Primary sarcoma of the uterine cervix (144)

Abstract

<b>Objectives:</b> To investigate the clinical-pathologic characteristics and prognosis of patients diagnosed with primary sarcoma of the uterine cervix. <b>Methods:</b> We identified all patients with primary cervical sarcomas treated at our institution from 2002 to 2020. Clinical-pathologic variables, treatment modalities, and prognosis were abstracted from the medical records. <b>Results:</b> Thirty-four patients were identified. Of these patients, seven (20.6%) had leiomyosarcoma, six (17.6%) had carcinosarcoma or malignant mixed Müllerian tumor, five (14.7%) patients had Ewing's sarcoma, four (11.8%) patients had rhabdomyosarcoma, four (11.8%) patients had undifferentiated sarcoma, three (8.8%) patients had sarcoma not otherwise specified, two (5.9%) patients had adenosarcoma, two (5.9%) patients had endometrial stromal sarcoma, and one (2.9%) patient had alveolar soft tissue sarcoma. The median age of the patients was 43.5 years (range: 13-63). The median age of patients with Ewing's sarcoma or rhabdomyosarcoma was 22 years (range: 13-39) and 17 years (range: 13-36), respectively. Twenty-four patients presented with vaginal bleeding, which was the most common primary symptom. Thirty-one patients received primary treatment after diagnosis at our institution; 27 of them received surgical therapy with or without postoperative therapy. The FIGO 2018 distribution by stage was: stage I in 18 patients, stage II in five patients, stage III in five patients, and stage IV in three patients. The median follow-up was 26.0 months (range: 3.6-153.8 months). Fourteen patients died during follow-up, and 11 patients died within two years after diagnosis; most of them were patients with carcinosarcoma or undifferentiated sarcoma (45.5%, 5/11). The 2-year overall survival (OS) and 5-year OS were 64.0% and 52.4%, respectively. Four patients with advanced-stage or metastatic cervical sarcoma received systematic therapy. All of them died within one year after diagnosis. Among patients with sarcoma, 5-year OS was 53.3% for Ewing's sarcoma, 50.0% for rhabdomyosarcoma, 40.0% for leiomyosarcoma, 25.0% for undifferentiated sarcoma, 60.0% for carcinosarcoma, and 75.0% for others. The 5-year OS for stage I and II-IV patients was 63.5% and 36.9%, respectively. <b>Conclusions:</b> Cervical sarcomas are rare neoplasms with multiple histological subtypes and follow an aggressive course. Surgery is the main treatment for cervical sarcoma. Prognosis may be associated with tumor histology and stage.