adnexal tumors are recently named tumors of the female adnexal region, associated with Peutz-Jeghers syndrome (PJS). There is a lack of studies on the clinical phenotypes of PJS concerning the pathology and molecular characteristics of adnexal tumors. We searched for 781 relevant studies through PubMed and Web of Science, and preliminary statistical and grouping comparisons were made using the screened study data combined with our patient. A total of 25 patients with adnexal tumors (excluding tumors with no detectable alterations) were reported in 4 studies. The tumors were most common in middle-aged women (mean age of 39 years) and typically located at paratubal sites (76%). Nearly half of patients (45%) had PJS manifestations or molecular changes. Compared with sporadic (non-PJS) adnexal tumors, PJS-associated adnexal tumors were slightly larger in diameter (mean 12.6 cm 8.9 cm) and had a longer interval between first recurrences (median 19 months 7 months). Primary tumors with pelvic recurrence had a reduced keratin 7 positive rate and tended to grow in non-paratubal sites. However, overall, the clinicopathological characteristics of PJS-associated tumors and sporadic tumors were similar, as was the immunohistochemical expression of primary and recurrent tumors. More specimens and studies are needed to support these observations and further delineate the characteristics of adnexal tumors.

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http://dx.doi.org/10.1177/10668969241309939DOI Listing

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