Clinical Study on Female Genital Tract Rhabdomyosarcoma in Childhood: Changes During 20 Years in One Center.

Abstract

Objective: Rhabdomyosarcoma (RMS) is a rare tumor in girls. We intend to analyze the clinical feature, therapeutic effect, and prognosis of pediatric and adolescent RMS of the female genitourinary tract.

Methods: Pediatric or adolescent patients with female genital tract RMS in 1 tertiary hospital from 1995 to 2015 were retrospectively reviewed. The medical records were collected, including general information, tumor primary site, stage, histology, treatments, adverse effects, and survival outcomes.

Results: There were 8 girls with genital tract tumor in 403 patients with RMS during 20 years in 1 hospital: 5 originated in the vagina, 2 in the cervix, and 1 in the pelvis. Except for the patient with pelvic RMS who was diagnosed after acute abdominal pain, all the other patients presented with polypoid masses protruding from the vagina accompanied by mucosanguineous discharge. Mean age of patients at diagnosis was 5.4 years (8 months-14 years). Patients were categorized by using clinical group and TNM classification of malignant tumours staging system, 7 with stage I and 1 with stage II; none of them were classified to a higher stage. Histologically, all RMS were embryonal tumors with 3 botryoid variants. Patients were treated with surgery and adjuvant chemotherapy during which 6 patients developed fourth degree myelosuppression. All patients achieved complete remission. Two patients with vaginal RMS and the patient with pelvic RMS experienced tumor relapse and 2 patients died.

Conclusions: Rhabdomyosarcoma is a rare malignant sarcoma with a higher incidence in children and adolescents. Tumor sites are strongly associated with age at diagnosis. After combined therapy with local excision and chemotherapy, most patients can achieve a good prognosis. The prognosis is highly correlated with tumor site and histologic type.