Objective: To compare the clinicopathological features, diagnosis, treatment, and prognosis of two types of uterine sex cord-like tumors.
Methods: The clinicopathological features of four uterine tumors resembling ovarian sex cord tumors (UTROSCTs) and two endometrial stromal tumors with sex cord-like elements (ESTSCLEs) were analyzed retrospectively.
Results: All patients were premenopausal women. The most common clinical presentation was vaginal bleeding (four cases). Total hysterectomy with or without bilateral adnexectomy was the most common treatment pattern (five cases). A patient with UTROSCTs, presenting with recurrence 10 months after transvaginal submucous myomectomy, underwent a total hysterectomy (case 2). All tumors were polypoid or intramural masses, usually located in the uterine fundus or submucosa. The majority of UTROSCTs were positive for cytokeratin (4/4 cases), one was positive for Wilms tumor protein, and of two cases with smooth muscle actin immunoreactivity, two were positive for desmin. UTROSCTs were positive for two or more sex cord markers, whereas sex cord markers were less frequently detected in ESTSCLEs. CD10 was variably positive in two UTROSCT patients and strongly positive in all ESTSCLE patients. Three UTROSCTs and one ESTSCLE were positive for both estrogen and progesterone receptors. All patients with UTROSCTs were alive without evidence of recurrence. One patient with ESTSCLEs underwent postoperative chemotherapy after total vaginal hysterectomy but developed recurrence at the vaginal stump (case 5). The other patient with ESTSCLEs was lost to follow-up.
Conclusion: These UTROSCTs are polymorphic neoplasms with true sex cord differentiation and uncertain malignant potential, which possess a distinct biology from ESTSCLEs.
Keywords: Uterine tumors resembling ovarian sex cord tumors; clinical; diagnosis; endometrial stromal tumors with sex cord-like elements; pathology; treatment outcome.