Undifferentiated embryonal sarcoma of liver in an adult masquerading as complicated hydatid cyst

Kalra, Naveen; Vyas, Sameer; Das, Palash Jyoti; Kochhar, Rakesh; Srinivasan, Radhika; Khandelwal, Niranjan

doi:10.1016/s1665-2681(19)31592-3

Cited by 17 publications

(2 citation statements)

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“…These reports often present as masses with solid and cystic components. Due to the rarity of UESL in adult patients, these patients are often misdiagnosed as hepatic abscess, hemorrhage cystic tumor, or hydatic cyst, as in the current case [2]. The diagnosis of UESL is difficult because it has no specific clinical characteristics, as shown in this case.…”

Section: Discussionmentioning

confidence: 86%

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Undifferentiated embryonal sarcoma of the liver occurring in an adolescent: a case report with genomic analysis

et al. 2022

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Background Undifferentiated embryonal sarcoma of the liver (UESL) is a rare malignant mesenchymal tumor that usually occurs in children and is rarely diagnosed in adults. Case presentation The case was a female in her late 20s who presented with a huge liver mass found upon the examination of fever. Imaging analysis showed a well-defined mass measuring 9 cm in the largest dimension in the right posterior segment of the liver. The patient underwent right hemi-hepatectomy. Histopathological studies revealed that the circumscribed tumor was composed of a proliferation of atypical epithelioid to spindle-shaped cells with pleomorphic nuclei arranged in haphazard pattern. Histopathological features observed in immunohistochemical analyses confirmed a final diagnosis of UESL. Genome analysis using FoundationOne CDx revealed 11 somatic mutations including TP53 (R196*) and STK11 (F354L). Adjuvant chemotherapy with ifosfamide and etoposide was performed, and the case has been followed up without recurrence for 1 year after hepatectomy. Conclusions A UESL should be considered in the differential diagnosis of large and well-defined solid liver lesions. Although the prognosis of UESL is extremely unfavorable, aggressive surgical resection with adjuvant chemotherapy and genomic analysis may be helpful for ensuring long-term survival.

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Section: Discussionmentioning

confidence: 86%

“…Undifferentiated embryonal sarcoma of the liver (UESL) is an extremely rare entity in adulthood, with fewer than 60 cases reported in the literature [1][2][3]. Embryonal sarcoma more typically occurs in children, with a peak incidence between the ages of 6 and 10 years with no sex predilection [4].…”

Section: Introductionmentioning

confidence: 99%