Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0035412 (rhabdomyosarcoma)
6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 63-year-old man with symptoms of obstruction of the inferior vena cava was examined by computed tomography, ultrasound imaging and angiography. Examination revealed a tumor in the inferior vena cava, and transvenous biopsy revealed a rhabdomyosarcoma. The tumor was surgically resected and was easily separated from the surrounding tissues. Nevertheless, a local recurrence developed 43 days after the operation, and the patient's condition deteriorated rapidly. Hepatomegaly and ascites believed to represent the Budd-Chiari syndrome were noted. The patient died on the 163rd postoperative day. Autopsy revealed a tumor extending from the inferior vena cava just above the right renal vein to the right atrium and involving the lobus caudatus of the liver. Clinically, the tumor was thought to have arisen from the middle segment of the inferior vena cava. However, a diagnosis of primary hepatic rhabdomyosarcoma with extrahepatic growth could not be excluded. Only 12 cases of primary liver rhabdomyosarcoma have been reported, and none of those patients demonstrated Budd-Chiari syndrome. Our patient, diagnosed as rhabdomyosarcoma with secondary Budd-Chiari syndrome, is believed to be the first such report.
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PMID:Rhabdomyosarcoma in the inferior vena cava with secondary Budd-Chiari syndrome. 849 51

Primary malignant tumors of the heart, specifically cardiac sarcomas, are rare and mainly diagnosed at autopsy. Acute Budd-Chiari syndrome is a recognized cause of acute liver failure and has been associated with several rare cardiac tumors: atrial myxoma, caval rhabdomyosarcoma, and primary cardiac adenocarcinoma. We present the first case of a fatal, highly differentiated cardiac synovial sarcoma that presented as acute liver failure from Budd-Chiari syndrome.
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PMID:Acute Liver Failure Due to Budd-Chiari Syndrome in the Setting of Cardiac Synovial Sarcoma. 2615 57

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.
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PMID: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. 2722 46