He dawei
Children's Hospital of Chongqing Medical University. China
Title: Primary Renal Rhabdomyosarcoma of pediatrics: 7 cases in an 23-Year Retrospective Study
Biography
Biography: He dawei
Abstract
Background: Rhabdomyosarcoma (RMS) of the renal is an extremely rare lesion for which only a few reports are available.
PURPOSE: To describe clinical and pathologic characteristics and outcome of patients with renal Rhabdomyosarcoma.
Material and methods: A retrospective review of all the patients diagnosed with renal RMS within a single regional teaching hospital between 1st January 1993 and 1st October 2016 was undertaken.
Results: There were 7 patients with renal rhabdomyosarcoma registered. They all presented to us with a palpable lower quadrant mass or gross hematuria. An increase in white blood cell count, C-reactive protein, aspartate aminotransferase, lactate dehydrogenase and red blood cell count of Urinalysis were observed. Abdominal ultrasonography and Computed tomography revealed a pancake kidney with a large mass (right-side: left-side= 3:4), measuring 5.7× 4.1× 4.4 cm3-13.3× 12.5× 9.5 cm3. All 7 patients underwent radical nephrectomy and lymph node dissection. Chemotherapy was performed according to the ICE and VDC regimen after surgery. Follow-up information was obtained for 85.7% of patients. Median follow-up time of the patients is 26.5 months from diagnosis and they were all alive.
Conclusion: Currently, there is a lack of knowledge and consensus regarding the treatment of primary renal RMS among children. Renal RMS seems to require aggressive treatment, and multimodal therapy may play an important role.
Keywords: renal RMS - pediatrics - immunophenotype – chemotherapy