Uterine tumors resembling ovarian sex cord tumors: an update. Int J Gynecol Pathol

Patho-Lab Diagnostics, Ness-Ziona, Israel.
International journal of gynecological pathology: official journal of the International Society of Gynecological Pathologists (Impact Factor: 1.67). 05/2008; 27(2):229-35. DOI: 10.1097/PGP.0b013e3181569a21
Source: PubMed


Tumors of the uterus resembling ovarian sex cord tumors were reported by Clement and Scully in 1976 and were divided in 2 groups: group 1, endometrial stromal tumors, and group 2, mural uterine tumors-both with elements resembling ovarian sex cord tumors. In the former, the sex cord component constitutes a minor portion of an endometrial stromal neoplasm, whereas in the latter, it is the predominant or exclusive component of a uterine wall lesion composed of a variety of mesenchymal elements. An origin from endometrial stromal cells, adenomyosis, stromal myosis, endometriosis, or multipotential cells within the myometrium was postulated in both groups of tumors. In group 1 tumors, the prognosis depends on the type, grade, and stage of the underlying stromal neoplasm. Group 2 tumors seemed to be benign, although because of the occasional recurrence of these tumors, they should be considered of low-grade malignant potential. In recent years, the histological features in group 2 were found to be much more varied than those in group 1 and consisted among others of retiform areas, glomeruloid structures, and Leydig-like cells. In group 1 tumors, the sex cord elements remained limited to cords, trabeculae, nests, and tubules. Eventually, the abbreviation ESTSCLE, or endometrial stromal tumors with sex cord-like elements, was given to group 1 tumors, whereas UTROSCT, or uterine tumor resembling ovarian sex cord tumor, was used for group 2 tumors. The most significant information in recently conducted studies concerns the immunophenotype of these lesions especially of UTROSCT. Out of the plethora of the immunohistochemical stains, a panel of 4 including calretinin, inhibin, CD99, and Melan A has emerged which seemed to be the most characteristic sex cord markers. Positivity for calretinin and at least for 1 of the other above-mentioned markers may thus confirm the diagnosis of UTROSCT. Endometrial stromal tumors with sex cord-like elements, on the other hand, usually express only 1 sex cord marker, mostly calretinin. However, additional studies are necessary to confirm these observations. In conclusion, UTROSCT and, to a lesser degree, ESTSCLE, are polyphenotypic neoplasms, which, according to the evidence available at present, most likely arise from pluripotential uterine mesenchymal cells. In UTROSCT, the differentiation into sex cord components is predominant or exclusive, whereas in ESTSCLE, it is minor.

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    • "Annals of Diagnostic Pathology component may be seen. These tumors are characterized by a complex immunophenotype, often with a coexpression of epithelial, smooth muscle, and sex cord markers as well as hormone receptors [7] [8] [9]. Uterine tumors resembling ovarian sex cord tumors occur in women of different ages, but often in women of reproductive age. "
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    ABSTRACT: Uterine tumors resembling ovarian sex-cord tumors (UTROSCT) cause difficulties, both with respect to diagnosis as well as to the nomenclature. They belong to the group of low grade malignant neoplasms and their clinical course likely depends on the percentage of the sex cord-like component. Morphologically, they can be divided into Type I and Type II with less or more than 50% of sex cord-like areas, respectively. Six patients with an age range of 24-63 years underwent the treatment for primary UTROSCT at the Cancer Center and Institute of Oncology in Warsaw, Poland, between 2000 and 2011. In addition to the surgery, four patients were treated with gestagens. Biopsies or excisions from the tumors were examined microscopically and immunohistochemically (IHC). Two cases were classified as Type I and four cases as Type II tumors. The tumor size ranged from 3 to 24 cm. The sex cord component varied from 25-70%. By IHC examination, the sex cord-like component was calretinin-positive, while the stromal component was positive for CD10 and negative for h-caldesmon in all the cases studied. Additionally, PGR positivity was found in all the cases and four tumors were positive for SMA, CKAE1/3 and inhibin. No recurrences were noted in any of the six patients over a 3-14.5 years of follow-up period. A correct subclassification of sarcomas of UTROSCT type is of crucial importance, since most patients with this rare neoplasm respond well to gestagen therapy and have a good prognosis, compared to other uterine stromal sarcomas.
    Full-text · Article · Aug 2014 · Annals of Diagnostic Pathology
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    • "In our cases, typical sex cord-like appearance was often masked by diffuse proliferation of epithelioid or spindle cells. Recent microscopic observation of UTROSCT expanded the original histopathological spectrum manifested by cords, trabeculae , nests and tubules of epithelioid cells to encompass some related features such as retiform, solid, and glomeruloid patterns [9]. Our cases might be a variant with solid pattern predominance. "
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    ABSTRACT: Uterine tumors with sex cord-like elements are divided in two groups; uterine tumors resembling ovarian sex cord tumors (UTROSCT), and endometrial stromal tumors with sex cord-like elements (ESTSCLE). UTROSCT is currently defined as the neoplasm predominantly or exclusively composed of sex cord-like elements, and generally behaves in a benign fashion. We studied two unusual cases of UTROSCT with metastasis. One case was a 38-year-old multiparous woman presented with hypermenorrhea. The tumor grew as an intramural mass, and metastasized to a pelvic lymph node. Another case was a 57-year-old woman presented with genital bleeding. The tumor grew as a submucosal exophytic mass, and metastasized to the epiploic appendix. Microscopic examination of the 2 cases revealed that they were composed of sex cord-like cells, epithelioid cells and spindle cells. They exhibited solid pattern in predominance. Both solid and sex cord-like elements showed similar immunoreactivities for more than 3 sex cord markers, but simultaneously showed different staining patterns for some other markers. Characteristic features of endometrial stroma such as tongue-like infiltration and spiral arteries-like arterioles were not observed. RT-PCR analysis confirmed the absence of JAZF1-SUZ12 gene fusion, supporting the histopathological diagnosis of UTROSCT rather than ESTSCLE. The current cases warned the potential risk of UTROSCT whose biological behavior is still uncertain. We discuss histopathological, immunohistochemical and molecular findings of UTROSCT with metastasis.
    Full-text · Article · Apr 2014 · International journal of clinical and experimental pathology
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    • "Thereby, the now established morphological and immunohistochemical features favor the origin of UTROSCT from pluripotential mesenchymal uterine cells. As in our cases, the presence of mixed epithelial-myoid immunophenotype in some UTROSCT strengthens this hypothesis [13] [14] [15]. Because UTROSCT have divergent morphologic features , the diagnosis of these tumors can be challenging for general surgical pathologists, especially in small biopsies [11]. "
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    ABSTRACT: Uterine tumors resembling ovarian sex cord-stromal tumors (UTROSCTs) are very rare. In this article, we present 3 cases that manifest classical histomorphological features alongside diverse immunohistochemical findings. As a distinctive finding, one of the patients had UTROSCT in the uterus and an ovarian sex cord tumor, called granulosa cell tumor, in the left ovary, simultaneously. Problems in diagnosing such pathologic condition generally arise because of the variable histologic picture of UTROSCT and may cause problems for general and other nongynecologic surgical pathologists. Immunohistochemically, these tumors express different markers that indicate their polyphenotypic origins.
    Full-text · Article · Dec 2010 · Annals of diagnostic pathology
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