Accepted Manuscript

Rhabdomyosarcoma of the vagina in an adolescent girl

Geetha Narayanan, MD, DM, Varun Rajan, MD, Ridu Kumar, MD, Lali V. Soman

PII: S1083-3188(17)30034-7
DOI: 10.1016/j.jpag.2017.05.008
Reference: PEDADO 2126

To appear in: Journal of Pediatric and Adolescent Gynecology

Received Date: 29 January 2017

Revised Date: 18 May 2017
Accepted Date: 25 May 2017

Please cite this article as: Narayanan G, Rajan V, Kumar R, Soman LV, Rhabdomyosarcoma of the
vagina in an adolescent girl, Journal of Pediatric and Adolescent Gynecology (2017), doi: 10.1016/
j.jpag.2017.05.008.

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Type of submission – Case Report

Title – Rhabdomyosarcoma of the vagina in an adolescent girl

Authors
1. Geetha Narayanan, MD, DM

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Professor and Head of Medical Oncology
2. Varun Rajan, MD

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Senior Resident, Dept. of Medical Oncology
3. Ridu Kumar, MD

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Senior Resident, Dept. of Medical Oncology
4. Lali V Soman
Medical Officer, Dept. of Medical Oncology

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Institute -Regional Cancer Centre, Trivandrum 695011, Kerala, India
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Running title – RMS vagina

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Verified that all authors have read and approved the manuscript and there is no conflict of
interest or any financial disclosures.
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Address for correspondence

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Dr. Geetha Narayanan, MD, DM

Professor and Head, Department of Medical Oncology
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Regional Cancer Centre, Trivandrum 695011, Kerala, India

Email – geenarayanan@yahoo.com
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Phone 91 9447500920
Fax 914712443498
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Title – Rhabdomyosarcoma of the vagina in an adolescent girl

Abstract

Background-

Gynaecologic neoplasms are rare in children and represent only <5% of all childhood tumors.

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Rhabdomyosarcoma (RMS) of the female genital tract of children accounts for only 3.5% of

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the cases.

Case -

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A 16 year old adolescent presented with a proliferating growth and foul smelling discharge

from vagina, which on biopsy was diagnosed as RMS. She received chemotherapy and

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radiation to the primary site. She is alive in remission at 8 years.
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Summary –
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RMS of the vagina is a rare, but highly curable tumor in adolescent girls. Any abnormal

vaginal bleeding in girls should be promptly investigated through the use of pelvic
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examination and appropriate imaging. An organ preserving approach should be considered in

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these patients.
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Key words - RMS; vagina; adolescent

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Introduction

Gynaecologic neoplasms are rare in children and represent only <5% of all childhood tumors.

[1] Rhabdomyosarcoma (RMS) of the female genital tract of children accounts for only

3.5% of the cases, of which about half arise from vagina. [2] A population based study on 67

female children with genitourinary RMS showed that vagina was the primary site in 68.4% of

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children between 0-9 years, however it contributed only 6.9% in the 10-19 year age group.

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[3] We present the case of a 16 year old adolescent girl who presented with a proliferating

growth in the vagina and was diagnosed as RMS and discuss the treatment of this rare entity.

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Case Report

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A 16 year old girl presented with presented with vaginal discharge and a mass protruding
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from the vaginal orifice noted a week back. A pervaginal examination showed a 12x10cm
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friable necrotic foul smelling mass from the introitus. A computed tomogram (CT) of the

pelvis showed a large heterogeneously enhancing mass lesion measuring 10x9x8 cm arising
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from the region of vagina and anal canal and extending exophytically inferiorly. The lesion
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was infiltrating into the anal canal, rectum and cervix. (Figure 1) She underwent a biopsy

from the lesion. Histopathological examination showed a neoplasm composed of markedly

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pleomorphic cells with moderate amount of eosinophilic cytoplasm, vesicular nucleus with
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prominent nucleoli with extensive areas of necrosis. On immunohistochemistry, the tumor

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cells were strongly positive for desmin and myogenin. A diagnosis of rhabdomyosarcoma

was made. A CT scan of the chest, bone scan and bone marrow were normal. She received

combination chemotherapy with vincristine, dactinomycin, doxorubicin and

cyclophosphamide for 18 months. Radiation to the primary site was given between 9-12th

week at a dose of 45Gy/25#. At present she is alive in complete remission at 96 months.

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Discussion

Rhabdomyosarcoma accounts for 3-4%of all childhood cancers and 5-15% of all childhood

solid tumors, with an annual incidence of 4.3cases per million in children under the age of 20

years.[4] Genitourinary tract forms the primary site in 15-20% of all pediatric RMS, the

common sites being prostate, bladder, paratesticular region followed by vagina and uterus.

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In a study at St. Jude Children Research Hospital, there were only18 children with vaginal

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tumors among 4485 patients under 21 years over a period of 39 years.[5] RMS was the

commonst followed by germ cell tumor and clear cell adenocarcinoma. Thirteen children had

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RMS, 10 botryoid subtype and 3 embryonal subtype, with a median age at presentation of

3.7 years.[5]

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Vaginal tumors in children clinically present with abdominal pain or mass, bloody discharge
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or mass protruding from vagina or a genital ulcer. [5] RMS belongs to the group of small blue
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round cell neoplasms and shows skeletal muscle differentiation. The botryoid subtype seen in

vaginal primary has typical grape like appearance due to spindle cells pushing up beneath the
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mucosa in the polypoid mass. Our patient also had a similar presentation.
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The management of vaginal RMS involves a multidisciplinary approach with chemotherapy

and local control with surgery or radiotherapy. Although upfront radical surgery was used in
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the first IRS studies, the treatment paradigm has shifted now towards organ preservation and
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includes initial biopsy, neoadjuvant chemotherapy, local control with surgical resection
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and/or radiation followed by further systemic chemotherapy. The most common

chemotherapeutic agents used are vincristine, dactinimycin, doxorubicin, cyclophosphamide

and recently ifosphamide and etoposide.

Local treatment is planned between 9th and 12th week of chemotherapy when there is a

cytoreduction. However, the optimal locoregional treatment approach for patients with

vaginal RMS remains controversial since the tumor usually cannot be surgically resected if
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organ integrity is to be preserved. The surgical approach is variable and includes

hysterectomy, vaginectomy and rarely anterior pelvic exenteration.[5] Surgical

complications included recto-vaginal fistula, vesico-vaginal fistula and urinary incontinence.

At a median follow up of 21.4 years, 84% were disease free.[5] Andrassy et al. reviewed 25

years of publications in vulvovaginal RMS and confirmed the role of organ-preserving

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approach. [6] The rate of radical surgery decreased from 100% in 1972–78 to 13% in 1988–

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96. Intergroup RMS Study Group (IRS) /Children’s Oncology Group (COG) clinical trials

have routinely included radiation for local control of tumors at most primary sites that are not

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resected prior to chemotherapy. Walterhouse et al reviewed the locoregional treatment and

outcome for patients with localized RMS of the vagina on the two most recent COG low-risk

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RMS studies and reported a higher local failure rates than expected for patients with Group
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IIA or III vaginal RMS. The local therapy guidelines for vaginal primary was thus amended
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to include radiation for patients with Group II or III vaginal RMS similar to that for other

primary sites.[7]
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Recently, Arndt et al evaluated the factors affecting outcome in patients with RMS of the
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female genital tract treated on IRS protocols I–IV.[2] The rate of hysterectomy decreased

from 48% in IRS-I/II to 22% in IRS-III/IV and the overall 5-year survival was 82%. Analysis
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of prognostic factors revealed that an age of 1–9 years at the time of diagnosis, noninvasive
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tumors, and the use of IRS-II or IRS-IV treatments based on VAC regimen, a conservative
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surgical approach, and the use of radiation therapy for selected patients were associated

significantly with better outcome.[2] The present case was also treated with chemotherapy

and radiation.

Although the overall survival of patients with vaginal RMS has improved, little is known

about the late effects of treatment in long-term survivors. Spunt et al. reviewed the spectrum

and severity of late effects in female survivors of pelvic RMS and found significant late
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effects that reduced the quality of life and the functional capacity. These included vaginal

stenosis and fistulas, uterine growth and functional abnormalities, ovarian failure,

musculoskeletal hypoplasia and psychologic disorders.[8] The prognosis of vulvovaginal

RMS is excellent; 25 of 27 girls with vulvovaginal tumors survived >5 years, with an 88%

preservation rate for the uterus. All patients were treated with chemotherapy and 11 received

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additional radiotherapy. [6]

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Our patient who has survived 8 years without relapse also confirms the excellent prognosis of

RMS arising in the vagina, and emphasize the efficacy of a conservative approach to local

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therapy.

Conclusions

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Rhabdomyosarcoma of the vagina is a rare, but highly curable tumor in adolescent girls. Any
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abnormal vaginal bleeding in girls should be promptly investigated through the use of pelvic
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examination and appropriate imaging. Biopsy and primary chemotherapy constitute the

current initial treatment for vaginal RMS. An organ preserving approach should be
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considered when local treatment is planned and is similar to that used for other primary sites.
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Surgical resection should reserved for truly persistent or recurrent disease.

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References

1. Hassan E, Creatsas G, Michalas S. Genital tumors during childhood and adolescence:

a clinical and pathological study of 71 cases. Clin Exp Obstet Gynecol. 1999;26:20

2. Arndt CA, Donaldson SS, Anderson JR et al. What constitutes optimal therapy for

patients with rhabdomyosarcoma of the female genital tract? Cancer, 2001; 91 (12)

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:2454

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3. Kirsch CH, Goodman M, Esiashvili N. Outcome of female pediatric patients

diagnosed with genital tract rhabdomyosarcoma based on analysis of cases registered

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in SEER database between 1973 and 2006. Am J Clin Oncol. 2014; 37(1): 47.

4. Ries LAG, Harkins D, Krapcho M, Mariotto A, Miller BA, Feuer EJ, Clegg L, Eisner

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http://seer.cancer.gov/csr/1975_2003. based on November 2005 SEER data

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5. Fernandez-Pineda I, Spunt SL, Parida L et al. Vaginal tumors in childhood: the

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experienceof St. Jude Children’s research Hospital. J Pediatr Surg. 2011; 46 (11):

2071.
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6. Andrassy RJ, Hays DM, Raney RB et al. Conservative surgical management of

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vaginal and vulvar pediatric rhabdomyosarcoma: A report from the Intergroup

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Rhabdomyosarcoma Study III. J Pediatr Surg.1995;30 (7):1034.

7. Walterhouse DO, Meza JL, Breneman JC et al. Local control and outcome in children

with localised vaginal rhabdomyosarcoma: A report from the Soft Tissue sarcoma

Committee of the Children’s Oncology group. Pediatr Blood Cancer. 2011;57 (1): 76

8. Spunt SL, Sweeney TA, Hudson MM et al. Late effects of pelvic rhabdomyosarcoma

and its treatment in female survivors. J Clin Oncol. 2005;23 (28):7143.

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Legend

Figure 1

CT scan of the pelvis showing a large heterogeneously enhancing lobulated mass

lesion arising in the vulvovaginal region involving distal anal canal.

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