Journal of Pediatric Surgery 53 (2018) 1374–1380

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Journal of Pediatric Surgery

journal homepage: www.elsevier.com/locate/jpedsurg

Local control for vaginal botryoid rhabdomyosarcoma with pre-rectal

transperineal surgical resection and autologous buccal graft vaginal
replacement: A novel, minimally invasive, radiation-sparing approach
Jennifer G. Michlitsch a, Rodrigo L.P. Romao b, Joseph M. Gleason c, Luis H. Braga d, Lisa Allen e,
Abha Gupta f, Armando J. Lorenzo g,⁎
a
Children's Hospital & Research Center, Oakland, CA, United States of America
b
Department of Surgery and Urology, IWK Health Centre, Dalhousie University, Halifax, Nova Scotia, Canada
c
Division of Urology, Department of Surgery, St. Jude Children's Research Hospital and University of Tennessee Health Sciences Center, Memphis, TN, USA
d
Division of Urology, McMaster Children's Hospital; Department of Surgery, McMaster University, Hamilton, Ontario, Canada
e
Section of Pediatric Gynecology, Department of Surgery, The Hospital for Sick Children; Department of Obstetrics and Gynecology, University of Toronto, Toronto, Ontario, Canada
f
Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children; Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada
g
Division of Urology, Department of Surgery, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada

a r t i c l e i n f o a b s t r a c t

Article history: Purpose: Localized vaginal rhabdomyosarcoma (RMS) is associated with a favorable prognosis, but strategies for
Received 30 April 2017 local control remain controversial. The use of radiotherapy (RT) can have important long-term sequelae, while
Received in revised form 5 November 2017 traditional resection involves major reconstructive surgery. We describe a new surgical approach employing a
Accepted 14 November 2017 minimally-invasive resection and immediate reconstruction.
Materials and methods: Records from 4 consecutive patients with localized vaginal RMS managed in 4 major
Key words:
pediatric referral centers were reviewed. All cases were performed with a standardized technique.
Vaginal rhabdomyosarcoma
Local control
Results: Patients were diagnosed at a median age of 24 months. Each underwent a total/subtotal vaginectomy
Vaginectomy with autologous buccal graft vaginal replacement. Final margins were focally positive in one patient and negative
Buccal mucosa in three. None received radiotherapy. To date, all patients have patent buccal neovaginas, enjoy a favorable
Radiation aesthetic result, and remain disease-free at a median follow-up of 35 months.
Children Conclusions: We report 4 cases of localized vaginal RMS successfully treated with a minimally invasive surgical
approach. All patients have avoided radiation and remain disease-free. Our initial data suggest that surgical
local control and immediate reconstruction are feasible and can spare these patients the long-term complications
of RT. Longer follow-up is critical to ensure disease-free survival with a functional, successfully reconstructed
neovagina.
Type of study: Case series.
Level of evidence: Level IV.
© 2017 Elsevier Inc. All rights reserved.

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma
of childhood. Outcomes have improved significantly over the past few
decades with the implementation of a multidisciplinary approach,
which relies on the selective combination of surgical resection, chemo-
therapy, and radiotherapy (RT) [1]. RMS arising in the female genital
tract is rare, accounting for approximately 3.5% of cases, with approxi-
mately half of these cases arising in the vagina [2]. This location is
Abbreviations: ASTRA, Anterior sagittal trans-rectal approach; CAH, Congenital adrenal
hyperplasia; COG, Children's Oncology Group; RT, Radiotherapy; RMS, Rhabdomyosarco-
ma; VAC, Vincristine, actinomycin-D (dactinomycin) and cyclophosphamide.
⁎ Corresponding author at: The Hospital for Sick Children, 555 University Avenue,
Toronto, ON M5G 1X8, Canada. Tel.: +1 416 813 6465; fax: +1 416 813 6461.
E-mail address: armando.lorenzo@sickkids.ca (A.J. Lorenzo).
associated with a favorable prognosis, and is considered to be one of
the most curable forms of RMS [3].
Despite great advances in management, cure often comes at a price.
Addressing the primary tumor with either surgery or radiation can be
associated with important complications. To date, an optimal and pre-
ferred approach for local control is lacking. Owing to the vagina's ana-
tomical location and limited space for dissection within the
prepubertal pelvis, these tumors are usually considered to be difficult
to resect while preserving adjacent organ integrity. Therefore, RT – ei-
ther as external beam or brachytherapy – is often favored. Despite
good oncological outcomes, this strategy can be associated with impor-
tant late effects [4]. Additionally, even many years after pelvic RT for
RMS, surgical repair of these problems can be exceptionally challenging,
and carries a considerable risk for further complications [4].

https://doi.org/10.1016/j.jpedsurg.2017.11.044
0022-3468/© 2017 Elsevier Inc. All rights reserved.
 J.G. Michlitsch et al. / Journal of Pediatric Surgery 53 (2018) 1374–1380
To minimize side effects from local therapy, a response-based ap-
proach to local control was offered to females with vaginal RMS in a re-
cently completed Children's Oncology Group (COG) study for newly-
diagnosed, low-risk RMS. RT was eliminated for patients who achieved
a complete response following chemotherapy and/or surgical resection.
However, higher than expected local failure rates were observed for pa-
tients with Group IIA or III vaginal RMS [5]. This unfortunate study out-
come triggered modifications to the proposed treatment approach, with
the recommendation that local control is critical for these patients. In
accordance with protocols for other primary RMS sites, in girls with
nonresected RMS of the vagina, RT remains mandatory.
Given the frequency and severity of late effects from vaginal RT,
along with possible unforeseen issues that may arise as this patient pop-
ulation ages, it appears prudent to reconsider the value of surgery. Com-
plete surgical resection may allow us to preserve excellent oncological
outcomes while sparing these young patients from radiation while pre-
serving function. With that goal in mind, our group has pioneered and
developed a minimally invasive approach to widely resect affected vag-
inal tissue and perform immediate reconstruction employing autolo-
gous grafts. Herein we present extended follow-up of the first 4 cases
of localized vaginal RMS treated with a novel method of radical
transperineal surgical resection (vaginectomy) followed by vaginal re-
construction using buccal mucosa grafts, without adjuvant radiation.
1. Materials and methods
The complete medical records from 4 patients with localized vaginal
RMS surgically managed between November 2013 and June 2016 were
retrospectively reviewed. All cases were consecutively planned and
conducted with the involvement of the senior author of this report
(AJL). Data including chemotherapy protocol, surgical approach, pathol-
ogy, and current disease status were captured.
1.1. Surgical technique
The buccal mucosa can be procured at the beginning of the case, as
the remainder of the intervention will be done prone, or after resection
is completed and the proper amount of tissue needed is measured.
Nasotracheal intubation is favored to facilitate unhindered access to
the whole oral cavity. The mucosa of each cheek is exposed with the
aid of a mouth retractor and the area infiltrated with bupivacaine and
epinephrine solution. Care is taken to avoid injury to the parotid gland
Fig. 1. Patient positioning for prerectal prone approach. Child is placed on the modified “jackknife” position, with gentle elevation of the pelvis (A). This allows dissection to be sequentially
performed from posterior to anterior, employing the following steps: 1. Define the prerectal space, 2. Posterior retraction of the rectum, 3. Dissection of the posterior and lateral vaginal
walls, and 4. Dissection of the vaginal wall from the periurethral and bladder neck area. Legs are abducted and the perineum is exposed by placing traction tape strips directed laterally and
slightly superior (arrows, B).
1375
duct. Symmetric, bilateral, rectangular-shaped grafts are obtained in
cases where total vaginal replacement is required. A more limited exci-
sion of the labial buccal mucosa of the lower lip was employed in one
case where less tissue was needed. The donor sites are left open, and he-
mostasis is revised prior to extubation.
The patient is then positioned prone, in a modified “jackknife” posi-
tion (Fig. 1A and B). Vaginoscopy is performed to confirm the location of
the tumor while prone. Indwelling Foley catheters are inserted in the
bladder and vaginal cavity to provide traction and aid with identifica-
tion during dissection. A midline (sagittal) incision is made between
the vagina and the rectum, extended posteriorly in an “inverted lamb-
da” fashion around the anterior aspect of the rectum and anal sphincter
complex (Fig. 2A). The incision can also be extended anteriorly, around
the vagina, distal to the hymeneal ring (Fig. 2B).
Dissection starts by developing the prerectal space. Retraction of the
anterior rectal wall delivers a good plane for exposure of the posterior
vaginal wall. Alternatively, the anterior wall of the rectum can be incised
following the ASTRA (anterior sagittal transrectal approach), as de-
scribed for vaginoplasty in patients with congenital adrenal hyperplasia
(CAH) [6] and high urogenital sinus [7]. None of the patients in this se-
ries required incision of the rectal wall, however this technique may
allow for greater exposure in selected cases. The lateral walls of the va-
gina are subsequently developed by blunt dissection, a step that is
straightforward as this is a relatively avascular plane (Fig. 3A). The
most difficult part of the resection involves the anterior wall. The sur-
geon needs to exercise caution developing the space between the vagi-
na and the urethra (Fig. 3B). The vagina can then be mobilized
circumferentially up to the level of the cervix (Fig. 3C).
The extent of the dissection can be tailored based on the location of
the tumor and assessment of margins. Unless felt to be involved (based
on presentation features, vaginoscopy and assessment of margins by
frozen section), the cervix is spared along with unaffected forniceal mu-
cosa near the vaginal dome. The vagina is resected en bloc, and margins
are confirmed intraoperatively by frozen section. The buccal grafts are
then sewn in place, first at the level of the vaginal dome, anteriorly
and posteriorly, and then laterally to each other. Vaginal reconstruction
is concluded by anastomosing the buccal grafts to the incision distal to
the hymeneal ring (Figs. 4 and 5). If a more limited resection is per-
formed, the buccal graft is sewn into place to cover the newly created
defect in the vagina. The perineum is then approximated in the midline,
and a pediatric chest tube tip is left in place for 10–14 days to act as a
mold. The Foley catheter is removed 1–2 after surgery.
1376 J.G. Michlitsch et al. / Journal of Pediatric Surgery 53 (2018) 1374–1380

Fig. 2. Incision in an “inverted lambda” configuration, with the superior aspect defined by the anal verge and the expected location of the anal sphincter (A). As the plane between the vagina
and rectum is developed, exposure is gained by gentle upwards traction provided by a narrow Deaver retractor (arrow, B) and lateral retraction with a Beckmann–Adson or similar
laminectomy self-retaining retractor (bidirectional arrow, B). Note that the hymeneal ring and vulva have not been incised (arrowhead).

2. Results response to chemotherapy, and this information can provide guidance

The patients presented at a median age of 24 months (range 11–
30 months) and were treated at 4 large pediatric academic centers
(Hospital for Sick Children, McMaster Children's Hospital, IWK Health
Centre and St. Jude Children's Research Hospital). In all cases, preopera-
tive evaluation included vaginoscopy and magnetic resonance imaging.
A critical technical point during these assessments is documentation of
extent and location of the tumor, as vaginal RMS may have a dramatic
regarding extent and location of the resection.
2.1. Patient #1
This child presented at 11 months of age after passing tumor frag-
ments per vagina. Vaginoscopy demonstrated involvement of the distal
2/3 of the vagina, without gross involvement of the cervix. Pathology
demonstrated embryonal RMS. Imaging studies, inguinal lymph node

Fig. 3. Steps for vaginal dissection: With assistance of a filled Foley catheter balloon, the vaginal wall is circumferentially developed from the introitus up to the cervix area. Note
preservation of the anal sphincter muscles (arrowhead, A). The urethra is identified and preserved, aided by palpation of a separate indwelling Foley catheter in the bladder (B). The
specimen is manipulated with multiple silk sutures placed at regular intervals along the distal dissection margin, following a similar approach to dissection of an imperforate anus
with the Peña procedure. The proximal margin can be defined up to the level of the cervix (dotted line, C).
 J.G. Michlitsch et al. / Journal of Pediatric Surgery 53 (2018) 1374–1380 1377

Fig. 4. Steps for buccal vaginoplasty: Bilateral rectangular (symmetric) grafts are harvested from both cheeks. The anterior graft is secured in place at the proximal margin (arrow, A) and at
the introitus level. A second graft of identical proportions is the placed on top and sutured to the first one on their lateral aspects, while the superior end is sutured to the forniceal margin
(B). Note the distal aspect of the second graft at the level of the introitus (arrowhead). During harvest, the grafts are procured with the length (b, c, b` and c`) planned to comfortably bridge
the distance between the fornix/cervical margin and the distal dissection margin (at or near the hymeneal ring, C). Similarly, the width (a and a`) should accommodate for approximately
half the circumference of the fornix/cervical margin and the corresponding distal edge. The first (anterior) graft is secured at the fornix and introitus (a to a`), followed by the posterior one,
with the edges sutures to each other (b to b` and c to c`).

biopsy and bone marrow biopsies were negative for metastatic disease.
The patient underwent an upfront attempted partial vaginectomy
which was converted to a total vaginectomy owing to multiple positive
margins on frozen section. Final pathology demonstrated focal anaplasia
with a discrete, small microscopic positive margin. She was treated as a
stage 1 group IIa tumor with VAC therapy, as per ARST0531 [8], Arm A,
with a cumulative cyclophosphamide dose of 16.8 g/m 2 and no RT. After
38 months of follow-up, she remains disease-free with no evidence of
local or distant recurrence. Two surveillance vaginoscopies have been
performed, demonstrating no evidence of recurrence and widely patent,
well-healed grafts without stenosis.
2.2. Patient #2
She presented at 30 months of age with an exophytic vaginal mass,
confirmed to be botryoid RMS on biopsy. She was treated as a stage 2,
group III tumor with VAC therapy as per D9803 Regimen A [9], receiving
a cumulative cyclophosphamide dose of 30.8 g/m 2, and no RT. At
12 weeks, vaginoscopy showed a good response with residual lesions.
At 24 weeks, she underwent surgical resection and reconstruction.
Pathology revealed rhabdomyoblasts with negative margins. The pa-
tient had a superficial dehiscence of the perineal body at 15 days postop,
which healed nicely by secondary intention. She remains disease-free at
41 months following diagnosis, with no evidence of recurrence on phys-
ical exam and MRI.
2.3. Patient #3
This girl presented at 24 months of age with vaginal bleeding. A
Doppler ultrasound demonstrated a well-vascularized vaginal mass,
found to be botryoid RMS on biopsy. Workup, including imaging studies
and bone marrow evaluation, was negative for metastatic disease, and
she was treated with VAC therapy as per COG ARST0331 [10] protocol.
She received a cumulative cyclophosphamide dose of 4.8 g/m 2, and
did not receive RT. At week 16, repeat vaginoscopy showed significant
residual tumor burden around the proximal anterior and lateral walls
of the vagina. At week 20 she underwent a total vaginectomy with re-
construction. Final surgical margins were negative for tumor involve-
ment. She remains disease-free at 43 months with no evidence of
recurrence.

Fig. 5. Diagram illustrating the steps followed during vaginectomy and buccal vaginoplasty. The vagina (Vg) is dissected from posterior to anterior (1, 2, 3), preserving the cervix (Cx),
uterus (Ut), bladder (Bl) and urethra (A). The vagina is dissected and removed intact using a Foley catheter for guidance (B). The buccal mucosa grafts are sutured in place at the
surgical bed (C).
1378 J.G. Michlitsch et al. / Journal of Pediatric Surgery 53 (2018) 1374–1380
2.4. Patient #4
She presented at 25 months of age with a protruding vaginal mass,
managed initially as a urethral prolapse, but eventually biopsied be-
cause of growth, despite lack of symptoms, and found to be botryoid
RMS. Workup, including imaging studies and bone marrow evaluation,
was negative for metastatic disease, and she was treated with VAC ther-
apy as per RMS 13 low risk subset 2 protocol. Her cumulative cyclophos-
phamide dose was 14.7 g/m 2. She did not receive RT. Her EUA after
week 6 showed diminished tumor size, and repeat evaluation at week
11 showed no evidence of tumor, with multiple directed biopsies
being negative for residual disease, indicating a complete response.
After week 15 of therapy, she underwent an anterior vaginal resection
of roughly 180 degree circumference, from the urethral meatus to just
proximal to the cervix, and frozen sections were negative for tumor. A
lower lip labial buccal graft was used for her vaginal reconstruction.
Final surgical margins were negative for tumor involvement.
Vaginoscopy 3 months postoperatively showed a well healed graft
without stenosis, and no evidence of recurrence. She remains disease-
free at 16 months with no evidence of recurrence.
Although it is too early to claim a functional vaginal replacement,
postoperative examinations demonstrate a favorable cosmetic result
(Fig. 6A–C) and good graft take without stenosis (Fig. 6D).
3. Discussion
Localized vaginal RMS is associated with a favorable prognosis, with
overall survival rates of 88%–100% [5]. This success has been achieved
Fig. 6. Postoperative appearance during surveillance after surgery. Note normal appearance of the external genitalia with a well-hidden, aesthetically favorable surgical Scar (A, B and C).
Appearance of the healed graft during vaginoscopy (D, arrowhead at cervix).
using a multimodal approach, combining systemic chemotherapy and
aggressive local control with surgical resection or RT. However, long-
term survivors face several therapy-related sequelae. These include vag-
inal stenosis and fistulas, musculoskeletal hypoplasia, ovarian failure,
uterine growth and functional abnormalities, and psychologic disorders
[4]. Even with brachytherapy, vaginal stenosis appears to be a relatively
common occurrence in young girls in follow-up [3,11]. Furthermore,
most patients with vaginal RMS present before 2 years of age, which
makes them especially susceptible to long-term effects of RT. As ad-
vances in therapy have allowed patients to survive, while this group of
survivors ages, it is likely that other long-term consequences from ther-
apy will become apparent.
To minimize late effects of therapy in these typically young individ-
uals, attempts have been made to minimize radiation to the genitouri-
nary tract. To that end, two recent COG trials for localized vaginal
embryonal RMS planned to delay or eliminate RT altogether if a com-
plete response was achieved with or without delayed primary resection
[12–15]. According to protocols D9602 [16] and ARST0331 [10], girls
with vaginal tumors with completely resected localized disease (Clinical
Group I) did not receive RT. Those with incomplete upfront resections
(Group IIA or III on D9602 and Group III on ARST0331, without lymph
node involvement) received RT only if there was evidence of residual
gross or microscopic tumor after chemotherapy. Those with vaginal pri-
maries who were rendered histologically disease-free after 12 weeks of
chemotherapy with or without delayed primary resection, were spared
RT altogether. The 5-year cumulative incidence of local recurrence for
patients with Group IIA or III N0 vaginal RMS treated on D9602 was
26%, which compared unfavorably with the 14% 5-year cumulative
 J.G. Michlitsch et al. / Journal of Pediatric Surgery 53 (2018) 1374–1380
incidence of local recurrence for patients with Group IIA or III N0 low-
risk RMS at sites other than the vagina. Subsequently, during a sched-
uled interim analysis of ARST0331, a higher than expected rate of local
recurrences was also noted, largely accounted for by patients with
Group III vaginal tumors. Accrual to the study was suspended, and
later reopened with modified local control guidelines. Ultimately, the
estimated 2-year cumulative incidence of local recurrence was 43% for
patients with embryonal RMS of the female genitourinary tract treated
on ARST0331. The high local failure rates appeared to be related to the
attempt to delay or avoid local RT in those patients with nonresected
vaginal RMS, and in those who also received a lower dose of cyclophos-
phamide. Thus, local therapy guidelines have been amended to include
RT for patients with Group II or III vaginal RMS in a manner similar to
that for other primary sites.
Despite the current guidelines, concerns remain regarding the con-
siderable long-term toxicity of RT for vaginal tumors. Aggressive surgi-
cal local control at this site offers the advantage of sparing these
young patients RT-associated complications. Patients who developed
an isolated local recurrence in the aforementioned studies were amena-
ble to salvage therapy with excellent overall survival [5]. Hence, even if
a surgical strategy for local control was unsuccessful, these patients
could still be rescued with other treatment strategies including the ini-
tially omitted radiation. Nevertheless, surgical resection is not without
its own possible complications, including wound infections, fistulas,
and stenosis [17]. Thus, a surgical alternative must fulfill important re-
quirements: be minimally invasive, be nonmutilating, preserve function
and be aesthetically and functionally acceptable.
Complete vaginal resection with negative margins is an appealing al-
ternative to RT for local control. The paucity of reports on surgical treat-
ment for this condition underscores the difficult access with traditional
surgical techniques and absence of an ideal tissue to replace the prepu-
bertal vagina. Customarily, vaginoplasty techniques are aimed for older
patients with vaginal agenesis and Mullerian abnormalities, relying on
serial dilation, local flaps or bowel interposition. All of these options
can be associated with suboptimal functional outcomes and important
morbidity [18–20].
Buccal mucosa grafts are quite versatile. In genitourinary surgery,
they are widely employed for urethral reconstruction in boys and
adult men with strictures and failed hypospadias repairs, with good re-
sults [21–23]. Evidence suggests that the grafts retain favorable charac-
teristics over time, and adapt well to rapid growth demands such as
those imposed by puberty [24]. There are also case series reporting suc-
cessful use for vaginoplasty [25–28], with promising the outcomes. For
example, in a long-term follow-up study of 38 females, 32 were sexually
active and satisfied with the results [29]. Harvesting the graft is straight-
forward and associated with minimal morbidity. Patients are able to eat
a normal diet soon after surgery, and the donor site heals with a well-
concealed scar [30]. Moreover, buccal grafts have a striking similarity
to the vaginal tissue it aims to replace.
The herein presented children were treated in different centers, with
varying cumulative doses of alkylating agent (cyclophosphamide) and
with different timing of surgery. Despite the heterogeneity in therapy,
certain themes remain consistent. Our preference is always to consider
upfront resection in order to render patients Group 1. However, if tumor
bulk precludes confidence in an R0 resection, neoadjuvant therapy is
administered. Regardless of response to chemotherapy, that is radiolog-
ical PR or CR or even pathological CR based on biopsy, all patients were
still taken for complete surgical resection. The early failures in the ab-
sence of definitive local control documented by ARST0331 behoove us
to commit to an R0 resection if ever avoidance of RT is to be tested. At-
tempts at reducing RT post DPE are being explored [31].
As with any new procedure, long-term follow-up to assess oncolog-
ical and functional outcomes is mandatory. Even though our patients
have been followed beyond completing chemotherapy and outside of
the expected timeframe for local recurrence, it is unreasonable to de-
clare equivalency or superiority over established protocols. Our paper
1379
does not represent a new standard of care, but rather the presented sur-
gical intervention does hold promise as a viable option with minimal
aesthetic impact and reasonable conceptual basis for long-term favor-
able functional outcomes, particularly in infants with localized disease.
Although randomized trials may not be feasible owing to the highly-
specialized techniques required, further multicenter prospective evalu-
ation is required to understand the role that vaginectomy has in the
treatment of females with RMS. While newer RT strategies may achieve
focused doses to the tumor, there is unavoidable exposure to adjacent
structures, notably the cervix, rectum and urethra. We hope that this re-
port will generate interest and potentially be the first step towards in-
clusion in future investigational protocols.
4. Conclusion
Transperineal prerectal vaginal resection with autologous buccal
mucosa graft vaginoplasty appears to be a promising option for local
control in vaginal RMS. Our data suggest that this novel approach merits
further evaluation and comparison against other strategies for local
control.
Acknowledgment
We gratefully acknowledge the contribution of Michael Corrin BFA,
BA, Hons BSc, MScBMC, CMI, Assistant Professor, Biomedical Communi-
cations, University of Toronto for his expertise, recommendations, and
artwork for the illustrations included in this manuscript.
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