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Case Reports
. 2010 Oct 7;16(37):4725-32.
doi: 10.3748/wjg.v16.i37.4725.

Undifferentiated liver embryonal sarcoma in adults: a report of four cases and literature review

Xiao-Wei Li  1 , Shao-Juan Gong, Wei-Hua Song, Jun-Jun Zhu, Chun-Hua Pan, Meng-Chao Wu, Ai-Min Xu
Affiliations
Case Reports

Undifferentiated liver embryonal sarcoma in adults: a report of four cases and literature review

Xiao-Wei Li et al. World J Gastroenterol. .

Abstract

Aim: To evaluate the undifferentiated embryonal sarcoma of liver (UESL) in adults in order to improve its diagnosis and treatment.

Methods: Four primary and one recurrent cases of UESL were clinicopathologically evaluated and immunohistochemically investigated with a panel of antibodies using the EnVision+ system. Relevant literature about UESL in adults was reviewed.

Results: Three males and one female were enrolled in this study. Their chief complaints were abdominal pain, weight loss, or fever. Laboratory tests, imaging and pathological features of UESL in adults were similar to those in children. Immunohistochemistry showed evidence of widely divergent differentiation into mesenchymal and epithelial phenotypes. The survival time of patients who underwent complete tumor resection followed by adjuvant transcatheter arterial chemoembolization (TACE) was significantly longer than that of those who underwent surgical treatment alone.

Conclusion: UESL in adults may undergo pluripotential differentiation and its diagnosis should be made based on its morphological and immunohistochemical features. Complete tumor resection after adjuvant TACE may improve the survival time of such patients.

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Figures

Figure 1
Figure 1
Abdominal ultrasonography showing a 16.5 cm × 11.2 cm multilocular cystic liver mass (A), computed tomography imaging demonstrating a large, hypodense tumor occupying the right lobe of liver with multicystic (B) and solid portions (C), and polychromatic cut surface which is soft with fluid and mucoid zones, firm with fleshy areas and necrotico-hemorrhagic changes (D).
Figure 2
Figure 2
Histology showing residual hepatocytes and bile ducts in the tumor (A), giant cells containing eosinophilic hyaline globules in the cytoplasm (B), loose oedematous myxoid matrix with sparse stellate atypical mesenchymal cells (C), fibroblast-like fascicles (D), angiosarcoma-like cells (E) and pericytoma-like and rhabdomyosarcoma-like cells (F) in compact areas (HE, × 400).
Figure 3
Figure 3
Immunohistochemistry showing tumor cells strongly reactive to vimentin (A), diffuse membranous immunostaining for CD56 in mesenchymal cells (B), diffuse multifocal cytoplasmic immunostaining with a distinct paranuclear dot-like staining using cytokeratin 19 (C), focal cytoplasmic positivity for desmin in some tumor cells (D), tumor cells focally positive for α-smooth muscle actin (E) and S100 (F) (EnVision+, × 400).
See this image and copyright information in PMC

References

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