Fetal rhabdomyomatous nephroblastoma: diagnosis and therapy / 中华泌尿外科杂志

ABSTRACT

Objective To explore the characteristic clinical profiles and treatment modalities of Fetal rhabdomyomatous nephroblastoma(FRN).Methods A retrospective study was conducted for 14 FRN patients from Jan.2000 to Oct.2015.Their clinical data were collected including clinical presentations,pathology and treatment modalities.There were 8 males and 6 females with a mean age of 23 months.There were 3 cases at left side and right side 5 cases,bilateral 6 cases.2 patients were classified as stage Ⅰ,1 stage Ⅱ,5 stageⅢ and 6 stageⅤ.Abdominal mass was the main clinical presentation in 11 patients,and 1 case with hematuria,1 with abdominal pain,and 1 with vomit.Most tumors showed cysts or completely solid from the ultrasonography.Computed tomographic scan revealed a large inhomogeneous enhancement tumor from the kidney pole with necrotic,cystic,bleeding or calcification.Ultrasonography and Computed tomography (CT) had no different performance from Wilms' tumor.9 patients received preoperative chemotherapy,and the response was none in all of them.8 unilateral patients underwent tumor nephrectomy and another 4 had nephron-sparing surgery.Results Pathology showed that FRN contained more than 70％ of fetal rhabdomyomatous tissue.Immunohistochemistry had no specificity,most FRN shows Desmin (+) and Myogenin(+).Bilateral FRN tumors were seen in 2,one side with FRN and another side with nephroblastomatosis were seen in 3,one side with FRN and another side with Wilm's tumor was seen in 1 patient.Postoperative pathology confirmed FRN in all 14 cases.All patients received postoperative chemotheraphyAct-D and VCR for 6 month(stage Ⅰ),Act-D and VCR for 15 month(stage Ⅱ),Act-D +VCR + ADR and radiotherapy for 15 month(stageⅢ).During follow-up of 6 months to 15 years,10 of them were alive without tumor and no evidence of recurrence.Conclusions FRN is a rare histologic variant of Wilm's tumor with less aggressive behavior.FRN usually has a huge volume and is bilateral with a poor responder to preoperative chemotherapy,but it is associated with a generally favorable outcome.Surgery and chemically treatment appears the effective measure.