Prognostic factors in children undergoing salvage surgery for bladder/prostate rhabdomyosarcoma

5-yr PFS proved unrelated to excision margins of the surgical specimen, that is on whether there was evidence of microscopic residues left behind during surgery. These data suggest that the outcome is more influenced by the biological properties of the tumor. If small foci of differentiated cells are left behind, these probably do not compromise final prognosis. In our patients requiring salvage bladder-prostate surgery after failure of chemotherapy ± radiotherapy, long-term progression-free survival seemed unrelated to patient/tumor characteristics at presentation and preoperative management. Regarding the characteristics of the removed specimen, instead, an undifferentiated tumor histology and a diameter of the removed tumor >5 cm negatively influenced prognosis, whereas the presence of positive excision margins did not.PFS >5 years n/N (%)pVariables at diagnosisAge at diagnosis<5 years14/20 (70)0.4>5 years3/6 (50)GenderMale12/20 (60)0.3Female5/6 (83)Histology on initial biopsyEmbryonal9/16 (56)0.1Botryoid8/9 (89)Tumor locationProstate4/7 (57)0.6Bladder11/15 (73)Bladder-prostate2/4 (50)Tumor size<5 cm5/7 (71)0.7>5 cm12/19 (63)T-stageT17/10 (70)0.7T210/16 (62.5)Treatment variablesTreatment protocolRMS 885/7 (71.5)0.5RMS 964/8 (50)RMS 20056/8 (75)Tumor response at 9 weeksComplete response (CR)0/1 (0)0.4Partial response (PR) > 2/39/13 (69)Minor response (MR) > 1/3 but < 2/34/7 (57)No response or stable disease (SD) < 1/34/4 (100)Preoperative radiotherapyNo8/12 (67)1Yes9/14 (64)Pathology variablesTumor location on surgical specimenProstate5/8 (62.5)0.1Bladder11/14 (78.5)Bladder-prostate1/4 (25)Surgical marginsNegative7/12 (58)0.1Positive7/8 (87.5)Diameter of tumor at the surgical specimen<5 cm10/12 (83)0.06>5 cm3/8 (37)Histological differentiationDifferentiated11/13 (84.5)0.002Undifferentiated1/7 (14)Myxoid3/4 (75)