Neoadjuvant transcatheter arterial chemoembolization and systemic chemotherapy for the treatment of undifferentiated embryonal sarcoma of the liver in children

He, Min; Cai, Jia‐Bin; Lai, Can; Mao, Jun-Qing; Xiong, Jieni; Guan, Zhonghai; Li, Lin-Jie; Shu, Qiang; Ying, Meidan; Jin-hu, Wang

doi:10.12998/wjcc.v10.i19.6437

Cited by 3 publications

(2 citation statements)

References 26 publications

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“…An effective response with a reduced tumor volume, clearance of the tumor margin, and massive tumor necrosis were reported. This proposed alternative route of chemotherapy administration may serve as a promising alternative treatment in children with UESL [ 30 ].…”

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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“…According to the latest UESL report, neoadjuvant therapy (NAT) including preoperative transcatheter arterial chemoembolization (TACE) and systemic chemotherapy can effectively reduce the tumor volume, clear the tumor margin, and cause extensive tumor necrosis in UESL ( 41 ). It provides effective value for further complete resection of the tumor and prevention of intraoperative tumor rupture and dissemination.…”

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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Ultrasonography, contrast-enhanced ultrasonography and contrast- enhanced computer tomography features of hepatic sarcomatoid carcinoma and hepatic sarcoma: a retrospective study of 23 cases

Long,

Wu,

Liu

et al. 2024

Preprint

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Background Hepatic sarcomatoid carcinoma (HSC) and hepatic sarcoma (HS) are rare malignancies. Without pathology, the differential diagnosis between these two tumors are difficult due to their frequent overlaps in clinical presentations and imaging features. Currently, there are limited analyses about the ultrasound (US), contrast-enhanced ultrasound (CEUS) and contrast-enhanced computer tomography (CECT) characteristics of HSC and HS. Therefore, the purpose of our study is to evaluate the value of US, CEUS and CECT on the differential diagnosis between HSC and HS. Methods From 2015 to 2022, a total of 23 patients with HSC (n = 11) and HS (n = 12) are included in this retrospective study. We analyze the clinical, pathological, and imaging data of these patients. Analysis of differences is performed to determine the consistent and distinctive features. Results HSCs have a considerably higher prevalence of chronic hepatitis (p = 0.005) and cirrhosis (P = 0.027) than HSs, while metastases are more prevalent in HSs (P = 0.005). The lesion size of HSCs (8.1 ± 2.2 cm) is slightly larger than that of HSs (6.2 ± 3.4 cm). On conventional US, the characteristics of HS and HSC are similar. On CEUS, HSC can be differentiated from HS by heterogeneous rim-like enhancement with necrotic zone during arterial phase (AP). Notably, for different pathological types of HS, the presence of enhancement in AP on CECT can be used to distinguish hepatic fibrosarcoma from hepatic liposarcoma. Conclusion HSC and HS generally present as masses with hypo-echoic and hypo-vascularity. HSC usually presents heterogeneous density. The degree of enhancement, the time of wash-out start, and the presence of necrotic areas may contribute to distinguish the different pathological types of HS.

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Neoadjuvant transcatheter arterial chemoembolization and systemic chemotherapy for the treatment of undifferentiated embryonal sarcoma of the liver in children

Cited by 3 publications

References 26 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

Ultrasonography, contrast-enhanced ultrasonography and contrast- enhanced computer tomography features of hepatic sarcomatoid carcinoma and hepatic sarcoma: a retrospective study of 23 cases

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