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恶性横纹肌样瘤——极具迷惑性的疾病:两例病例报告

Malignant rhabdoid tumor-The great mimicker: Two case reports.

作者信息

Ivana Dasic, Sofija Cvejic, Polina Pavicevic, Smilja Rancic

机构信息

Department of Radiology, University Children's Hospital, Tirsova 10, 11000 Belgrade, Serbia.

出版信息

Radiol Case Rep. 2021 Jan 27;16(4):815-818. doi: 10.1016/j.radcr.2021.01.018. eCollection 2021 Apr.

Abstract

Malignant rhabdoid tumors (MRT) represent a very rare group of tumors in infants that have an aggressive clinical behavior, are refractory to therapy, and have a high mortality rate. We present two cases that demonstrate the importance of early diagnosis for further treatment. The first case was a 4 and a half month old boy that presented with an enlarged liver and was treated as hepatoblastoma with metastatic changes in the lung parenchyma after the diagnosis. The second case was a female neonate presenting with hardening on the right side of the abdomen which was treated as MRT of the liver after radiological and clinical evaluation. In both cases, the initial histopathologic diagnosis was hepatoblastoma, but later analyses confirmed the diagnosis of MRT. From our experience, imaging findings can be a valuable tool in directing further evaluation since even histopathologic differentiation from the small cell undifferentiated hepatoblastoma is a real challenge.

摘要

恶性横纹肌样瘤(MRT)是婴儿中一类非常罕见的肿瘤,具有侵袭性的临床行为,对治疗难治,且死亡率高。我们报告两例病例,展示了早期诊断对进一步治疗的重要性。第一例是一名4个半月大的男孩,表现为肝脏肿大,诊断后被当作伴有肺实质转移改变的肝母细胞瘤进行治疗。第二例是一名女新生儿,表现为腹部右侧变硬,经放射学和临床评估后被当作肝脏MRT进行治疗。在这两例病例中,最初的组织病理学诊断均为肝母细胞瘤,但后来的分析证实为MRT。根据我们的经验,影像学检查结果在指导进一步评估方面可能是一个有价值的工具,因为即使是与小细胞未分化肝母细胞瘤进行组织病理学鉴别也颇具挑战。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c451/7847829/e8c8a47c5d2d/gr1.jpg

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