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小儿肝横纹肌肉瘤:儿童腹部肿块的罕见病因。

Pediatric hepatic rhabdoid tumor: A rare cause of abdominal mass in children.

作者信息

Kapral Nicole, Melmer Patrick, Druzgal Colleen Harkins, Lancaster Luke

机构信息

Department of Radiology, University of Virginia Health System, 1215 Lee St., Charlottesville, VA, 22908.

Department of Surgery, Grand Strand Medical Center, 809 82nd Pkwy, Myrtle Beach, SC, 29572.

出版信息

Radiol Case Rep. 2018 May 10;13(3):724-727. doi: 10.1016/j.radcr.2018.03.017. eCollection 2018 Jun.

Abstract

Pediatric hepatic rhabdoid tumors are rare tumors of the liver, with few cases reported in the literature. These aggressive tumors can be difficult to differentiate from hepatoblastomas on imaging alone, and surgical biopsy combined with special immunohistochemical stains can assist in differentiating these 2 tumor types. We present a case of hepatic rhabdoid tumor in a 7-month-old female infant, which was originally thought to be a hepatoblastoma; however, using BAF47 staining for INI-1 we were able to diagnose a rhabdoid tumor and affect the patient's medical oncologic therapy. Earlier detection and a better understanding of the imaging features of hepatic rhabdoid tumor may aid in improved patient management and treatment planning.

摘要

小儿肝脏横纹肌样瘤是一种罕见的肝脏肿瘤,文献报道的病例较少。这些侵袭性肿瘤仅通过影像学检查很难与肝母细胞瘤区分开来,手术活检结合特殊免疫组化染色有助于区分这两种肿瘤类型。我们报告一例7个月大女婴的肝脏横纹肌样瘤病例,该病例最初被认为是肝母细胞瘤;然而,通过使用针对INI-1的BAF47染色,我们得以诊断出横纹肌样瘤,并影响了患者的医学肿瘤治疗。更早地发现并更好地了解肝脏横纹肌样瘤的影像学特征可能有助于改善患者管理和治疗规划。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/79de/6066605/0bb8e737d4a8/radcr500-fig-0001.jpg

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