European Journal of Obstetrics & Gynecology and Reproductive Biology
ReviewClinical characteristics and outcomes of uterine tumors resembling ovarian sex-cord tumors (UTROSCT): a systematic review of literature
Introduction
Uterine tumor resembling ovarian sex-cord tumor (UTROSCT) is a rare, relatively newly defined clinical entity. The original identification of UTROSCT as a histopathological entity by Clement and Scully in 1976 included two distinct tumors of unclear origin; hypothesized originating cells included endometrial stromal cells, adenomyosis, stromal myosis, endometriosis, or multipotential cells within the myometrium [1]. Since then, the entity has been recently delineated into the two distinct subtypes. Type I tumors, known as endometrial stromal tumors with sex cord-like elements (ESTSCLE), have been recognized to have more malignant potential than Type II, and the outcome of Type I disease is contingent upon type, grade, and stage of the underlying stromal neoplasm [2]. Type II tumors, comprising classic UTROSCTs, are considered to be of low-grade malignant potential secondary to occasional recurrence, although they typically exhibit benign behavior [2]. While both ESTSCLE and UTROSCT most likely arise from pluripotential uterine mesenchymal cells, UTROSCT is predominantly differentiated into sex-cord components, unlike ESTSCLEs, which typically express only one sex-cord marker [2]. This classification of UTROSCT into two histologic subtypes is relatively new, and a large portion of literature refers to UTROSCT generally without subcategorization.
Diagnosis of UTROSCT is primarily based upon morphologic features on hematoxylin/eosin staining with confirmation by immunohistochemical staining. Positive staining for at least two sex-cord markers is supportive, including calretinin and at least one other marker [3], [4], [5]. Other commonly expressed markers include are inhibin, cluster of differentiation 99 (CD99), and Melan A [5]. Additionally, these tumors are variably immunoreactive for mesenchymal and epithelial elements as well; frequently positive stains include vimentin, desmin, cytokeratin, epithelial membrane antigen (EMA), CD10, and estrogen/progesterone receptors (ER/PR) [3]. A review of immunohistochemical markers associated with UTROSCTs identified inhibin as the most specific marker and calretinin as the most sensitive marker of the tumor [3], [6], [7].
Given its rarity, the diagnosis of UTROSCT is usually made postoperatively per histopathological analysis. In addition, current available literature mainly focuses on the diagnosis of UTROSCT, and there is scant information available to define the clinical characteristics and outcomes of UTROSCT. The purpose of this study was to elucidate clinical, as opposed to pathological, characteristics of UTROSCT in order to best recognize the condition in clinical practice. Furthermore, optimal treatment for the disease has yet to be defined. As such, by conducting a detailed literature review of cases of UTROSCT, optimal treatment methodologies for the disease will be proposed.
Section snippets
Source of the study
This review was conducted based on MOOSE guidelines for systematic literature searches using PubMed and MEDLINE between January 1955 and December 2013 [8]. Keywords searched included “UTROSCT” or “uterine tumor resembling ovarian sex cord tumor” limited to English literature.
Study selection
Case reports or case series with a detailed description of both clinical and histopathological findings were included. Exclusion criteria included review papers, cases with no or inadequate clinical information to
Characteristics and symptoms
There were 35 articles with a total of 43 cases of tumors classified as UTROSCT identified during the study period [2], [7], [9], [10], [11], [12], [13], [14], [3], [15], [16], [17], [18], [19], [20], [21], [22], [23], [24], [25], [26], [27], [28], [29], [30], [31], [32], [33], [34], [35], [36], [37], [38], [39], [40]. Among 43 cases reporting UTROSCT, Type I and Type II were reported in 5 (11.6%) and 17 (39.5%), respectively, and nearly half of reported UTROSCT did not subcategorize the
Discussion
UTROSCT is a relatively newly identified tumor; originally defined by Clement and Scully in 1976 essentially as one entity divided into two groups [1]. While both groups had elements resembling ovarian sex-cord tumors, these components constituted proportionally less of Type I tumors compared to Type II tumors, which was defined as being predominantly or exclusively composed of sex-cord components. As these tumors have become more widely recognized and diagnostic technology has improved, the
Disclosure
The authors declare that there is no conflict of interest to the study.
Funding support
None.
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2020, Gynecologic Oncology ReportsCitation Excerpt :Group I tumors contain focal sex cord-like elements (although > 10% by definition), and these tumors appear to be closely related to low-grade endometrial stromal sarcomas, sometimes harboring characteristic genetic rearrangements such as JAZF1–SUZ12. Group II tumors appear to be a separate entity (Blake et al., 2014; Clement and Scully, 1976). Molecular features of UTROSCT were elusive until RNA sequencing (RNA-seq) revealed recurrent NCOA1 rearrangements (Dickson et al., 2019; Goebel et al., 2020), usually partnering with estrogen receptor 1 (ESR1) or growth regulation by estrogen in breast cancer (GREB1).