Is fertility-preservation safe for adult non-metastatic gynecologic rhabdomyosarcoma patients? Systematic review and pooled survival analysis of 137 patients

Elsebaie, Maha A. T.; Elsayed, Zeinab

doi:10.1007/s00404-017-4591-6

Cited by 10 publications

(16 citation statements)

References 56 publications

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“…This is in contrast to the recently-advocated favorable overall prognosis for gynecologic rhabdomyosarcoma. Predicting a favorable outcome is the basis for introducing fertility-sparing options and locally conservative approaches, therefore it is of the outmost importance 11…”

Section: Discussionmentioning

confidence: 99%

“…A conservative approach appears to be safe in women with a small (<5 cm) embryonal, resectable (IRS I) tumor with negative node at imaging. Patients with non-embryonal histology, multiple satellite lesions, deep stromal invasion, lymph node or parametrial involvement, and IRS grouping up to I should undergo a radical treatment (at least extrafascial hysterectomy) 11. The use of neoadjuvant chemotherapy in association with radiation therapy is not supported by data in adults, and future research is highly encouraged.…”

Section: Discussionmentioning

confidence: 99%

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

Ricciardi

Plett

Sangiorgio

et al. 2020

International Journal of Gynecological Cancer

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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.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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

Ricciardi

Plett

Sangiorgio

et al. 2020

International Journal of Gynecological Cancer

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“…Our patient had both cervical and uterine involvement with no evidence of parametrial invasion which necessitated a simple total hysterectomy. Lymphadenectomy is not routine and is reserved only for patients with high-risk clinical features [ 14 ].…”

Section: Discussionmentioning

confidence: 99%

“…According to Elsebaie et al, the overall 5-year survival rate is 65% [ 14 ]. Although some patients may be cured with combined surgical and adjuvant treatment, the risk of recurrence and metastatic spread remains a concern [ 14 ].…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Embryonal Rhabdomyosarcoma of the Uterine Cervix

Bahall

Barry

Sankar

2022

Case Reports in Pathology

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Embryonal rhabdomyosarcoma (RMS) of the uterine cervix is an exceedingly rare mesenchymal tumor that accounts for less than 1% of all cervical cancers. This highly malignant tumor primarily affects adolescents and young adults. Due to the paucity of publications on this clinical entity, there are no clearly established treatment protocols. However, a multimodal approach to treatment that involves surgical intervention combined with adjuvant chemoradiotherapy appears to improve patient outcomes. Herein, we report a case of embryonal rhabdomyosarcoma of the uterine cervix in a 24-year-old female, who presented with an exophytic cervical mass and vaginal bleeding. Histopathology and immunohistochemistry confirmed embryonal rhabdomyosarcoma of the uterine cervix with extension into the lower uterine segment. This patient was successfully managed with a combination of neoadjuvant chemoradiotherapy, a total abdominal hysterectomy with bilateral salpingo-oophorectomy, and adjuvant chemoradiotherapy.

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“…evaluated 137 patients with nonmetastatic gynecologic RMS, in a systematic review. Surgery was the main approach for local control of tumor in all patients [ 13 ]. According to the suggested scheme in this study, our patient was in the high-risk group owing to a large bulky pelvic mass, heavy vaginal bleeding, and alveolar variant of RMS.…”

Section: Discussionmentioning

confidence: 99%

Rhabdomyosarcoma of the uterus in an adult patient with osteopetrosis: a case report

Aminimoghaddam

Rahbari²,

Pourali³

2021

J Med Case Reports

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Background Uterine sarcoma accounts for 3–7% of uterine malignant neoplasms. It is more aggressive than epithelial neoplasms, and patients have a poor prognosis. Rhabdomyosarcoma is classified as a heterologous uterine sarcoma. It is the most common soft tissue malignancy in children while rare in adults. In young patients, the majority of genital tract rhabdomyosarcomas occur in vagina; however, the most common site of gynecologic rhabdomyosarcoma is cervix followed by uterine corpus, in adults. Uterine corpus rhabdomyosarcoma is rare in adults. Diagnosis of pure rhabdomyosarcoma in uterus involves widespread and perfect sampling as well as precise histopathological evaluation to uncover any epithelial component. Case presentation Here we report a case of pure rhabdomyosarcoma of uterine corpus in a 60-year-old Iranian postmenopausal female who had osteopetrosis, presenting with 8-month heavy vaginal bleeding and a protruding cervical mass. She is alive on 18-month follow-up after treatment. Conclusions Rhabdomyosarcoma of uterine corpus is rare in adults. Diagnosis of pure rhabdomyosarcoma in uterus involves widespread and perfect sampling as well as precise histopathological evaluation to uncover any epithelial component. Treatment options in adult gynecological rhabdomyosarcoma are based on studies in younger patients, and more studies may help us choose the best approach for improving outcome.

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Is fertility-preservation safe for adult non-metastatic gynecologic rhabdomyosarcoma patients? Systematic review and pooled survival analysis of 137 patients

Cited by 10 publications

References 56 publications

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

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

A Rare Case of Embryonal Rhabdomyosarcoma of the Uterine Cervix

Rhabdomyosarcoma of the uterus in an adult patient with osteopetrosis: a case report

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