Uterine Tumors Resembling Ovarian Sex Cord Tumors (UTROSCT) Lack the JAZF1-JJAZ1 Translocation Frequently Seen in Endometrial Stromal Tumors

Staats, Paul N.; García, Joaquín J.; Dias‐Santagata, Dora; Kuhlmann, Georgiana; Stubbs, Hannah; McCluggage, W. Glenn; Nictolis, Michele De; Kommoss, Friedrich; Soslow, Robert A.; Iafrate, A. John; Oliva, Esther

doi:10.1097/pas.0b013e3181a7b9cf

Cited by 79 publications

(51 citation statements)

References 47 publications

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“…17,18 Endometrioid carcinoma with sex-cordlike features is an unusual tumor of the endometrium. Microscopically, the tumor consists of small, hollow tubules, anastomosing cords and trabeculae and tightly packed nests.…”

Section: Differential Diagnosismentioning

confidence: 99%

Uterine Tumors Resembling Ovarian Sex Cord Tumors

Pradhan¹,

Mohanty²

2013

Archives of Pathology &Amp; Laboratory Medicine

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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.

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Section: Differential Diagnosismentioning

confidence: 99%

Uterine Tumors Resembling Ovarian Sex Cord Tumors

Pradhan¹,

Mohanty²

2013

Archives of Pathology &Amp; Laboratory Medicine

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“…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.…”

Section: Introductionmentioning

confidence: 99%

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

Cömert

Kılıç²,

Çavuşoğlu³

et al. 2018

TJPATH

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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...

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“…Whether the gene fusion is present in other types of variant components found in fusion-positive variant ESTs remains to be determined. The JAZF1/JJAZ1 gene fusion also appears specific to ESTs and has not been found in leiomyomas (11), leiomyosarcomas (5), uterine tumors resembling ovarian sex-cord tumors (24), lung carcinomas (3), gastric carcinomas (3), or hepatic carcinomas (3) by RT-PCR [59,68,69,72]. Among these tumors, the t(7;17) translocation was not found in 4 cellular leiomyomas [69] and 20 uterine tumors resembling ovarian sex-cord tumors by FISH [72].…”

Section: Molecular Alterations In Endometrial Stromal Tumorsmentioning

confidence: 97%