng/mL). Computed tomography (CT) revealed a large heterogeneous mass, predominantly hypodense with a cystic-like appearance, and multiple septations and internal serpiginous structures in the right hepatic lobe (Fig. 1B and C). The mass showed hyperdense central foci, probably corresponding to areas of hemorrhage. Considering the discrepancy between the predominantly solid appearance on ultrasound and the cystic-like appearance on CT, a diagnosis of undifferentiated embryonal sarcoma of the liver (UESL) was initially proposed. Consequently, the patient was immediately transferred to an oncologic hospital where he underwent a percutaneous biopsy that confirmed this diagnosis. Before undergoing right hepatectomy, the patient received neoadjuvant chemotherapy with a VAC regimen (vincristine, dactinomycin, and cyclophosphamide) for 5 months. Since then, he has been undergoing adjuvant chemotherapy with the same regimen while showing good response and tolerability.
Figure 1(A) Ultrasound showing a large solid and heterogeneous lesion in the right hepatic lobe that is predominantly hyperechoic (star) and with sparse cystic areas (arrows). (B) Axial unenhanced computed tomography (CT) of the abdomen revealing a large and mostly hypodense lesion on the right hepatic lobe (star) that contains spontaneous hyperdense foci (circle), possibly corresponding to the hemorrhage areas. (C) Axial contrast-enhanced CT images showing that the lesion has cystic appearance (star) with internal septa and filamentous vascular structures (arrows).
UESL, a rare yet highly aggressive mesenchymal tumor, is the third most common hepatic malignant tumor in children after hepatoblastoma and hepatocellular carcinoma.
The discrepancy between the ultrasound and CT appearance is a characteristic finding, with the majority of cases exhibiting a predominantly solid appearance on ultrasound and cystic-like appearance on CT.
Chung EM, Lattin Jr GE, Cube R, Lewis RB, Marichal-Hernández C, Shawhan R, et al., From the archives of the AFIP: pediatric liver masses: radiologic-pathologic correlation. Part 2. Malignant tumors, Radiographics, 31, 2011, 483–507.
Chung EM, Lattin Jr GE, Cube R, Lewis RB, Marichal-Hernández C, Shawhan R, et al., From the archives of the AFIP: pediatric liver masses: radiologic-pathologic correlation. Part 2. Malignant tumors, Radiographics, 31, 2011, 483–507.
Chung EM, Lattin Jr GE, Cube R, Lewis RB, Marichal-Hernández C, Shawhan R, et al., From the archives of the AFIP: pediatric liver masses: radiologic-pathologic correlation. Part 2. Malignant tumors, Radiographics, 31, 2011, 483–507.
CT findings in other studies have demonstrated that UESL presents as a large solitary mass that is predominantly hypodense with a few internal septations,
Chung EM, Lattin Jr GE, Cube R, Lewis RB, Marichal-Hernández C, Shawhan R, et al., From the archives of the AFIP: pediatric liver masses: radiologic-pathologic correlation. Part 2. Malignant tumors, Radiographics, 31, 2011, 483–507.
The primary differential diagnoses include mesenchymal hamartomas of the liver, abscesses, hydatid cysts, cystic degeneration in hepatoblastomas, hepatocellular carcinomas, and cystic metastases.
Chung EM, Lattin Jr GE, Cube R, Lewis RB, Marichal-Hernández C, Shawhan R, et al., From the archives of the AFIP: pediatric liver masses: radiologic-pathologic correlation. Part 2. Malignant tumors, Radiographics, 31, 2011, 483–507.
Although the diagnosis of UESL is indeed challenging, knowledge regarding this discrepancy in imaging findings can help establish an appropriate diagnosis.
Chung EM, Lattin Jr GE, Cube R, Lewis RB, Marichal-Hernández C, Shawhan R, et al., From the archives of the AFIP: pediatric liver masses: radiologic-pathologic correlation. Part 2. Malignant tumors, Radiographics, 31, 2011, 483–507.
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