Pediatric radical abdominal trachelectomy for anaplastic embryonal rhabdomyosarcoma of the uterine cervix: an alternative to radical hysterectomy

Abstract

Rhabdomyosarcoma arising in the female genital tract carries five-year survival in excess of 80%, but lifelong infertility may be a consequence of local control strategies. We present the technique and outcome for a fertility-sparing, radical abdominal trachelectomy in a 12-year-old girl with anaplastic, embryonal rhabdomyosarcoma involving the uterine cervix. The patient had presented to our center following the piecemeal resection of a uterine cervical mass; because of concern about microscopic residual disease, we classified her as group II-A according to the International Rhabdomyosarcoma Study (IRS) system. Staging studies excluded the presence of distant disease. The patient received four cycles of multiagent chemotherapy and then underwent radical abdominal trachelectomy, with removal of the uterine cervix, parametria, vaginal cuff, and regional lymph nodes. Microscopically, the specimen showed treatment effect and no residual tumor. Regional nodes were negative. Radical abdominal trachelectomy, which has not been previously reported for rhabdomyosarcoma, has appeared to secure local disease control in this case, while preserving the patient’s future fertility potential. In properly selected cases of rhabdomyosarcoma of the uterine cervix, where involvement of the uterus proper is not present, radical abdominal trachelectomy may be an attractive fertility-sparing alternative to radical hysterectomy.

Embryonal rhabdomyosarcoma of the cervix

“…[3,5,7] Surgery has inclined towards a more conservative approach including radical abdominal trachelectomy with a view to preserve the patient's future fertility potential. [4,8] Unfortunately, our patient did not have fertility-sparing surgery due to the advanced nature of the disease and did not receive chemotherapy and radiotherapy because she did not return for follow-up. Patient follow-up is a big problem in our environment due to poverty and ignorance.…”

“…[5,6] A combined modality approach to treating this condition using multidrug chemotherapy, radiotherapy, and surgery has markedly improved survival. [7,8] This case report illustrates this uncommon genital tract cancer and the problems associated with its management in our environment.…”

Importance of examination under anaesthesia in adolescent age group for effective diagnosis and treatment in diagnostic dilemma: case series

“…The prognostic factors are histological type (embryonal botryoid having favorable outcome than alveolar subtype), metastatic disease at presentation and poor response to chemotherapy. 3,4 The extent of the disease following primary surgical procedure is the most important prognostic factor. An early diagnosis is important as it favors a good prognosis.…”

A Case Report of Cervical Embroynal Rhabdomyosarcoma Treated with Multimodality Approach

“…Studies (1, 2) comparing aggressive surgery and radiation with chemotherapy have reported similar survival, while maintaining fertility. IRSG (3,4) states that fertility sparing surgery followed by chemotherapy is the appropriate treatment for local control. However, there are case reports of unfavorable outcomes with this management.…”