High grade sarcoma, with predominant neuroectodermal and minor embryonal rhabdomyosarcomatous tumor of the uterus: A case report

Abstract

Background There have been few documented cases of combined primitive neuroectodermal and embryonal rhabdomyosarcomas (ERMS) in the uterus. Due to their rarity, there is no consensus on the optimal treatment for patients with primitive neuroectodermal tumor (PNET) and ERMS of the uterus. Studies on treatment and outcome are limited. Case presentation A 32 year-old female presented with heavy vaginal bleeding. Ultrasound revealed an 18 cm uterus with thickened endometrium. Histopathology revealed embryonal rhabdomyosarcoma. She underwent a total abdominal hysterectomy, bilateral salpingectomy, lymph node dissection, and omentectomy. Pathologic review confirmed a tumor with mainly central-type PNET and focally ERMS within the uterus and cervix. She was treated with adjuvant chemoradiation. Conclusion Treatment of the predominant tumor, PNET, should be the primary goal of therapy. Vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide with tumor directed radiation may be efficacious for the treatment of this specific high grade uterine sarcoma.

Embryonal Rhabdomyosarcoma of the Uterine Cervix

“…Both patients were postmenopausal and tumors were centered in the uterine corpus without a botryoid appearance. Another phenomenon occasionally reported in ERMS of the uterine cervix and corpus, although not encountered in our series, is the presence of a primitive neuroectodermal component 4,63–67. These tumors are classified as “ectomesenchymoma” in the most recent World Health Organization (WHO) classification29 and typically demonstrate ERMS admixed with a variable component of neural differentiation ranging from primitive neuroblastoma to mature ganglioneuroma 29.…”

“…Another phenomenon occasionally reported in ERMS of the uterine cervix and corpus, although not encountered in our series, is the presence of a primitive neuroectodermal component. 4,[63][64][65][66][67] These tumors are classified as "ectomesenchymoma" in the most recent World Health Organization (WHO) classification 29 and typically demonstrate ERMS admixed with a variable component of neural differentiation ranging from primitive neuroblastoma to mature ganglioneuroma. 29 In reports of uterine tumors, the relative amount of the primitive neuroectodermal tumor component has varied considerably, but in some it was a minor component of an otherwise botryoid ERMS in young patients.…”

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

“…31 Five patients presented with symptoms of cervical polyps, which usually occurred in sarcoma botryoides, a subtype of embryonal rhabdomyosarcoma, because it arose beneath a mucous membrane. Although the largest diameter of the mass was 10 cm among these cases, 15–35 the current case was 18 cm, indicating that the patient’s condition was progressing rapidly and that her prognosis was poor. The IRS group of most patients was stage I, so the current case was no exception.…”

“…Since the first report of a case of cervical rhabdomyosarcoma in a 75-year-old woman in 1971, 24 cases in adults have been reported (Table 1). 15–35 The age ranges from 19 to 75 years. The most common symptoms were vaginal bleeding and swelling.…”