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Keywords:

  • embryonal rhabdomyosarcoma;
  • multimodal therapy

Abstract

  1. Top of page
  2. Abstract
  3. Case report
  4. Discussion
  5. References

Rhabdomyosarcoma is the most common soft tissue sarcoma found in children. A polypoid form of the embryonal type of rhabdomyosarcoma, namely, sarcoma botryoides, is often found in girls younger than 5 years, and typically presents as a polypoid mass protruding from the vagina. Over the years, there has been a shift in the treatment of this condition from radical surgery to a multimodal approach involving conservative surgery with chemotherapy and radiotherapy. This approach has been associated with improved survival and preservation of normal anatomy and function. We discuss the initial management of a 4-year-old girl with pelvic embryonal rhabdomyosarcoma using a multimodal approach.

Embryonal rhabdomyosarcoma (sarcoma botryoides) is the most common sarcoma occurring in infants and young adolescent girls(1). The appropriate management of pelvic embryonal rhabdomyosarcoma is not well documented, and there is sparse literature on this subject due to a lack of randomized controlled trials. Therapeutic options include surgery, chemotherapy, and radiotherapy.

We describe the course of events and early outcome of a patient with genital tract embryonal rhabdomyosarcoma over 6 months, using a multimodal approach to treatment.

Case report

  1. Top of page
  2. Abstract
  3. Case report
  4. Discussion
  5. References

A 4-year-old girl presented with a 3-month history of abdominal swelling, intermittent vaginal bleeding, and painful micturition and defecation. General examination revealed an ill-looking child with a large firm mass occupying almost the entire abdomen. A further 4–6 cm mass was noted, protruding from the vagina. This mass, lobulated, pink and semisolid in nature, extended deep into the vagina, and there was no significant lymphadenopathy.

An examination under anesthesia was performed, and biopsies of the vaginal mass revealed a diagnosis of embryonal rhabdomyosarcoma. A magnetic resonance imaging (MRI) scan was then performed to assess the extent of spread of the tumor, after which the tumor was staged as group 2, using the Intergroup Rhabdomyosarcoma classification(2). Following the administration of three cycles of chemotherapy (VAC–vincristine, dactinomycin, cyclophosphamide, doxorubicin regimen), a significant reduction in the size of the tumor was noted and surgery comprising a total abdominal hysterectomy and removal of a vaginal cuff 1 cm below palpable tumor was performed. The ovaries were preserved and suspended away from the pelvis.

The histopathologic features were consistent with embryonal rhabdomyosarcoma, however, there was presence of tumor at the vaginal cuff margin. Due to this, brachytherapy was planned, only external beam radiotherapy (EBRT) was possible. A total of 50.4 Gy was given in doses of 1.8 Gy with rest intervals, which was tolerated well. No further recurrence of tumor was noted following an examination under anesthesia, and the patient was subsequently discharged, with a plan for further follow-up visits.

Discussion

  1. Top of page
  2. Abstract
  3. Case report
  4. Discussion
  5. References

Rhabdomyosarcoma is generally regarded as a neoplasm affecting children, and data on its management have largely been obtained from pediatric patients. It accounts for 4–6% of all malignancies in this age group, and the prognosis depends on the size and extent of the tumor at the time of presentation(2). Unfortunately, patients have often been misdiagnosed as cases of “abuse.” Initially, no clear distinction concerning management was made, nor any difference in prognosis recognized between primary vulvar, cervical, vaginal, or uterine tumors(3). The management of this tumor is based largely on limited series, and the philosophy of therapy has evolved slowly from ultraradical surgery without adjuvant therapy to neoadjuvant chemotherapy followed by less radical surgery and postoperative radiation(3). Multimodal approaches have remarkably improved prognosis and decreased the need for radical surgery with its associated morbidity(4,5).

The surgical management of sarcoma botryoides has evolved from the ultraradical pelvic exenteration to a more conservative approach following better outcomes with combination therapy with radiation or chemotherapy. Fleming et al.(6) reported good results obtained from hysterovaginectomy for vaginal rhabdomyosarcoma with combination therapy in a series of 36 children with genitourinary rhabdomyosarcoma. Gruessner et al.(2), in a 30-year literature review, and based on their own observations, recommended minor surgical approaches in combination with chemotherapy as the treatment of choice for early stage I cervical rhabdomyosarcoma. Also, a focus on bladder preservation has not adversely affected survival in most studies(7). In cases where organ preservation is not possible, improvements in urinary diversion techniques still offer improved quality of life(4).

The most common chemotherapy regimens use combinations of vincristine, actinomycin D, and cyclophosphomide (VAC); vincristine, actinomycin D, and ifosfamide (VAI). These regimens are usually started preoperatively, particularly in patients with advanced tumor stages. Common complications associated with the VAC therapy are nausea and vomiting, and severe complications include leucopenia and neutropenia. Although the acute side effects of cytotoxic chemotherapy can be managed effectively, certain risks remain(8).

Conventional EBRT is integral to the treatment of most pediatric sarcomas. The Intergroup Rhabdomyosarcoma protocol IRS-V recommends postoperative doses of 36–41.4 Gy for treating microscopic residual disease and 50.4 Gy in 1.8 Gy fractions for treating gross disease(9). Unfortunately, EBRT, even in the 20–30 Gy range, can cause growth retardation or adversely affect organ function in young children(9). Brachytherapy is an option that can be used to limit the dose to normal tissues and initiate radiation after resection. Nag et al.(10), using high–dose rate brachytherapy, achieved excellent local control and disease-free survival in properly selected children with soft tissue sarcomas, while preserving normal bone and organ development. High–dose rate brachytherapy also eliminates the problems of associated radiation hazards and prolonged hospital stay associated with conventional low–dose rate techniques.

These potential side effects must be seen in the context of less invasive surgical therapy with its overall very low associated morbidity and good long-term outcome(2).

Multimodal therapy probably constitutes optimal therapy for the management of these patients, with or without recurrence. The International Society of Pediatric Oncology uses initial chemotherapy for patients with local or regional rhabdomyosarcoma irrespective of histology, reserving local therapy for those patients who do not achieve a clinical cure, have residual tumor after initial chemotherapy, or who experience local recurrence(11). The Intergroup Rhabdomyosarcoma Group reviewed the management of this unusual and challenging disease over four consecutive trials from 1972 to 1997, and concluded that localized female genital rhabdomyosarcoma may be curable with combination chemotherapy, a conservative surgical approach, and the use of radiotherapy for selected patients(11). The initial use of multimodal therapy is, therefore, likely to remain as the mainstay of treatment, however its long-term benefit, particularly in the patient described, rests on the outcome of long-term follow-up.

References

  1. Top of page
  2. Abstract
  3. Case report
  4. Discussion
  5. References
  • 1
    Sedlis A, Robboy SJ. Diseases of the vagina. In: Kurman, ed. Blausteins’s Pathology of the Female Genital Tract. New York: Springer, 1987:98140.
  • 2
    Gruessner SEM, Omwandho COA, Dreyer T et al. Management of stage 1 cervical sarcoma botryoides in childhood and adolescence. Europ J Paed 2004;163:4526.
  • 3
    Hahlin M, Jaworski RC, Wain GV et al. Integrated multimodality therapy for embryonal rhabdomyosarcoma of the lower genital tract in postpubertal females. Gynecol Oncol 1998;70:1416.
  • 4
    Andrassy RJ. Advances in the surgical management of children. Am J Surg 2002;184:48491.
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    Andrassy RJ, Weiner ES, Raney RB, Hays DM, Arndt CAS, Lobe TE. Progress in the surgical management of rhabdomyosarcoma: a 25 year review from the Intergroup Rhabdomyosarcoma Study Group. J Paed Surg 1999;34:7315.
  • 6
    Fleming DI, Ectubanas E, Patterson R et al. The role of surgical resection when combined with chemotherapy and radiation in the management of pelvic rhabdomyosarcoma. Ann Surg 1984;199:50914.
  • 7
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  • 8
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  • 9
    Shiu MH, Turnbull AD, Nori D et al. Control of locally advanced extremity soft tissue sarcomas by function saving resection and brachytherapy. Cancer 1984;53:138592.
  • 10
    Nag S, Tippin D, Ruymann FB. Long term morbidity in children treated with fractionated high-dose-rate brachytherapy for soft tissue sarcomas. J Pediatr Hematol Oncol 2003;25:44852.
  • 11
    Arndt CAS, Donaldson SS, Anderson JR et al. What constitutes optimal therapy for patients with rhabdomyosarcoma of the female genital tract? Cancer 2001;91:245468.