Rhabdomyosarcoma Of Female Genital Tract Research Articles

Female genital tract rhabdomyosarcoma in childhood and adolescence: A single center experience.

e22518 Background: Rhabdomyosarcoma is a rare tumor in children and adolescents, presenting 3% to 4% of all pediatric cancers. The female genital tract is considered as a favorable site of childhood RMS. The outcome has improved significantly during the last two decades, attributed to risk stratification and multimodality management of these challenging tumors. Here we present the clinical features and treatment results of girls with genital tract rhabdomyosarcoma to discuss. Methods: Fourteen girls with vaginal and uterine servical rhabdomyosarcoma younger than 18 years of age diagnosed and followed up between the years of 1995 and 2019 were included in this analysis. The clinical features and treatment results of patients were recorded from patient files and hospital information system retrospectively. Results: There were seven cases with vaginal and seven with uterin cervical carcinoma. All patients presented with polypoid masses protruding from the vagina. Median age of patients at diagnosis was 71 months (range 8-200 months). The IRS modified TNM staging was stage I for all the patients. All cases had embryonic type of RMS except one with alveolar type. All patients were treated with surgery and adjuvant chemotherapy, three received radiotherapy additionally. All achieved complete remission. Four patients experienced tumor relapse, 1 patient died with progressive disease. The median follow-up time was 75 months (7-271 months) for 13 patients who were alive with remission. Conclusions: Rhabdomyosarcoma is a rare sarcoma with a higher incidence in children and adolescents. With current treatment strategies, female genital tract rhabdomyosarcomas have a good prognosis. Favorable prognostic factors such as early stage at diagnosis and a favorable histology may contribute to the excellent observed survival. All parties who had involved on the care of these girls must be aware of the high survival with proper treatment to avoid treatment related morbidities and mortalities. Because of the rareness of the disease we wanted to share our experience.

Rhabdomyosarcoma of the female genital tract: Long-term outcome and association with DICER1 variation.

10538 Background: Rhabdomyosarcoma (RMS) of the female genital tract is rare, accounting for 3.5% of cases of rhabdomyosarcomas. Germline DICER1 mutations are associated with predisposition to pleuropulmonary blastoma and other tumors including sarcomas. Recently DICER1-associated RMS of the uterus/ovary has ben reported.. The aim of this study is to evaluate demographic characteristics, molecular pathogenesis, treatment and long term outcome of female genital tract rhabdomyosarcoma. Methods: Files of children with RMS of the female genital tract diagnosed at the Istanbul University, Oncology Institute during 1990-2019 were reviewed. Molecular genetic sequencing was performed by polymerase chain reaction amplification of genomic DNA extracted from the formalin-fixed, paraffin embedded tumors, followed by Sanger sequencing. Genetic testing for DICER1 variants of the proband and family members was performed if DICER1 mutation was detected in the tumor of the proband. Results: Of 210 RMS cases, 11 arose from the female genital tract. The median age at diagnosis was 52 months (10 months-15 years). Primary sites were vaginal (n = 5), uterus (n = 4), and ovary(n = 2). Presenting symptoms included vaginal mass (n = 6), vaginal bleeding (n = 5), and abdominal pain (n = 3). Four had group 1, five group 3, two group 2 disease and all received chemotherapy (vincristine, actinomycinD + cyclophosphamide).Three received radiotherapy; three underwent hysterectomy. DICER1 mutation was detected in tumor tissue in three patients:[ c.5113G > A (p.E1705K); c.5428G > T (p.D1810Y); c.1870C > T (p.Arg624Ter)] Genetic testing revealed germline DICER1 pathogenic variation in two patients and their family members, one of whom had cystic nephroma in infancy and history of Wilms tumor in an uncle. They were referred for surveillance for the DICER1 related diseases. A patient with metachronous bilateral ovarian RMS died. Two are married, one has children. The 5 year survival for RMS of the female genital tract was 85.7 % at a median follow-up of 34 (4-298) months. Conclusions: Rhabdomyosarcoma of the female genital tract is associated with a favorable prognosis, however some individuals undergo aggressive local therapies. Individuals with RMS of the female genital tract should be tested for DICER1 pathogenic variation. The detection of a DICER1 mutation in an individual or family members is important to facilitate surveillance for related tumors, so that they may be detected at the earliest possible stage, potentially increasing survival and decreasing risks of late effects.

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

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. 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. 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. 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.

Rhabdomyosarcoma of Reproductive Organs in Adult Females. Description of 5 Cases- Literature Review

Background: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcomas. It was described for the first time by Weber in 1854. RMS is recognized in approx. 90% of patients fewer than 20 years of age. The disease in adults is extremely rare. Materials and methods: A retrospective analysis was performed on 5 cases of RMS of the female genital tract. These 5 cases of the disease in women aged 20 and 87 years of age treated in a Warsaw hospital from 1991 to 2013. In all women the diagnosis was made on the basis of histopathological examination of tissues after excision of changes in the vulva and vagina. Tumors were classified according to the Intergroup Rhabdomyosarcoma Study (IRS) staging. All patients were treated with multimodality treatment: surgery, chemotherapy and/or ratiotherapy. Results: Of the five patients with RMS, three originated from the vagina one from vulvae and one from Bartholin’s glands. Two out of 5 patients presented with distant metastasis and death after 9 and 4 years. Tree patients alive for 3, 10, 11 years without recurrence or active disease a median duration was of 7.7 years. Conclusion: RMS in adults have poor prognosis as compared to childhood RMS. Adult RMS should therefore be treated aggressively with multidisciplinary approach comprising of surgery, radiation, and chemotherapy to achieve cure and prolonged survival. The prognosis of embryonal rhabdomyosarcoma of female genital tract is good. The standard treatment for adults RMS consisting the multimodal therapy with radical surgery and chemotherapy and radiotherapy.

Outcome of Pediatric Female Genital Tract Rhabdomyosarcoma Based on Analysis of Cases Registered in SEER Database

Outcome of Pediatric Female Genital Tract Rhabdomyosarcoma Based on Analysis of Cases Registered in SEER Database

Lower genital tract rhabdomyosarcoma: case series and literature review

Rhabdomyosarcoma (RMS) of the lower genital tract is a rare tumor. It tends to occur in childhood in the vagina and in rare cases, RMS can originate in the uterine cervix, with a peak incidence in the 2nd decade. A hospital-based tumor registry was searched to find all patients with female genital tract RMS, which were treated between 1999 and 2004. The medical records of all patients were reviewed. A single pathologist reviewed all pathologic specimens. Among the 1,528 patients with genital tract malignancies, six RMS were found: three vaginas, three cervixes. All patients presented with vaginal bleeding. Mean age of patients was 16(13-30). The lesions in vagina were clinically staged as stage I (2), stage II (1) and in cervix were stage I (2) and stage III(1). All of the patients were treated with surgery and adjuvant chemotherapy. One patient with cervical RMS was treated with adjuvant chemo radiation. Two patients with cervical RMS died from the large size and extent of the disease 9 and 11 months after diagnosis, but all patients with vaginal RMS remain alive after a mean follow-up of 38 months. Most patients present with vaginal bleeding and a palpable cervical or vaginal mass. While the optimal management of these tumors is uncertain, primary therapy with wide local excision and chemotherapy can result in prolonged survival and cure in patients with early stage RMS. Vaginal lesions have a better prognosis than cervical lesions. In patients with un-embrional RMS, large size of lesion, cervical origin and extent of disease, survival rates was decreased.