Uterine Tumors Resembling Ovarian Sex Cord Tumors

Abstract

Uterine tumors resembling ovarian sex cord tumors (UTROSCT) are rare neoplasms of unknown etiology. Only 67 cases have been reported in the literature, to our knowledge, so far. The neoplasm usually occurs in middle-aged women. Most patients present with abnormal uterine bleeding and/or abdominal pain, along with an enlarged uterus or a palpable uterine mass. There is no specific imaging finding, and the diagnosis is made exclusively on histopathologic examination. A multitude of architectural patterns are described, which include plexiform cords, anastomosing trabeculae, watered-silk, microfollicle, macrofollicle, tubules, retiform, solid cellular islands, and diffuse pattern of growth. The neoplastic cells are usually small with round to ovoid nuclei, nuclear monotony, mild nuclear hyperchromasia, and inconspicuous nucleoli with scant eosinophilic cytoplasm. Nuclear grooves are rare. Mitotic figures are infrequent, and necrosis is mostly absent. This tumor depicts a diverse immunohistochemical profile with expression of sex cord, epithelial, and smooth muscle lineages markers. Sex cord markers, such as inhibin, calretinin, CD99, WT1, and MART-1; epithelial markers, such as pancytokeratin and epithelial membrane antigen; smooth muscle markers, such as smooth muscle actin, desmin, and histone deacetylase 8; and miscellaneous markers, such as CD10, estrogen receptor, progesterone receptor, S100, and CD117, are often coexpressed. Immunoexpression for calretinin and at least for one of the other sex cord markers is required to establish a diagnosis of UTROSCT. Hysterectomy with or without bilateral salpingo-oophorectomy is usually the treatment for UTROSCT. Although most UTROSCTs behave benignly, some do recur, and thus, this entity should be considered as a tumor of low malignant potential. In this review, we discuss the current knowledge on UTROSCT and its clinical relevance.

Uterine tumors resembling ovarian sex cord tumors: A case report and literature review

“…Histologically, the tumors are comprised of epithelioid cells and nests of sex cord-like elements (6). The diagnosis is typically reinforced by the results of polyphenotypic IHC expression in the form of positivity for sex cord (calretinin, CD99, inhibin, Wilms' tumor protein 1 and melanoma-associated antigen recognized by T cells), epithelial (pancytokeratin and EMA), myeloid (SMA, desmin and histone deacetylase 8) and a number of other markers (CD10, ER, PR, s100 and CD117) (6). Furthermore, the identification of two balanced chromosomal translocations, t(X;6) (p22.3;q23.1) and t(4;18)(q21.1;q21.3), was reported in a case of UTROSCT in a study by Wang et al (11).…”

“…Staats et al (12) indicated the presence of juxtaposed with another zinc finger protein 1-suppressor-of-zeste 12 protein gene fusion in a case of ESTSCLE, but not in cases of UTROSCT. Epithelioid leiomyoma, low-grade endometrial stromal sarcoma with sex cord elements, endometrioid carcinoma with sex cord-like features, adenosarcoma and carcinosarcoma are examples of tumors listed in the differential diagnoses (6). ESTSCLEs are differentiated from UTROSCTs due to their more aggressive nature and the possibility of metastasis and recurrence (6).…”

“…UTROSCTs are composed of epitheloid cells and nests of sex cord-like elements (6). Certain malignant neoplasms, including endometrial stromal sarcoma with sex cord elements, endometrioid carcinoma with sex cord-like features, adenosarcoma and carcinosarcoma may cause difficulties with regard to UTROSCT diagnosis, due to histopathological similarities (6). These tumors exhibit a low malignant potential (7), however, extrauterine or lymph node metastasis has been reported previously (2,5,8).…”

“…The tumors are associated with a risk of metastasis and recurrence, and are known as endometrial stromal tumors with sex cord-like elements (ESTSCLEs). By contrast, group II tumors are composed of >50% sex cord-like cells, typically behave benignly and are known as UTROSCTs (2,5,6). It has been determined that UTROSCTs exhibit polyphenotypic immunohistochemical (IHC) expression in the form of positivity for sex cord, epithelial and myeloid markers, and are classified as miscellaneous tumors of the uterine body (6).…”

“…To date, no specific diagnostic imaging features have been identified and thus, UTROSCTs are usually diagnosed by tissue biopsy (7). UTROSCTs are composed of epitheloid cells and nests of sex cord-like elements (6). Certain malignant neoplasms, including endometrial stromal sarcoma with sex cord elements, endometrioid carcinoma with sex cord-like features, adenosarcoma and carcinosarcoma may cause difficulties with regard to UTROSCT diagnosis, due to histopathological similarities (6).…”