Undifferentiated embryonal sarcoma of the liver in a middle-aged adult with systemic lupus erythematosus

Jia, Changjun; Zhao, Wei; Dai, Chaoliu; Wang, Xinlu; Bu, Xianmin; Peng, Songlin; Xu, Feng; Xu, Yongqing; Yang, Zhao

doi:10.1186/1477-7819-11-244

Cited by 9 publications

(3 citation statements)

References 26 publications

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“…The inconsistency between ultrasound and CT/MRI imaging may be helpful to improve the accuracy of preoperative diagnosis ( 29 ). When there is a large solitary cystic focus of liver disease ( 30 ) or systemic lupus erythematosus (SLE) with hepatic mass ( 31 ), UESL should also be considered. Since primary hepatocellular carcinoma (HCC) or peritoneal carcinoma can be converted into mesenchymal and epithelial differentiation, the coexistence of epithelial differentiation of tumor cells and carcinoma should be excluded to confirm the diagnosis of UESL ( 32 ).…”

Section: Discussionmentioning

confidence: 99%

Undifferentiated embryonic sarcoma of the adult liver with paraneoplastic syndrome: a case report

Luan¹,

Mei²,

Yuan³

et al. 2022

Transl Cancer Res

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Background: Undifferentiated embryonic sarcoma of the liver (UESL) is a rare primary liver sarcoma that mainly affects children. Since it was first reported in 1978, less than 100 cases of UESL in adults have been reported. Due to the lack of specificity of clinical symptoms and imaging findings, the misdiagnosis rate of UESL is very high, and the definite diagnosis is usually in the late stage. Paraneoplastic syndrome (PNS) is a clinical symptom caused by the production of hormones or hormone-like substances in some tumors. This report describes the clinical diagnosis and treatment of patients with these 2 rare disorders. The imaging, histological, and genetic manifestations of UESL are described. This study provides support for the clinical research progress of UESL.Case Description: We report the case of a 57-year-old male with UESL and a progressive abdominal mass. Preoperative fine needle aspiration cytology (FNAC) of intrahepatic mass and postoperative routine pathology showed UESL. Ultrasound examination showed solid intrahepatic mass. Both computed tomography (CT) and magnetic resonance imaging (MRI) showed a large necrotic lesion in the right lobe of the liver. Laboratory examination showed leukocytosis [white blood cells (WBC): 10,005/μL]. The percentage of neutrophils increased (Ne%: 85%). Blood glucose was increased [glucose (Glu): 19.27 mmol/L]. Moderate anemia was observed [hemoglobin (Hb): 64 g/L]. Serum tumor markers (including AFP, PIVIKA, CA19-9, and CEA)were normal. Hepatitis virus markers [hepatitis B surface antigen (HBsAg) and antibodies to hepatitis C virus (anti-HCV)] were negative. The patient presented with paraneoplastic symptoms such as fever, cough, night sweats, hyperinflammation, and hyperglycemia before surgery. After right hemihepatectomy, the paraneoplastic symptoms disappeared completely, and there was no recurrence in the follow-up for six months.Conclusions: UESL is rare, especially in adults. The clinical manifestations of UESL are very similar to those of liver abscess and liver cystic tumor at the initial stage of the disease, for which differentiation is necessary. UESL should be considered when there is large cystic liver disease. FNAC is a good way to confirm the diagnosis. Complete resection of the tumor and postoperative chemotherapy is still the main treatment for UESL. The long-term effects of the therapeutic agents identified by genetic testing in this patient on the disease remain to be seen.

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Section: Discussionmentioning

confidence: 99%

Undifferentiated embryonic sarcoma of the adult liver with paraneoplastic syndrome: a case report

Luan¹,

Mei²,

Yuan³

et al. 2022

Transl Cancer Res

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“…Some data suggest that HAV infection could trigger antiphospholipid syndrome development and precipitation (10) and this syndrome, beyond AIH (38), is also linked with HCC manifestations (39,40). In addition, SLE, potentially triggering by HAV (11,41), apart from AIH (42), is also associated with an increased risk of tumors including HCC (43). However, the pathophysiological background of HAV-mediated tissue damage cannot be a direct and absolute risk factor for HCC, although it could play a theoretical role at least in some subpopulations possibly implying a potential contribution to HCC (44).…”

Section: Hepatitis A-related Henoch-schönlein Purpuramentioning

confidence: 99%

Local and systemic autoimmune manifestations linked to hepatitis A infection

Doulberis,

Papaefthymiou,

Polyzos

et al. 2023

AGEB

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Hepatitis A virus (HAV) represents a global burdening infectious agent causing in the majority of cases a self-limiting acute icteric syndrome, the outcome is related to the hepatic substrate and the potential pre-existing damage, whereas a plethora of extra-hepatic manifestations has also been reported. Despite the absence of post- HAV chronicity it has been associated with an additional burden on existing chronic liver diseases. Moreover, the induced immune response and the antigenic molecular mimicry are considered as triggering factors of autoimmunity with regional and distal impact. Diseases such as autoimmune hepatitis, Guillain-Barré syndrome, rheumatoid arthritis, Still’s syndrome, Henoch-Schönlein purpura, autoimmune hemolytic anemia, antiphospholipid syndrome, systematic lupus erythematosus or cryoglobulinemic vasculitis have been described in patients with HAV infection. Although the exact mechanisms remain unclear, this review aims to accumulate and clarify the pathways related to this linkage.

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“…The occurrence of UESL in patients aged ≥45 years is extremely rare, with only 27 reported cases in the English language literature up to 2012. Patients commonly complain of idiopathic upper abdominal pain, lasting for >10 days ( 4 ). The genetic aberrations of undifferentiated embryonal sarcoma (UES) are not completely understood and the misdiagnosis rate of UES is high.…”

Section: Introductionmentioning

confidence: 99%

Undifferentiated embryonal sarcoma of the liver mistaken for hepatic abscess in an adult

Xie

Sun

et al. 2014

Oncology Letters

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Undifferentiated embryonal sarcoma of the liver (UESL) predominantly occurs in children under the age of 10 years, and ~90% of cases occur in children <15 years old. Patients may complain of abdominal pain, fever or other symptoms. No significant decrease has been identified in the hepatic function or elevation of α-fetoprotein, which differentiates UESL from primary carcinomas of the liver. In the present study, a rare and misdiagnosed case of an UESL arising in a male, which was mistaken for a hepatic abscess and retrospectively re-diagnosed, is reported. This case was misdiagnosed as a hepatic abscess initially, and it was diagnosed as UESL subsequent to performing tests, including a type-B ultrasonic scan and computed tomography (CT), and evaluating pathological findings. The rapid recurrence of the tumor in this patient was identified by CT, and this is associated with the malignancy of the disease. Currently, patients with UESL have a poor prognosis as there is not a successful treatment strategy. The present study analyzes the course of diagnosis and potential treatment for the disease.

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Undifferentiated embryonal sarcoma of the liver in a middle-aged adult with systemic lupus erythematosus

Cited by 9 publications

References 26 publications

Undifferentiated embryonic sarcoma of the adult liver with paraneoplastic syndrome: a case report

Undifferentiated embryonic sarcoma of the adult liver with paraneoplastic syndrome: a case report

Local and systemic autoimmune manifestations linked to hepatitis A infection

Undifferentiated embryonal sarcoma of the liver mistaken for hepatic abscess in an adult

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