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

Abstract

The probability of rhabdomyosarcoma occurring in the cervix is less than 0.5% and may be associated with a pathogenic dicer 1, ribonuclease III ( DICER1) gene variation. Tumour-induced hypercoagulability and high levels of cancer antigen (CA) 125 are risk factors for cerebral venous sinus thrombosis (CVST). In addition, although nonpuerperal uterine inversion is very rare and is usually caused by leiomyomas from the uterus, large cervical masses can also be the cause. This case report describes a 24-year-old woman with uterine inversion caused by an embryonic cervical rhabdomyosarcoma that presented with CVST as her first symptom. The patient underwent laparoscopic total uterus and bilateral salpingectomy, during which the uterus was found to be completely inverted. Postoperative pathology confirmed embryonic cervical rhabdomyosarcoma. The patient quickly developed lung and para-aortic lymph node metastases. Two months later, the patient died of complications. When coagulation indices in patients with tumours are abnormal, especially when the levels of D-dimer and CA125 increase, it is recommended that anticoagulant therapy is administered in a timely manner to prevent the occurrence of CVST. Furthermore, for large cervical tumours, physicians should also be alert to the occurrence of uterine inversion.

Pleomorphic Rhabdomyosarcoma of the Uterine Corpus in an Adult Who Underwent Multi-gene Panel Testing

“…women with genital tract RMS, including three cases of uterine corpus RMS and eight cases of cervical RMS, and reported that 70% of patients had abnormal bleeding and 15% had abdominal pain. In a review of 25 reported cases of cervical RMS in adults, Meng et al (9) reported that bleeding was the most frequent symptom, together with vaginal masses or polyps being common. The most common subtype of adult RMS is PRMS, which is associated with unfavorable outcomes (8,10).…”

“…Multimodal therapy consisting of surgery, chemotherapy, and radiation, has also been conducted for uterine RMS in adults as well as children (5,9,11). Fertility preservation is a treatment strategy for children with uterine RMS.…”

“…Pelvic and/or aortic lymph node dissection has been performed in several cases; however, whether it can improve the outcome of uterine RMS remains unknown. Lymph node excision should be performed with caution because some patients die several days or weeks following surgery (9,11,13). Most patients undergo additional therapies, including chemotherapy and/or radiotherapy, after surgery.…”

Embryonal rhabdomyosarcoma of the uterine cervix in an adult female

“…Other clinical symptoms include amenorrhea or menorrhagia. 19 Advanced cases may present with cervical pain radiating to the back, bladder irritation, and tenesmus. Terminal stages of the malignancy give rise to anemia and cachexia and may even develop hydronephrosis.…”

“…IHC is essential for a confirmatory diagnosis. 19,20 Histopathologically, this tumor needs to be differentiated from other benign and malignant mimics such as cervical mesodermal polyp (pseudosarcoma botryoides), rhabdomyoma, cervical adenosarcoma, pleomorphic undifferentiated uterine sarcoma, high-grade endometrial stromal sarcoma, and dedifferentiated cervical leiomyosarcoma.…”