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Mesoblastic Nephroma
When diagnosed in the first 7 months of life, the 5 year EFS and overall survival is 94% and 96%.[1]
A prospective clinical trial that enrolled 50 patients confirmed that complete surgical resection, which includes the entire capsule, is adequate therapy for most patients with mesoblastic nephroma.[2] In this study, only 2 of 50 patients died. Patients were at increased risk for local and eventually metastatic recurrence if there was stage III (incomplete resection and/or histologically positive resection margin), cellular subtype, and aged 3 months or older at diagnosis. Because of the small numbers of patients and the overlapping incidence of these characteristics (5 of 50 patients), the significance of the individual characteristics could not be discriminated. Adjuvant chemotherapy has been recommended for patients who share these three characteristics, though the benefit of adjuvant therapy will remain unproven with such a low incidence of disease.
References
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van den Heuvel-Eibrink MM, Grundy P, Graf N, et al.: Characteristics and survival of 750 children diagnosed with a renal tumor in the first seven months of life: A collaborative study by the SIOP/GPOH/SFOP, NWTSG, and UKCCSG Wilms tumor study groups. Pediatr Blood Cancer 50 (6): 1130-4, 2008.
[PUBMED Abstract]
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Furtwaengler R, Reinhard H, Leuschner I, et al.: Mesoblastic nephroma--a report from the Gesellschaft fur Pädiatrische Onkologie und Hämatologie (GPOH). Cancer 106 (10): 2275-83, 2006.
[PUBMED Abstract]
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