Embryonal rhabdomyosarcoma (Sarcoma botryoides) of the uterine cervix is an uncommon entity. Because of extreme rarity its discussion has mainly been in the light of individual case reports. We report a case of a 14-year old female who presented with irregular vaginal bleeding and cervical polyp. Her biopsy specimen confirmed sarcoma botryoides and she underwent abdominal hysterectomy with wide excision of vaginal cuff after a multidisciplinary consultation

Chakrabarty, Salil Bindu

Ray, Jayanta

Sarkar, Atanu

Tigga, Maureen Prativa

English

International Journal of Reproduction, Contraception, Obstetrics and Gynecology

   http://dx.doi.org/10.18203/2320-1770.ijrcog20150132                                                                                   Volume 4 · Issue 3    Page 927 International Journal of Reproduction, Contraception, Obstetrics and Gynecology Chakrabarty SB et al. Int J Reprod Contracept Obstet Gynecol. 2015 Jun;4(3):927-929 www.ijrcog.org pISSN 2320-1770 | eISSN 2320-1789 Case Report Sarcoma botryoides in a 14 year old girl: a rare case Salil Bindu Chakrabarty, Atanu Sarkar, Jayanta Ray, Maureen Prativa Tigga*             INTRODUCTION Embryonal rhabdomyosarcoma (ERM) originates from embryonal mesoderm and arises under the mucosal surface of body orifices such as the vagina, bladder and cervix. ERM is the most common soft tissue sarcoma in children and young adults and accounts for 4% to 6% of all malignancies in this age group.1,2 While affecting the genitourinary tract, the ERM is most commonly manifested in the vagina,3 and it usually presents before the age of 4 years.2,4 Only 0.5% of  ERM in females affect the cervix,4 and is usually occurs in the second decade of life as seen  in our patient, however it has also been reported in babies of 5 months age.5 Few cases of ERM have been reported in the women older than 40 years.6-9 A subset of ERM are grossly polypoid and are descriptively referred to as botryoid (grape like), which was a salient feature of our case too.  CASE REPORT We report here a case of 14 year old school student who had attained menarche 3 months back, and had been experiencing irregular bleeding per vagnium since then. She also felt some mass coming out through her introitus on straining, for the same duration. The mass was fleshy, gradually increasing in size and often associated with foul smelling mucoid discharge. There was no significant past medical or surgical history and no family history of any malignancy. On examination, patient had mild pallor and there was no lymphadenopathy. Her secondary sexual characters were normally developed. On local examination, multiple grape like lesions were protruding through the introitus. The lesions had smooth glistening surface smeared with mucosanguinous secretion. Per vaginal examination revealed a spacious vagina occupied by the lesion, however the vaginal mucosa was free. A normal sized uterus with normal adnexa was palpated and the growth seemed to arise from the cervix. Rectal mucosa was free on per rectal examination. In the course of local examination few fragments from the growth got detached and these were submitted for histopathological examination. Ultrasonography of the patient showed utero cervical growth and CECT abdomen revealed a well-defined 8.2 x 8 x 7 cm mass lesion in the lower part of body of uterus and cervix. There was no evidence of pelvic lymphadenopathy and parametrium was reported normal. The histological examination of the lesion was consistent with an embryonal rhabdomyosarcoma (botryoide type). After a multidisciplinary consultation Department of Obstetrics & Gynaecology, Agartala Government Medical College & GB Pant Hospital, Agartala, Tripura, India   Received: 21 April 2015 Accepted: 09 May 2015  *Correspondence: Dr. Maureen Prativa Tigga, E-mail: maureentigga@gmail.com Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. ABSTRACT Embryonal rhabdomyosarcoma (Sarcoma botryoides) of the uterine cervix is an uncommon entity. Because of extreme rarity its discussion has mainly been in the light of individual case reports. We report a case of a 14-year old female who presented with irregular vaginal bleeding and cervical polyp. Her biopsy specimen confirmed sarcoma botryoides and she underwent abdominal hysterectomy with wide excision of vaginal cuff after a multidisciplinary consultation.   Keywords: Embryonal rhabdomyosarcoma, Sarcoma botryoides DOI: 10.18203/2320-1770.ijrcog20150132 Chakrabarty SB et al. Int J Reprod Contracept Obstet Gynecol. 2015 Jun;4(3):927-929 International Journal of Reproduction, Contraception, Obstetrics and Gynecology                                       Volume 4 · Issue 3    Page 928 and counseling the patient and her family, abdominal hysterectomy with wide excision of vaginal cuff along with omental and pelvic lymph node sampling was performed. Later the specimen report showed disease free surgical margins and lymph nodes. Her post-operative period was uneventful and patient was referred to regional cancer centre for chemotherapy.  Figure 1: Grape like mass protruding through introitus.   Figure 2: Examination under anaesthesia revealing extensive growth.   Figure 3: Hysterectomised specimen showing uterus along with sarcoma botryoides of the cervix.  DISCUSSION Sarcoma botryoides in the cervix is rare and accounts for about 10% of all RMS cases.10 Case reports have been the major source of information regarding this topic. It typically occurs in young females and most commonly affected age group is between 12-26 years.11,12 Clinically sarcoma botryoides can present as abnormal vaginal bleeding, prolapsing mass per vaginum or an abdominopelvic mass. Histological features include undifferentiated mesodermal and striated muscle cells with fibromyxomatous stroma and a distinct “cambium layer” beneath the epithelium which is characteristic of sarcoma botryoides.13 The available treatment options for sarcoma botryoides include radical surgery, fertility sparing surgery, chemoradiation and combined approach. The optimal management of these tumors is still not codified owing to their rarity; however, combined approach to treatment may result in better outcome.14 According to some authors, the prognosis of cervical sarcoma botryoid is more favorable than other genital RMS, particularly when the tumor arises as a single polypoid lesion and the polyp is completely removed.15,16 Similarly in another study Daya and Scully reported favorable outcome for cervical botryoid in comparison to the vaginal counterpart and demonstrated  that 3 of the 13 patients treated with fertility-sparing surgery followed by chemotherapy had results comparable  to those treated with more radical surgery with or without chemotherapy.17 However a contrast report was shown by Gruessner et al. in their study, stating higher survival rates and better prognosis for vaginal lesions. They demonstrated 96% survival rate for vaginal lesions versus 60% for cervical botryoid.18 Regarding chemotherapy, the most widely used regimen is vincristine, d-actinomycin and cyclophosphamide (VAC). Gordon et al reported that between 6-12 cycles of VAC there was good chance of return of menstruation and reproductive function.19 Considering our patient, following a multidisciplinary consultation, abdominal hysterectomy with wide excision of vaginal cuff was performed. The decision for surgery was taken keeping in mind the poor patient profile likely to be lost to follow up. Literature too has stated a similar case of a 16 year old Moroccan girl11 with sarcoma botryoides where the lesion persisted even after chemotherapy and subsequently she had to undergo surgery. The risk of recurrence of the disease, poor patient compliance and follow up, and the patient party consenting for surgery were the main factors which prompted us to take a surgical approach. The post-operative period of the patient was uneventful and she was discharged on 10th day following surgery. CONCLUSION Sarcoma botryoides of the cervix is a rare disease and it presents as a cervicovaginal polyp in the 2nd decade of Chakrabarty SB et al. Int J Reprod Contracept Obstet Gynecol. 2015 Jun;4(3):927-929 International Journal of Reproduction, Contraception, Obstetrics and Gynecology                                       Volume 4 · Issue 3    Page 929 life. Histopathology is diagnostic but the treatment protocol is not codified due to its rarity. Surgery along with chemo radiation may results in prolonged survival, but requires more evidence. Funding: No funding sources Conflict of interest: None declared Ethical approval: Not required REFERENCES 1. Fletcher C, Bridge J, Hogendroom P, Mertrens F. WHO classification of tumours of soft tissue and bone. In: WHO, eds. WHO Classification. Lyon: IARC; 2013. 2. Behtash N, Mousavi A, Tehranian A, Khanafshar N, Hanjani P. 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Eur J Pediatr. 2004;163:452-6. 19. Gordon AN, Montag TW. Sarcoma botryoides of the cervix: excision followed by adjuvant chemotherapy for preservation of reproductive function. Gynecol Oncol. 1990;36:119-24.     DOI: 10.18203/2320-1770.ijrcog20150132 Cite this article as: Chakrabarty SB, Sarkar A, Ray J, Tigga MP. Sarcoma botryoides in a 14 year old girl: a rare case. Int J Reprod Contracept Obstet Gynecol 2015;4:927-9. 

Sarcoma botryoides in a 14 year old girl: a rare case

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Sarcoma botryoides in a 14 year old girl: a rare case

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International Journal of Reproduction, Contraception, Obstetrics and Gynecology