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肝脏复发性未分化胚胎性肉瘤伴腹膜和脾脏转移的罕见表现:病例报告及文献复习

A rare presentation of recurrent undifferentiated embryonal sarcoma of the liver with peritoneal and splenic metastases: case report and literature review.

作者信息

Alanbagi Alaa A, Aldarawsha Ali N, Alawad Asdah, Hasan Sora A, Kareem Murtadah R, Ahmed Aula, Al Gehadi Muntadher Y H, Bacha Zaryab, Al-Badri Sajjad G

机构信息

Oncology Department, Warith International Cancer Institute, Karbala, Iraq.

Radiology Department, Warith International Cancer Institute, Karbala, Iraq.

出版信息

J Surg Case Rep. 2025 Aug 30;2025(8):rjaf680. doi: 10.1093/jscr/rjaf680. eCollection 2025 Aug.

DOI:10.1093/jscr/rjaf680
PMID:40894941
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12398261/
Abstract

Undifferentiated embryonal sarcoma of the liver (UESL) is a rare, aggressive pediatric malignancy with a high recurrence rate. We present a case of a 16-year-old male with recurrent UESL and rare peritoneal and splenic metastases. Despite initial complete surgical resection, the tumor recurred, necessitating further surgeries and chemotherapy. PET-CT imaging played a crucial role in monitoring disease progression. This case highlights the importance of a multimodal approach, integrating surgery and systemic therapy, in managing recurrent UESL and underscores the need for long-term surveillance to improve patient outcomes.

摘要

肝未分化胚胎性肉瘤(UESL)是一种罕见的侵袭性儿科恶性肿瘤,复发率高。我们报告一例16岁男性复发性UESL,伴有罕见的腹膜和脾脏转移。尽管最初进行了完整的手术切除,但肿瘤仍复发,需要进一步手术和化疗。PET-CT成像在监测疾病进展中发挥了关键作用。该病例突出了多模式方法(整合手术和全身治疗)在复发性UESL管理中的重要性,并强调了长期监测以改善患者预后的必要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7a80/12398261/fd421e9ce482/rjaf680f6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7a80/12398261/41d2fc5c4c3c/rjaf680f1.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7a80/12398261/dcb6ad7856a7/rjaf680f4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7a80/12398261/9daf76ae7e2f/rjaf680f5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7a80/12398261/fd421e9ce482/rjaf680f6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7a80/12398261/41d2fc5c4c3c/rjaf680f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7a80/12398261/851b2f38e6cf/rjaf680f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7a80/12398261/5db0713008d7/rjaf680f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7a80/12398261/dcb6ad7856a7/rjaf680f4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7a80/12398261/9daf76ae7e2f/rjaf680f5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7a80/12398261/fd421e9ce482/rjaf680f6.jpg

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本文引用的文献

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Undifferentiated Embryonal Sarcoma of the Liver with Epithelioid Features: A Case Report of an Exceptional Histological Heterogeneity among Rare Diseases.肝未分化胚胎肉瘤伴上皮样特征:罕见病中一种特殊组织学异质性的病例报告。
Ann Ital Chir. 2024;95(2):119-125. doi: 10.62713/aic.3374.
2
Long-Term Survival in an Adolescent and Young Adult with Metastatic Relapse of an Undifferentiated Embryonal Sarcoma of the Liver.青少年和年轻成人肝未分化胚胎性肉瘤转移复发的长期生存。
J Adolesc Young Adult Oncol. 2024 Aug;13(4):714-719. doi: 10.1089/jayao.2023.0105. Epub 2024 Apr 5.
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Management of undifferentiated embryonal sarcoma of the liver: A Pediatric Surgical Oncology Research Collaborative study.
肝脏未分化胚胎性肉瘤的管理:一项儿科外科肿瘤学研究协作组的研究。
Pediatr Blood Cancer. 2024 Jun;71(6):e30975. doi: 10.1002/pbc.30975. Epub 2024 Mar 31.
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Treatment of Undifferentiated Embryonal Sarcoma of the Liver in Children.儿童肝脏未分化胚胎性肉瘤的治疗
Cancers (Basel). 2024 Feb 23;16(5):897. doi: 10.3390/cancers16050897.
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An Adult With Undifferentiated Embryonal Sarcoma of the Liver: A Case Report of a Rare Encounter.一名患有肝脏未分化胚胎性肉瘤的成年人:罕见病例报告。
Cureus. 2023 Sep 11;15(9):e45018. doi: 10.7759/cureus.45018. eCollection 2023 Sep.
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SA J Radiol. 2021 Aug 31;25(1):2179. doi: 10.4102/sajr.v25i1.2179. eCollection 2021.
7
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Hepat Oncol. 2020 Apr 7;7(2):HEP19. doi: 10.2217/hep-2020-0002.
8
The genomic landscape of undifferentiated embryonal sarcoma of the liver is typified by C19MC structural rearrangement and overexpression combined with TP53 mutation or loss.肝未分化胚胎性肉瘤的基因组图谱以 C19MC 结构重排和过表达为特征,同时伴有 TP53 突变或缺失。
PLoS Genet. 2020 Apr 20;16(4):e1008642. doi: 10.1371/journal.pgen.1008642. eCollection 2020 Apr.
9
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