Uterine Tumor Resembling Sex-Cord Tumors of the Ovary (Utrosct): A Case of Long-Term Response to Pazopanib

doi:10.29011/2574-7754.101090

Cited by 1 publication

(5 citation statements)

References 21 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Intraabdominal relapse and pulmonary metastases were the most common relapse sites [4,93,101,114,122]. Among all 511 cases, 18 patients died from or with the disease [4,93,100,111,112,114,122,129,130]. Those who died often experienced rapid disease progression, were metastatic at diagnosis, or progressed despite adjuvant therapies [93,111,112].…”

Section: Clinical Presentationmentioning

confidence: 99%

“…Among all 511 cases, 18 patients died from or with the disease [4,93,100,111,112,114,122,129,130]. Those who died often experienced rapid disease progression, were metastatic at diagnosis, or progressed despite adjuvant therapies [93,111,112]. This raises the question of whether the application of unproven chemotherapeutic or hormonal therapies in UTROSCT could potentially worsen prognosis.…”

Section: Clinical Presentationmentioning

confidence: 99%

“…Mutations in ESR1 ′ s ligand-binding domain have been correlated with resistance to hormone therapy in ER-positive breast cancer. Notably, UTROSCTs with ESR1 rearrangements may be resistant to estrogen blockade as the ER ligand-binding domain is lost in these fusions, potentially explaining the resistance to anti-hormonal treatments reported in relapsed UTROSCT [96,99,112].…”

Section: Gene Fusions In Utrosctmentioning

confidence: 99%

“…whereas Similarly, De Franciscis et al recommended transvaginal ultrasound examinations every six months and diagnostic hysteroscopy annually for five years [56]. The duration of follow-up remains undefined due to low number of recurrent cases, no established patterns of recurrence, and PFSs of 7 [112], 11 [96], 14 [110], 23 [99] or 32 [104] years being not uncommon.…”

Section: Follow-upmentioning

confidence: 99%

“…They continued with carboplatin, paclitaxel, and ombrabulin, with repeated disease progression. The subsequent treatments included epirubicin with pazopanib, letrozole, trabectedin, paclitaxel, BI860585, and exemestane and rechallenged with epirubicin, BMS-986148, and nivolumab until the final progression and death [112].…”

Section: Recurrence Treatmentsmentioning

confidence: 99%

See 4 more Smart Citations

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

View full text Add to dashboard Cite

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.

show abstract