Adjuvant chemotherapy for a primitive neuroectodermal tumor of the uterine corpus: A case report and literature review

Abstract

A primitive neuroectodermal tumor (PENT) belongs to the category of a Ewing sarcoma. A PENT of the uterus is rare and has been known to be very aggressive by nature. Owing to the rarity of the tumor, there is no optimal treatment at present. In many cases, after hysterectomy, chemotherapy or radiation therapy has been performed. However, an effective chemotherapy regimen was unclear. In the soft tissue sarcoma area, the chemotherapy approach has recently greatly improved. Vincristine, doxorubicin, cyclophosphamide, ifosfamide and etoposide (VDC-IE) therapy has improved the survival rate of patients with Ewing sarcoma/PENT. Thus, VDC-IE therapy may be used for a uterine PENT. Here, we report a case of a uterine PENT in a premenopausal woman successfully treated with multimodality treatment including VDC-IE therapy and discuss the optimal chemotherapy for a uterine PENT through a literature review.

Neoadjuvant chemotherapy for Ewing’s sarcoma family tumors of the uterine cervix: A case report

“…The diagnosis of ESFT is difficult based on hematoxylin and eosin staining alone, and, thus, requires a combination of morphological and immunohistochemical findings. CD99, an immunohistochemical marker specific for the diagnosis of ESFT, is detected in more than 97% of cases ( Wang et al, 2017 , Akazawa et al, 2018 , Bose et al, 2012 ). A molecular genetic analysis may identify chromosomal translocations that may be useful for differentiating ESFT from other types of round cell tumors.…”

“…A molecular genetic analysis may identify chromosomal translocations that may be useful for differentiating ESFT from other types of round cell tumors. Approximately 85% of ESFT have a balanced t(11;22)(q24;q12) translocation, forming the chimeric fusion of the EWS-FLI1 gene( Akazawa et al, 2018 ). The fusion of the EWS gene with ERG, the second member of the ETS family, was observed in the majority of the remaining cases (10–15%).…”

Uterine Primitive Neuroectodermal Tumor

“…Postmenopausal bleeding is usually the most common symptom, similar to endometrial adenocarcinoma. However, unlike endometrial cancer, which is usually diagnosed in the early stages, uterine PNET is more frequently diagnosed in advanced stages [ 1 ]. In the clinical case presented, the patient presented with postmenopausal bleeding, although she had not consulted previously for this reason; the diagnostic suspicion was a chance ultrasound finding.…”

“…Primitive neuroectodermal tumors (PNETs) are included in the family of Ewing's sarcomas [ 1 ]. They have a neuroectodermal origin as a consequence of the translocation (11.22) (q24; 12) that originates in the Ewing sarcoma breakpoint region 1 - friend leukemia integration 1 transcription factor (EWS-FLI1) gene [ 2 ].…”

Primitive neuroectodermal tumors of the abdominal wall and vulva in children: Report of two cases and review of the literature

“…A main characteristic of PNET[11] is the detection of t (11; 22) (q24; q12) translocation, wherein an EWS-FLI-1 fusion gene is formed. In recent years, scientists have discovered other genetic changes in PNETs.…”