Primary Renal Rhabdomyosarcoma in an Adolescent With Tumor Thrombosis in the Inferior Vena Cava and Right Atrium

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.

Primary renal embryonal rhabdomyosarcoma in an adult patient

“…RMS can be classified into four subtypes: alveolar, embryonal, spindle-cell and pleomorphic 2. While an estimated 25% of embryonal RMS (ERMS) cases in adolescents involve the genitourinary system, there are only a few genitourinary RMS cases reported in the adult literature 3. Our thus case adds to the limited amount of data on this disease.…”

“…Radiographically, primary renal RMS has characteristics similar to renal cell carcinoma with no predictive features on CT imaging. Thus, definitive diagnosis is dependent on postoperative immunohistochemical analysis after surgery 3 4. Grignon et al first established criteria necessary to make the diagnosis of primary sarcoma of the kidney.…”

Renal Rhabdomyosarcoma in a Young Woman with Gross Hematuria: A Case Report

“…RMS is a common tumor in pediatrics, but not in adults (1). According to the new world health organization (WHO) classification, RMS is divided into 4 groups: Embryonal rhabdomyosarcoma, alveolar rhabdomyosarcoma, pleomorphic rhabdomyosarcoma, and spindle cell/sclerosing rhabdomyosarcoma.…”

“…Rhabdomyosarcoma (RMS) is a malignancy of skeletal muscle that can occur everywhere in the body (1). Renal tumors comprise a diverse spectrum of neoplastic lesions with patterns that are relatively distinct for children and adults.…”

Rhabdomyosarcoma (RMS) of the Renal Pelvis in a Child: A Case Report

“…Few cases are described in the literature. 12,14,15 The most important statistic revealed 6 cases of primary renal embryonal rhabdomyosarcoma (ERMS). 16 Senga et al 17 reported 15 cases of RMS of the kidney in Japanese literature.…”