Pediatric radical abdominal trachelectomy for anaplastic embryonal rhabdomyosarcoma of the uterine cervix: an alternative to radical hysterectomy

Abstract

Rhabdomyosarcoma arising in the female genital tract carries 5-year survival in excess of 80%, but lifelong infertility may be a consequence of local control strategies. We present the technique and outcome for a fertility-sparing, radical abdominal trachelectomy in a 12-year-old girl with anaplastic, embryonal rhabdomyosarcoma involving the uterine cervix. The patient had presented to our center after the piecemeal resection of a uterine cervical mass; because of concern about microscopic residual disease, we classified her as group II-A according to the Intergroup Rhabdomyosarcoma Study system. Staging studies excluded the presence of distant disease. The patient received 4 cycles of multiagent chemotherapy and then underwent radical abdominal trachelectomy, with removal of the uterine cervix, parametria, vaginal cuff, and regional lymph nodes. Microscopically, the specimen showed treatment effect and no residual tumor. Regional nodes were negative. Radical abdominal trachelectomy, which has not been previously reported for rhabdomyosarcoma, has appeared to secure local disease control in this case while preserving the patient's future fertility potential. In properly selected cases of rhabdomyosarcoma of the uterine cervix, where involvement of the uterus proper is not present, radical abdominal trachelectomy may be an attractive fertility-sparing alternative to radical hysterectomy.

FIG. 1

The intent of this radical abdominal trachelectomy was to resect the cervix, upper 1–2 cm of the vagina, parametrium, and paracolpos in a similar manner to a type II radical abdominal hysterectomy while sparing the uterine corpus.

FIG. 2

The uterine fundus and cervix with parametria and upper vagina have been resected with the only remaining blood supply from the bilateral uteroovarian ligaments. The curved clamps demonstrate the level of the internal cervical os where the abdominal trachelectomy incision will be made. The Wertheim clamp has been replaced to occlude the vaginal margin.

FIG. 3

Specimen of radical abdominal trachelectomy.

FIG. 4

The remaining uterine fundus is demonstrated without evidence of ischemia.

FIG. 5

At the end of the procedure, the uterus was reconstructed to the upper vagina and the pelvic anatomy restored. The fundus was viable without evidence of ischemia with the remaining blood supply from bilateral uteroovarian ligaments.

FIG. 6

Sagittal T2-weighted magnetic resonance image demonstrating the healed uterine-vaginal anastomosis.