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STK11 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 STK11 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 STK11 adnexal tumors (excluding tumors with no detectable STK11 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) STK11 adnexal tumors, PJS-associated STK11 adnexal tumors were slightly larger in diameter (mean 12.6 cm vs 8.9 cm) and had a longer interval between first recurrences (median 19 months vs 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 STK11 adnexal tumors. [ABSTRACT FROM AUTHOR]