Uterine Tumor Resembling Ovarian Sex Cord Tumors Type II with Vaginal Vault Recurrence

Marrucci, Oriana; Nicoletti, Paola; Mauriello, Alessandro; Facchetti, Simone; Patrizi, Lodovico; Ticconi, Carlo; Sesti, Francesco; Piccione, Emilio

doi:10.1155/2019/5231219

Cited by 8 publications

(20 citation statements)

References 11 publications

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“…Neither the size (ranging from 1 to 20 cm), nor the relationship to the myometrium (whether submucosal or intramural) or endometrium (such as polypoid intracavitary growth), facilitates the differentiation of UTROSCT from myomas or polyps. Additionally, it is noteworthy that UTROSCT often coexists with one or more leiomyomas in the same patient [18,27,41,103].…”

Section: Discussionmentioning

confidence: 98%

“…The coexistence of UTROSCT with other tumors at the time of surgery was not unusual, mostly with typical leiomyomas [18,27,[41][42][43]45,48,60,103], but also with other neoplasms such as a second UTROSCT [29,60], ovarian sex cord stromal tumors [41], gastrointestinal stromal tumors [94], endometrial adenoacanthoma [17], cervical intraepithelial neoplasia [120], or cervical metastasis from breast cancer [40].…”

Section: Clinical Presentationmentioning

confidence: 99%

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Uterine Tumors Resembling Ovarian Sex Cord Tumors (UTROSCTs): A Scoping Review of 511 Cases, Including 2 New Cases

Watrowski,

Palumbo,

Guerra

et al. 2024

Medicina

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Uterine Tumors Resembling Ovarian Sex Cord Tumors (UTROSCTs) are rare uterine mesenchymal neoplasms with uncertain biological potential. These tumors, which affect both premenopausal and postmenopausal women, usually have a benign clinical course. Nevertheless, local recurrences and distant metastases have been described. By analyzing 511 cases retrieved from individual reports and cases series, we provide here the most comprehensive overview of UTROSCT cases available in the literature, supplemented by two new cases of UTROSCTs. Case 1 was an asymptomatic 31-year-old woman who underwent a laparoscopic resection of a presumed leiomyoma. Case 2 was a 58-year-old postmenopausal woman with abnormal vaginal bleeding who underwent an outpatient hysteroscopic biopsy of a suspicious endometrial area. In both cases, immunohistochemical positivity for Calretinin and Inhibin was noted, typical for a sex cord differentiation. In both cases, total laparoscopic hysterectomy with bilateral salpingo-oophorectomy was performed. In light of the available literature, no pathognomonic clinical or imaging finding can be attributed to UTROSCT. Patients usually present with abnormal uterine bleeding or pelvic discomfort, but 20% of them are asymptomatic. In most cases, a simple hysterectomy appears to be the appropriate treatment, but for women who wish to become pregnant, uterus-preserving approaches should be discussed after excluding risk factors. Age, tumor size, lymphovascular space invasion, nuclear atypia, and cervical involvement are not reliable prognostic factors in UTROSCT. The current research suggests that aggressive cases (with extrauterine spread or recurrence) can be identified based on a distinct genetic and immunohistochemical phenotype. For instance, UTROSCTs characterized by GREB1::NCOA1-3 fusions and PD-L1 molecule expression appear to be predisposed to more aggressive behaviors and recurrence, with GREB1::NCOA2 being the most common gene fusion in recurrent tumors. Hence, redefining the criteria for UTROSCTs may allow a better selection of women suitable for fertility-sparing treatments or requiring more aggressive treatments in the future.

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Section: Discussionmentioning

confidence: 98%

Section: Clinical Presentationmentioning

confidence: 99%

Uterine Tumors Resembling Ovarian Sex Cord Tumors (UTROSCTs): A Scoping Review of 511 Cases, Including 2 New Cases

Watrowski,

Palumbo,

Guerra

et al. 2024

Medicina

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show abstract

“…In fact, although, UTROSCT usually exhibits benign behavior with a favorable prognosis, this neoplasm is classified as being of uncertain malignant potential, given its low rate of recurrence and the fact that it can sometimes cause metastases. In table 1 we have summarized the cases of UTROSCT which we found in literature from 1986 to 2023, that recurred or caused metastases and occasionally also death [19,20,30,31,36, (Table 1 From our review we observed that many cases with UTROSCT, which recurred or developed metastases, were observed in single case reports [31,35,[42][43][44][46][47][48][49][52][53][54][55]58,59] or sometimes in very small case series [19,30,40,45,50,57] (Table 1). The most frequent sites of recurrences or metastases in these cases were lymph nodes, peritoneum, omentum, vaginal vault, lung, and liver [44][45][46]49,[52][53][54][55].…”

Section: Clinical Datamentioning

confidence: 99%

Uterine Tumor Resembling Ovarian Sex Cord Tumor (UTROSCT): A Rare Polyphenotypic Neoplasm

Giordano,

Guareschi,

Thai

2024

Preprint

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Uterine Tumor Resembling Ovarian Sex Cord Stromal Tumor (UTROSCT) is a rare uterine mesenchymal neoplasm, which resembles ovarian sex cord tumors of the ovary. Although, in general, UTROSCT exhibits benign behavior with a favorable prognosis, this neoplasm is nonetheless classified as being of an uncertain ma-lignant potential, given its low rate of recurrence and the fact that it rarely pro-duces metastases (lymph nodes, epiploic appendix, omentum, small bowel, subcutaneous, lungs). Its histogenesis too is uncertain. Typically, UTROSCT oc-curs in peri-menopausal or menopausal women but can sometimes be observed in young women. Usually, this neoplasm can be found in the uterine corpus as a nodular intramural lesion, less frequently submucosal, subserosal or poly-poid/intracavitary. UTROSCT can cause abnormal bleeding, pelvic pain, enlarged uterus, mass sensation, but sometimes it is found purely by chance. This neo-plasm can be considered polyphenotypic on morphological, immunohistochem-ical, and genetic analyses. Generally, on microscopic examination, UTROSCT shows a predominant pattern of the cords, nests, and trabeculae typical of sex cord tumors of the ovary while, immunohistochemically, it is characterized by a co-expression of epithelial, smooth muscle, and sex cord markers. The aim of this review is to report clinical and pathological data and genetic alterations to estab-lish its impact on the prognosis and management of patients affected by this rare entity.

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“…These are considered to have low malignant potential. [1][2][3] UTROSCTs are mostly surgically : A total of 701 histologic diagnoses comprised of benign, precursor, and malignant lesions and accounted for 86.6%, 2.6%, and 10.8% respectively with preponderance in females. The common benign lesions included melanocytic nevus (17.7%), epidermal cyst (11%), hemangioma (8.9%), dermoid cyst (8.2%), chalazion (6.7%), and squamous papilloma (6.4%).…”

Section: Introductionmentioning

confidence: 99%

Uterine tumor resembling ovarian sex cord tumor: A case report

Shrestha,

Khadgi

2023

J Pathol Nep

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Uterine tumor resembling ovarian sex cord tumor (UTROSCT) is a rare uterine neoplasm of uncertain histogenesis, with presence of ovarian sex cord-like elements. These tumours typically occur in perimenopausal and postmenopausal women, and are considered to behave in an indolent fashion in most of the cases. However, recurrences and metastases have also been reported. We report a case of a 37 year old female who presented with lower abdominal pain and abnormal uterine bleeding, and underwent hysterectomy following a clinical and radiological suspicion of a submucosal uterine fibroid. Macroscopically the tumor resembled a Leiomyoma. However, the diagnosis of UTROSCT was made after histopathological examination and immunohistochemical studies. Clinicians and pathologists need to be aware of this diagnosis, given the rarity of this condition and potential aggressive clinical course.

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Uterine Tumor Resembling Ovarian Sex Cord Tumors Type II with Vaginal Vault Recurrence

Cited by 8 publications

References 11 publications

Uterine Tumors Resembling Ovarian Sex Cord Tumors (UTROSCTs): A Scoping Review of 511 Cases, Including 2 New Cases

Uterine Tumors Resembling Ovarian Sex Cord Tumors (UTROSCTs): A Scoping Review of 511 Cases, Including 2 New Cases

Uterine Tumor Resembling Ovarian Sex Cord Tumor (UTROSCT): A Rare Polyphenotypic Neoplasm

Uterine tumor resembling ovarian sex cord tumor: A case report

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