PT number Author Year Country Sex Age at diagnosis (years/months) Symptoms Location Size (cm) Histology IRS group Therapy Outcome 1 Ransom [26 , 27 ] 1933 USA F 4 m Mass below the umbilicus and extending somewhat to the right Urachus (median UL): attached along the anterior wall from the umbilicus to the apex of the bladder 11.5 Spindle cell I SMAC, RT Last check-up examination (2 years) showed no evidence of recurrence 2 Ishikawa et al. [28 ] 1985 Japan F 1 y 4 m Abdominal mass, abdominal pain, hemoperitoneum Urachal (median UL): intra-abdominal rupture of the tumor Fist-sized NDF IV SMAC NDF 3 Yokoyama et al. [27 , 29 ] 1997 Japan M 2 y Episodic lower abdominal pain Urachus (median UL): extraperitoneal located between the umbilicus and apex of the bladder and was adhering tightly to both. The bilateral medial UL were seen entering the tumor. Ruptured with a small number of disseminated tumors scattered on the mesentery and Douglas pouch 12 Embryonal IV SMAI, ChT At 2 years of follow-up no recurrence had been detected Kerr et al. [7 , 8 ] 1998 England F 3 y 2 m Abdominal mass Urachus (median UL), adherent to the bladder NDF Embryonal IIa SMII, ChT Histology showed extending up to the resection margin.5 , 9 ] 5 Schulz and O’Leary27 , 30 ] 2001 Scotland M 2 y NDF Urachus (median UL) NDF Embryonal NDF S, ChT, RT Tumor-free age of 26 years 6 Fernández et al. [27 ] 2007 Spain F 6 y Intractable constipation, nausea, abdominal painless mass Urachus (median UL), infiltrating the ventral abdominal wall and fixed to the vesical dome 13.5 Embryonal Ib SMIC, ChT At 4 years of follow-up, the patient remains well and free of clinical disease Menke et al. [24 , 25 ] 2015 Germany F 3 y Abdominal mass Lower anterior abdomen, above the urinary bladder& (urachus, median UL by location) NDF Embryonal IIa SMII, ChT R1 resection (marginal tumor residue). Tumor-free age of 6 years 8 Cheikhelard et al. [31 ] 2015 France F 5 y 5 m NDF Urachus (median UL), peritoneal metastases and hypogastric lymph nodes 5 NDF IV SMAI, ChT Died 31 months after diagnosis with relapse in peritoneal liver at 11 months 9 Cheikhelard et al. [31 ] 2015 France F 3 y 3 m Peritoneal rupture, poor general status Urachus (median UL), lumboaortic and external iliac lymph nodes 10 Alveolar IV SMAI, ChT, RT Died 57 months after diagnosis with relapse in peritoneal liver at 30 months 10 Cheikhelard et al. [31 ] 2015 France F 2 y 6 m Mass, poor general status, dysuria Urachus (median UL) 15 Embryonal III SMII, ChT, RT Died 37 months after diagnosis with peritoneal relapse at 19 months 11 Cheikhelard et al. [31 ] 2015 France M 4 y 2 m Mass, poor general status, dysuria Urachus (median UL), peritoneal metastases and iliac lumboaortic lymph nodes 10 Embryonal IV SMII, ChT, RT Relapsed multifocal at 82 months, follow-up 100 months 12 Cheikhelard et al. [31 ] 2015 France M 5 y 3 m Mass, poor general status Urachus (median UL), peritoneal metastases and mesenteric lymph nodes 10.6 Embryonal IV SMIC, ChT, RT Tumor free after 95 months since diagnosis 13 Cheikhelard et al. [31 ] 2015 France M 2 y 5 m Mass, poor general status, obstructive renal insufficiency Urachus (median UL), peritoneal bone metastases and hypogastric lymph nodes 21 Embryonal IV SMII, ChT, RT Bone progression after 16 months; died 18 after diagnosis 14 Cheikhelard et al. [31 ] 2015 France M 4 y 5 m Abdominal pain Urachus (median UL) 7 Embryonal III SMIC, ChT Tumor free after 18 months since diagnosis 15 Cheikhelard et al. [31 ] 2015 France M 6 y Abdominal pain Urachus (median UL), peritoneal metastases 14 Embryonal IV SMAI, ChT, RT Tumor free after 45 months since diagnosis Sánchez-Montenegro et al. (this report) 2017 Spain F 2 y 4 m Asymptomatic Left medial UL 5 Embryonal Ia ChT, SMIC, RT Tumor-free age of 3 years and 9 months Sánchez-Montenegro et al. (this report) 2017 Spain F 3 y 10 m Asymptomatic Right medial UL 8.5 Spindle cell IIa SMII, ChT Tumor-free age of 4 years and 5 months
