Table 3.
Characteristics of Pediatric Patients with Umbilical Ligament Rhabdomyosarcoma.
| 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 |
| 4∗ | 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. Died aged 5 years [5, 9] |
| 5 | Schulz and O'Leary [27, 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 |
| 7∗ | 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 Dx: 1983 |
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 Dx: 2003 |
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 Dx: 2005 |
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 Dx: 2005 |
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 Dx: 2005 |
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 Dx: 2006 |
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 Dx: 2010 |
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 Dx: 2010 |
France | M | 6 y | Abdominal pain | Urachus (median UL), peritoneal metastases | 14 | Embryonal | IV | SMAI, ChT, RT | Tumor free after 45 months since diagnosis |
| 16∗ | Sánchez-Montenegro et al. (this report) | 2017 Dx: 2015 |
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 |
| 17∗ | Sánchez-Montenegro et al. (this report) | 2017 Dx: 2015 |
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 |
ChT, chemotherapy; Dx, diagnosed; F, female; IRS, intergroup rhabdomyosarcoma; M, male; NDF, no data found; RT, radiotherapy; S, surgery; SMAI, surgery macroscopically incomplete; SMAC, surgery macroscopically complete; SMIC, surgery microscopically complete; SMII, surgery microscopically incomplete; UL, umbilical ligament. &Personal communication by author. ∗Patients with Costello syndrome.