Recurrence in uterine tumors with ovarian sex-cord tumor resemblance: a case report and systematic review

Abstract

Objective: The aim of this study was to evaluate the prognostic factors of recurrence in uterine tumors resembling ovarian sex-cord tumors (UTROSCT) and to determine clinical-pathological characteristics, treatment options and outcome. Material and Method:An electronic literature search was conducted from 1976 to 2018. After the comprehensive evaluation and conjunction with our case, the study included 79 cases. Results:The median age at initial diagnosis was 49 years (range; 16-86 years). The age was under 40 years in 21 (26.6%) patients. Whereas 68 patients underwent at least hysterectomy, 9 patients had organ sparing surgery. There was necrosis in 4 (5.1%) patients, atypia in 16 (20.3%) patients, and infiltrative tumor border in 34 (43%) patients. At least one mitosis per 10 high power fields was determined in 36 (45.5%) patients. The tumor involved at least part of the myometrium in 54 (68.3%) patients. Median follow-up time was 30 months (range; 3-296 months). Recurrence was determined in 5 (6.3%) patients. The disease free survival (DFS) was significantly related only to surgery type. None of the pathologic features were associated with DFS. The 5-year DFS was 86% and 96% in patients who underwent organ sparing surgery or not, respectively (p=0.038). Conclusion:The accurate pathologic diagnosis of UTROSCT has great value in shaping surgical management and management during the follow-up period. Organ sparing surgery was related to poor DFS. Although recurrence is rare, it should be kept in mind for patients with UTROSCT.Key Words: Uterine neoplasms, Recurrence, UTROSCT, Surgery, Prognosis INTRODUCTIONUterine tumors with sex-cord-like elements can be divided into two groups. The first group of tumors is called endometrial stromal tumors with sex cord-like elements (ESTSCLEs) involving endometrial stromal neoplasms with focal areas (<50%) resembling ovarian sex-cord elements. The second group of tumors is called uterine tumors resembling ovarian sex-cord tumors (UTROSCTs) corresponding to uterine tumors with a predominant or exclusive pattern similar to ovarian sex-cord tumors (1, 2). This morphologic differentiation is clinically significant because these tumors have different biological behaviors. ESTSCLE has a tendency for recurrence and metastases, whereas UTROSCT usually shows more benign clinical behavior (3). UTROSCTs rarely recur and are thus considered uterine tumors with low malignant potential (4). UTROSCTs are extremely rare tumors mostly documented as case series in the literature; therefore, it is difficult to draw a distinct conclusion about the management or recurrence rates of these tumors.In this study, a recurrent case of UTROSCT is presented. This analysis evaluated prognostic factors related to UTROSCT recurrence and determined clinicalpathological characteristics, treatment options, and outcomes of UTROSCT. CASE REPORTA 61-year-old patient was referred to our gynecologiconcology clinic because of a UTROSCT diagnosis. She underwent total abdominal hysterectomy (TAH) and bilateral...

Therapeutic management of uterine tumors resembling ovarian sex cord tumors: Systematic Review

“…In this review, the median age at diagnosis was 50.0 years, similar to 49-51 years in previous reviews and case series reporting UTROSCTs [10,27]. Patients under 40 years of age with UTROSCTs accounted for a small number (21.3%); therefore, fertility preservation treatment should be considered.…”

Recurrent Uterine Tumor Resembling Ovarian Sex Cord Tumor: A Case report and Clinicopathological and Immunohistochemical Analysis

“…Moore et al [4] observed eight cases with recurrence in 34 cases and calculated a recurrence rate of 23.5% for UTROSCT. Kavneet et al [11] reported that one out of six cases (16.7%) relapsed within 1 year of diagnosis, while Günsu et al [9] reported a recurrence rate was only 6.3%. The reason for the rst high percentage rate attributed to the inclusion of patients with metastases or occurrence in other medical institution referred to their institution for consultation which increased the proportion of relapsed patients in the medical institution.…”

“…Clinically, ESTSCLE often recurs whereas UTROSCT is usually regarded as indolent or low malignant potential neoplasm [3]. However, several cases of UTROSCT have reported recurrence and metastasis during postoperative follow-up [4][5][6][7][8][9][10][11][12][13][14]. The purpose of this article was to provide more informations on the recurrence of UTROSCT and was aware of these speci c clinicopathological parameters those were of value in predicting malignant behavior for clinicians in dealing with the subset neoplasms with aggressive characters.…”

Uterine Tumor Resembling Ovarian Sex-Cord Tumors Initially Diagnosed as a Prolapsed Fibroid

“…In a systematic review published in 2018 that evaluated prognostic factors of recurrence in UTROSCT, recurrence rates of 6.3% were found for the 79 patients evaluated. Disease-free survival is high and is associated only with the type of surgery: 86% for patients submitted to resection of the tumor alone and 96% for those submitted to total hysterectomy, both for a five-year period [ 11 ]. The UTROSCT is considered of uncertain malignant potential, and there are no formal recommendations regarding treatment [ 7 ].…”