Subgaleal and epidural metastases of the undifferentiated embryonal sarcoma of the liver

Doğan, Güleç Mert; Sigirci, Ahmet; Okut, Gökalp; Doğan, Sait Murat; Yılmaz, Sezai

doi:10.1016/j.radcr.2021.10.017

Cited by 2 publications

(2 citation statements)

References 13 publications

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“…Metastatic diseases to the lung, pleura, peritoneum, diaphragm, and heart have been reported [ 12 , 14 ]. A rare case of subgaleal and epidural metastases in UESL has also been reported [ 15 ]. No distinctive laboratory findings are implicated in children with UESL.…”

Section: Discussionmentioning

confidence: 99%

Treatment of Undifferentiated Embryonal Sarcoma of the Liver in Children

Lin,

Wu,

Chen

et al. 2024

Cancers

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Undifferentiated embryonal sarcoma of the liver is a rare mesenchymal tumor with a highly malignant potential. It occurs almost exclusively in the pediatric population and typically has a poor outcome. Although previous studies have reported dismal prognoses, recent advances in combined treatment modalities, e.g., surgery and chemotherapy, have given cause for optimism. Even in those diseases not amenable to complete surgical resection or refractory diseases, other treatment modalities, such as liver transplant, have yielded promising results. This paper provides a review of the current treatment modalities for hepatic undifferentiated embryonal sarcoma in children.

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Section: Discussionmentioning

confidence: 99%

Treatment of Undifferentiated Embryonal Sarcoma of the Liver in Children

Lin,

Wu,

Chen

et al. 2024

Cancers

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“…Most of them are found in the middle and late stages, and the progression of UESL is extremely rapid. Metastases are present in most patients at the time of initial diagnosis, most often in the lung (2,3), pleural and peritoneal regions, and occasionally epidural and subcutaneous zones (4). The Surveillance, Epidemiology, and End Results (SEER) database analysis showed that UESL in adult patients mainly affected males, and the prognosis was significantly worse than that in children (5); the risk of death was significantly higher for adults than pediatric patients (6).…”

Section: Introductionmentioning

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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Subgaleal and epidural metastases of the undifferentiated embryonal sarcoma of the liver

Cited by 2 publications

References 13 publications

Treatment of Undifferentiated Embryonal Sarcoma of the Liver in Children

Treatment of Undifferentiated Embryonal Sarcoma of the Liver in Children

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

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