A Liver Transplant for Local Control in a Pediatric Patient with Metastatic TFE3-Associated Perivascular Epithelioid Cell Tumor (PEComa) to the Liver.

IF 0.7 Q4 PATHOLOGY Case Reports in Pathology Pub Date : 2021-10-05 eCollection Date: 2021-01-01 DOI:10.1155/2021/3924565
Whayoung Lee, Josephine HaDuong, Aaron Sassoon, Tuan Dao, Ali Nael
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引用次数: 1

Abstract

Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors with widespread distribution throughout the body and unpredictable clinical behavior. Recently, a subset of these tumors has been reported to harbor Transcription Factor E3 (TFE3) gene rearrangement with distinct morphologic and immunophenotypic features. Although limited, these tumors may represent a separate entity from the conventional PEComas and may require different treatment approaches. Surgery is the main treatment option with no clear consensus on systemic therapy. Here, we present the first case of a malignant pediatric colonic TFE3-associated PEComa with isolated liver metastasis leading to liver transplantation for the local control.

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肝脏转移tfe3相关血管周围上皮样细胞瘤(PEComa)患儿的局部控制肝移植
血管周围上皮样细胞瘤(PEComas)是一种罕见的间充质肿瘤,广泛分布于全身,临床行为难以预测。最近,这些肿瘤的一个亚群被报道含有转录因子E3 (TFE3)基因重排,具有独特的形态和免疫表型特征。尽管范围有限,但这些肿瘤可能与传统的PEComas不同,需要不同的治疗方法。手术是主要的治疗选择,对全身治疗没有明确的共识。在这里,我们报告了第一例儿童结肠tfe3相关的恶性PEComa,并伴有孤立的肝转移,导致肝移植作为局部对照。
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审稿时长
12 weeks
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