Table 1.
Clinical features in 15 patients from the Sixth Affiliated Hospital and the Cancer Center of Sun Yat-sen University
| Gender/age | Presenting symptoms | Misdiagnosis/mistreatment | Time interval from symptom onset to pathological diagnosis | Tumor site | Maximum diameter of tumor (cm) | Treatment* | Pathological subtype | IRS clincial group/stage | outcome | Course of disease/survival time (m/m) |
|---|---|---|---|---|---|---|---|---|---|---|
| 1/ < 10 y | Perianal mass | None | 3 d | Perineal | 6.6 | LRR(R0) + Chemotherapy | ERMS | IV/T2bN1M1(lung, pleura, Mediastinum) | AWD | –/86 |
| 2/ ≥ 20 y | Perianal mass | None | 1 m | Perineal | 4.5 | APR(R0) + Chemotherapy | ARMS | II/T2aN1M0 | DOD | 24/24 |
| 2/ ≥ 20 y | Perianal mass | None | 1 m | Perineal | 12.2 | Chemotherapy | ERMS | III/T2bN1M0 | DOD | 18/18 |
| 2/10–19 y | Perianal mass | None | 1 m | Perianal | 6.8 | Chemotherapy | ERMS | IV/T2bN1M1(bone; pelvic; retroperitoneum) | DOD | 19/19 |
| 2/ ≥ 20 y | Painfulperianal mass | Perianal abscess/abscessotomy | 2 m | Perianal | 10.0 | APR(Rx) + Chemotherapy | ARMS | II/T2bN1M0 | DOD | 8/8 |
| 2/ ≥ 20 y | Painful perianal mass | None | 7 d | Perianal | 4.7 | APR(R0) + Chemotherapy | ARMS | I/T2aN0M0 | AWD | 12/12 |
| 2/10–19 y | Perianal mass | None | 2 m | Perineal | 5 | Inguinal lymph node dissection + chemotherapy | ERMS | III/T2bN1M0 | DOD | 25/25 |
| 2/ ≥ 20 y | Perianal mass |
Perianal abscess/ Intravenous antibiotics |
2 m | Perianal | 8 | LRR(Rx) + Inguinal lymph node dissection + CRT | ARMS | II/T2bN1M0 | NED | 4/96 |
| 2/10–19 y | Perianal mass | None | 5 m | Perianal | 6.0 | LRR(R0) + CRT | ERMS | II/T2bN0M0 | NED | 15/120 |
| 1/10–19 y |
Perianal mass;; difficult defecation |
None | 1 m | Perianal | 1.8 | Chemotherapy | ERMS | IV/T2aN1M1(bone) | DOD | 11/11 |
| 2/ ≥ 20 y | Perianal mass | Perianal abscess/Abscessotomy + partial mass ectomy | 4 m | Perianal | 6.2 | APR(R0) + Chemotherapy | ARMS | II/T2bN0M0 | DOD | 11/11 |
| 2/10–19 y | Painful perianal mass | Abscessotomy | 2 m | Perianal | 6.3 | LRR(Rx) + CRT | ERMS | II/T2bN1M0 | NED | 10/24 |
| 2/10–19 y |
Painful perianal mass; Bloody stool |
None | 2 m | Perianal | 7.5 | Chemotherapy | ARMS | IV/T2bN1M1(breast; abdominopelvic) | DOD | 11/11 |
| 2/10–19 y | Inguinal mass | None | 3 m | Perineal | 5.9 | LRR(Rx) + abdominopelvic and inguinal lymph node dissection + CRT | ERMS | II/T2bN1M0 | NED | 12/12 |
| 2/ ≥ 20 y | Painful perianal mass | Abscessotomy | 3 m | Perianal | 10.0 | Chemotherapy | ARMS | III/T2bN1M0 | DOD | 11/11 |
y year, m month, d day, ARMS alveolar rhabdomyosarcoma, ERMS embryonal rhabdomyosarcoma, NED no evidence of disease, DOD died of disease, AWD alive with disease
*LRR local radical resection, APR abdominoperineal resection, CRT chemoradiation therapy, R0 microscopic negative margins, R1 microscopic positive margins, Rx not evaluated