Evidence
Direct evidence regarding local control for tumors of the cervix are
difficult to find since studies often combine patients with uterine
tumors. However, it has been observed that patients with cervical RMS
had a trend towards a superior survival rate compared to those with
vaginal tumors in the North American SEER database, possibly due to a
higher percentage of patients with favorable histology and lower stage
tumors.3 Residual RMS of the cervix after induction
chemotherapy does not seem to require a more radical surgical approach
than RMS of the vagina. In the International data analysis only 12% of
patients with tumors at the cervix were treated with radical surgery and
this site had a local control rate of 88%.4Similarily, Martelli et al. demonstrated that the outcome for patients
with uterus RMS can be favorable with a high rate of preserved
organs.7 They demonstrated that 10 of 11 girls with
uterine RMS survived and eight of them retained their uterus. Patients
with partial resection of the cervix (n=3) or chemotherapy alone (n=3)
had little or no local sequelae, but patients undergoing EBRT were found
to have long-term functional sequelae. In addition, the local disease
management may have had an impact on the outcome since more radical
surgical options are available for uterine tumors.3 In
contrast to the SEER data, Martelli et al. showed that these patients do
not do better with more aggressive resections since 65% of patients
achieved complete clinical and radiological remission with chemotherapy
alone.7 The good outcomes of these children occurred
with minimal local control. Nasioudis and colleagues analyzed 70
patients with cervix RMS and also found that there is no survival
benefit of extensive surgery since conservative tumor excision can
provide adequate local control.16
Trachelectomy has been described as an organ-preserving procedure in a
small number of children with RMS of the female genital tract. Kayton et
al. demonstrated that trachelectomy is technically
feasible.24 This has also been reported by other
groups.15, 25 A major advantage of trachelectomy seems
to be the long-term obstectric outcome. Currently, there is no pediatric
data on this issue, but trachelectomy is used in adult women with
cervical cancer as a fertility preserving procedure for tumors
< 2 cm. It has been shown that these fertility preserving
procedures resulted in an improved obstetric outcome without
compromising oncological safety. The recurrence rate in the literature
after this approach for cervical cancer is less than 5%, and 37% of
the women became pregnant with a live birth rate of 67%. Assisted
reproductive technologies may also assist these patients that wish to
have children.26
Complications of RT at this site appear to be more problematic. For
children with tumors near the cervix treated with RT, the OS is similar
to vaginal RT. However, for children with tumors more near the uterus,
BT of the uterus and cervix with placement of a BT tube into the
endometrium can lead to a non-functioning uterus. This complication has
also been observed in EBRT of the uterus with more than 20-30
Gy.26