Embryonal Rhabdomyosarcoma (Botryoid Type) of the Uterine Corpus and Cervix in Adult Women: Report of a Case Series and Review of the Literature

  title={Embryonal Rhabdomyosarcoma (Botryoid Type) of the Uterine Corpus and Cervix in Adult Women: Report of a Case Series and Review of the Literature},
  author={Rose Li and Mamta Gupta and W Glenn McCluggage and Brigitte M. Ronnett},
  journal={The American Journal of Surgical Pathology},
In its classical form, embryonal rhabdomyosarcoma (ERMS, botryoid type) is a vaginal neoplasm occurring in infants and young girls and is often not considered in the differential diagnosis of uterine corpus and cervical spindle cell tumors in adult women. Clinicopathologic and immunohistochemical features of 25 cases of ERMS in women 20 years of age or older were analyzed. Patient age ranged from 20 to 89 years (mean, 44.4 y; median, 46 y), with 8 patients aged 20 to 39 years, 14 patients aged… 
Embryonal rhabdomyosarcoma of the uterine corpus: a clinicopathological and molecular analysis of 21 cases highlighting a frequent association with DICER1 mutations
DICER1-associated ucERMS were more likely to show a classic histological appearance including heterologous elements than DICer1-independent tumors, and most DICER2-associated rhabdomyosarcomas to be sporadic.
A Case of Embryonal Rhabdomyosarcoma of Cervix Uteri in a 14 Year Old Girl
Embryonal rhabdomyosarcoma is an aggressive soft tissue malignancy of childhood and adolescence arising from embryonal mesenchyme and management poses a clinical change as preservation of hormonal, sexual and reproductive function is essential.
Uterine rhabdomyosarcoma in adults.
Embryonal Rhabdomyosarcoma of the Ovary and Fallopian Tube: Rare Neoplasms Associated With Germline and Somatic DICER1 Mutations.
DICER1 mutations (somatic or germline) are associated with a variety of uncommon neoplasms including cervical and genitourinary embryonal rhabdomyosarcoma (ERMS). We report a primary ovarian and 2
Rhabdomyosarcoma of the Cervix, Local Excision or Radical Surgery (Report of Two Cases and Review of the Literature)
There are several methods of surgical approach and variation in adjuvant therapy in the management of embryonal rhabdomyosarcoma, and if you choose a conservative approach for surgery of early stage, surgical margin should be negative and in other cases doing radical surgery is the best.
Genital Rhabdomyoma of the Lower Female Genital Tract: A Study of 12 Cases With Molecular Cytogenetic Findings
The clinical and pathologic findings in 12 cases were reviewed and 2 cases were processed for whole genome copy number analysis by single nucleotide polymorphism microarray, finding no somatic copy number alterations, particularly involving genes in Hedgehog signaling, were identified.
Significantly greater prevalence of DICER1 alterations in uterine embryonal rhabdomyosarcoma compared to adenosarcoma
Although DICER1 mutations are near ubiquitous in uterine ERMS and are significantly less common in uterusine adenosarcoma, DICer1 testing is only of value in distinguishing between the two neoplasms when a DicER1 mutation is absent, as this is helpful in excluding ERMS.
Mesenchymal and Miscellaneous Neoplasms of the Uterine Corpus
There have been significant recent advances in identifying subsets of endometrial stromal sarcomas with a molecular basis, which increasingly allows for the correct classification of cases that have previously been erroneously classified as undifferentiated uterine sarcoma or leiomyosarcoma.
DICER1-associated Tumors in the Female Genital Tract: Molecular Basis, Clinicopathologic Features, and Differential Diagnosis
The most current understanding of DICER1 genetic alterations is highlighted and the clinical, histopathologic, and immunohistochemical features and differential diagnoses for gynecologic tumors associated with DICer1 mutation are described.
Sarcomas of the Uterine Cervix: A United and Multidisciplinary Approach is Required
There are no definite guidelines avail ­ able for treatment of patients with rhabdomyo­ sarcoma of the cervix, although the majority are treated with a combination of surgery and chemotherapy.


Embryonal rhabdomyosarcoma of the uterine cervix: a report of 14 cases and a discussion of its unusual clinicopathological associations
C cervical embryonal rhabdomyosarcoma may be another pathological manifestation in the spectrum of extrapulmonary pathology in the setting of pleuropulmonary blastoma, as 12 of the 14 patients remain disease-free following conservative surgery and chemotherapy.
Sarcoma botryoides of the uterine cervix in a 46-year-old woman: case report and literature review.
This is the only the fourth case reported in detail of a sarcoma botryoides of the uterine cervix in a patient over the age of 40, and the patient was alive without recurrence 45 months postoperatively.
Clinicopathologic Features of Rhabdomyosarcoma of Gynecologic Origin in Adults
Adult RMS of gynecologic origin presents with locoregional disease and most are morphologically similar to pediatric RMS; however, adult RMS behaves more aggressively, with worse overall survival.
Cytogenetic study of botryoid rhabdomyosarcoma of the uterine cervix
This is the first reported case of sarcoma botryoides of the endocervix of the uterine cervix with chromosomal analysis and positive staining within the tumor cells for myoglobin, desmin, vimentin, muscle-specific actin and CD56.
Sarcoma botyroides of the cervix in a HIV positive 45-year-old woman: a case report.
The need for a high index of suspicion of sarcoma botyroides of the cervix must be borne in mind when making a diagnosis on all cervical polypoidal masses is kept in mind.
Embryonal rhabdomyosarcoma of the uterine cervix in a 47‐year‐old woman
A 47‐year‐old woman treated with radical hysterectomy followed by adjuvant chemotherapy and review the current literature on embryonal rhabdomyosarcoma is presented.
Pleomorphic Rhabdomyosarcoma of the Uterine Corpus: A Clinicopathologic Study of 4 Cases and a Review of the Literature
Data indicate that pleomorphic rhabdomyosarcoma of the uterine corpus is a highly aggressive, rapidly progressive tumor with a high case-fatality rate.
Embryonal Rhabdomyosarcoma of the Cervix and Appendiceal Carcinoid Tumor
It is proposed that appendectomy be considered during surgical management in women over the age of 40 years with embryonal rhabdomyosarcoma of the cervix and plans for treatment involve adjuvant chemotherapy with vincristine, dactinomycin, and cyclophosphamide.