title: Primary Renal Rhabdomyosarcoma in an Adolescent With Tumor Thrombosis in the Inferior Vena Cava and Right Atrium: A Case Report and Review of the Literature.
abstract: Although the second peak of the age distribution of rhabdomyosarcoma (RMS) is at adolescence, renal RMS is extremely rare at this age group. This tumor is indistinguishable from other renal tumors based on clinical and imaging findings, and the diagnosis relies on histology and immunohistochemical staining. We report a unique case of adolescent renal RMS associated with tumor thrombus extending into the inferior vena cava (IVC) and right atrium.An 18-year-old female adolescent presented with shortness of breath and palpitations, associated with right flank discomfort, and hematuria. A pleomorphic-type renal RMS with Budd-Chiari syndrome and arrhythmia induced by IVC and RA thrombosis was diagnosed. Despite complete tumor resection, the patient developed multiple lung metastases a month after surgery. Chemotherapy was recommended, but the patient declined. She died within a year of the initial operation.Adolescent renal RMS is rare and associated with poor outcome. Early aggressive multimodal therapy seems to be appropriate, in particular, in the presence of tumor thrombosis.
authors.affiliation: From the Department of Radiology (W-CL, C-HC), China Medical University Hospital; School of Medicine (W-CL, H-CW), China Medical University; Department of Biomedical Imaging and Radiological Science (W-CL, C-HL), College of Health Care, China Medical University, Taichung; E-Da Hospital and I-Shou University (J-HC), Kaohsiung, Taiwan (ROC.); Center for Functional Onco-Imaging (J-HC), School of Medicine, University of California, Irvine, CA; Departments of Radiology and Biomedical Imaging, and Urology (AW), University of California, San Francisco, CA; Department of Pathology (HC, I-PC), China Medical University Hospital; Department of Urology (H-CW), China Medical University Hospital; and China Medical University Children's Hospital (C-HL), Taichung, Taiwan (ROC).
authors.forename: Wei-Ching
authors.initials: WC
authors.lastname: Lin
authors.forename: Jeon-Hor
authors.initials: JH
authors.lastname: Chen
authors.forename: Antonio
authors.initials: A
authors.lastname: Westphalen
authors.forename: Han
authors.initials: H
authors.lastname: Chang
authors.forename: I-Ping
authors.initials: IP
authors.lastname: Chiang
authors.forename: Cheng-Hong
authors.initials: CH
authors.lastname: Chen
authors.forename: Hsi-Chin
authors.initials: HC
authors.lastname: Wu
authors.forename: Chien-Heng
authors.initials: CH
authors.lastname: Lin
mesh_terms: D000293:Adolescent; D001145:Arrhythmias, Cardiac / Q000150:complications; D006502:Budd-Chiari Syndrome / Q000150:complications; D005260:Female; D006325:Heart Atria / Q000473:pathology*; D006801:Humans; D007680:Kidney Neoplasms / Q000150:complications* / Q000473:pathology / Q000601:surgery; D008175:Lung Neoplasms / Q000556:secondary; D012208:Rhabdomyosarcoma / Q000473:pathology; D013927:Thrombosis / Q000209:etiology*; D014682:Vena Cava, Inferior / Q000473:pathology*