Adult primary cervical rhabdomyosarcomas: A Multicentric cross-national case series

Abstract

ObjectivesAdult primary cervical rhabdomyosarcoma is a very rare disease and data regarding treatment are sparce. The goal of this study was to report on our experience with the management of this rare entity, along with an evaluation of the literature.MethodsWe conducted a review of the medical records at four centers from January 1990 to December 2017. We reviewed clinical characteristics including age at diagnosis, BMI, medical history and tumor stage, as well as treatment in the primary and recurrent settings and follow-up data. We reclassified tumors according to the Intergroup Rhabdomyosarcoma Study (IRS) clinical group.ResultsA total of 15 patients were included in the analysis. Median age at diagnosis was 35 years (range 17–55). Median tumor size at presentation was 5 cm (range 3–10). Eleven patients had the embryonal variant, including five showing the botryoid subtype. Four patients had a pleomorphic rhabdomyosarcoma. Eleven patients had disease classified as IRS Clinical Group I, while the remaining four fell into groups II or III. Fertility-sparing treatment was offered to five patients. Primary treatment types were: surgery alone in eight patients, surgery followed by adjuvant chemotherapy in six patients, and neoadjuvant chemotherapy in two patients. The main risk factors for relapse were: IRS clinical group greater than I, tumor size greater than 5 cm, lymph nodal involvement, and non-embryonal histology. At a median follow-up of 35 months (range 3–282), we observed a 5-year overall survival rate of 78.2% and a progression-free survival of 58.2%. No patient in the IRS I group died of the disease. Three out of four patients in the IRS II-III group died of the disease (survival range 5–16 months following treatment).ConclusionOur data show that cervical rhabdomyosarcomas account for at least two prognostic groups, demonstrating the existence of low-risk and high-risk patterns. The best predictor of prognosis appearsd to be the IRS clinical group classification system. IRS Group I tumors had an overall good prognosis and rarely recurred; when they did recur they were mainly local, following conservative treatment.

Rhabdomyosarcoma of the Cervix in a Post-Menopausal Woman—An Unparalleled Phenomenon

“…The reason for that is the fact that sarcomas present a very diverse image. Embryonal rhabdomyosarcoma usually presents a few traits: agglomerations of spindle and round pleomorphic cells with hyperchromatic nuclei, rhabdomyoblasts and myxoid stroma [9][10][11]. Performing an immunohistochemical profile of the tissue is nowadays a must in diagnosing sarcomas.…”

“…Patients can be divided into those at low and high risk of developing poor outcomes of treatment. Among the favorable factors highlighted in recent studies are: embryonal type of tumor, a superficial infiltration (<50% in MRI or <10 mm in conization material), negative histological margins of dissected tumor or no regional or distant metastases found on preoperative imaging [11]. It is extremely important to qualify patients to a specific risk group for patients of childbearing age.…”

“…Cervical ERMS is extremely rare and there are only a few cases published in the English literature describing the management and treatment of this disease in adult women [9][10][11][12]. We report a case of a 61-year-old women with rhabdomyosarcoma embryonale of the cervix initially presenting itself as a cervical polyp.…”

Embryonic cervical rhabdomyosarcoma complicated with uterine inversion with cerebral venous sinus thrombosis as the first symptom: a case report and literature review

“…1 In addition, approximately 30% of rhabdomyosarcomas originate in the genitourinary tract in females, mainly in the vagina; less than 0.5% of cases occur in the cervix. 2 In adults, rhabdomyosarcomas of the uterine cervix are extremely rare. 2 Rhabdomyosarcoma of the female genital tract, especially uterine corpus and rarely uterine cervix, may be associated with a pathogenic dicer 1, ribonuclease III ( DICER1 ) gene variation.…”

“…2 In adults, rhabdomyosarcomas of the uterine cervix are extremely rare. 2 Rhabdomyosarcoma of the female genital tract, especially uterine corpus and rarely uterine cervix, may be associated with a pathogenic dicer 1, ribonuclease III ( DICER1 ) gene variation. 3 , 4…”