Case report and review of the literature regarding management of primary uterine primitive neuroectodermal tumors (PNET)

Primitive neuroectodermal tumors (PNET) arise primarily from the uterus, are rare, and lack consensus management recommendations. We present a case report of a patient with stage IVB uterine PNET and a comprehensive literature review on the topic. Fifty-three papers including 90 cases of PNET were identified with an additional case report from our institution included. Data was collected on patient presentation, demographics, oncologic information, and survival. Median age of diagnosis was 51 years old, with most patients presenting with stage III disease. Most treatment regimens included hysterectomy with chemotherapy. Chemotherapy regimens in 77 % of cases were sarcoma-based, 57 % included a platinum agent, 49 % included an anthracycline agent, <1 % involved Bevacizumab, and <1 % utilized immunotherapy. Regarding survival status, 50 % of patients had no evidence of disease ranging from 1 month to 12 years, 10 % of patients were alive with disease from 1-24 months follow-up, and 27 % patients died from disease 2-36 months after diagnosis. One- and two-year overall survival was stratified by clinical and treatment characteristics. Kaplan-Meier curve analysis was also performed for overall survival. Primary uterine PNETs are rare, and the literature is limited to case reports. Our patient had a positive response with carboplatin, etoposide, and atezolizumab. Our literature review found that most patients diagnosed with uterine PNET were postmenopausal women in their 50 s and 60 s who presented with vaginal bleeding at advanced stage disease. Treatment most commonly included a simple hysterectomy with chemotherapy, most commonly utilizing a regimen used to treat sarcomas. Stage I disease, younger age, and sarcoma-based chemotherapy regimen all demonstrated higher overall survival rates.