Distribution Characteristics in Clinical Phenotype and Immunohistochemistry of STK11 Adnexal Tumors: Case Report and Narrative Synthesis

Abstract

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.

Keywords

female adnexal tumor Peutz-Jeghers syndrome paratubal

Get full access to this article

View all access options for this article.

References

Bennett

Young

Howitt

, et al. A distinctive adnexal (usually paratubal) neoplasm often associated with peutz-jeghers syndrome and characterized by STK11 alterations (STK11 adnexal tumor): a report of 22 cases. Am J Surg Pathol. 2021;45(8):1061–1074. doi:https://doi.org/10.1097/PAS.0000000000001677

Bennett

Ritterhouse

Furtado

, et al. Female adnexal tumors of probable Wolffian origin: morphological, immunohistochemical, and molecular analysis of 15 cases. Mod Pathol. 2020;33(4):734–747. doi:https://doi.org/10.1038/s41379-019-0375-9

Shackelford

Shaw

. The LKB1-AMPK pathway: metabolism and growth control in tumour suppression. Nat Rev Cancer. 2009;9(8):563–575. doi:https://doi.org/10.1038/nrc2676

Connolly

Katabuchi

Cliby

Cho

. Somatic mutations in the STK11/LKB1 gene are uncommon in rare gynecological tumor types associated with Peutz-Jegher's syndrome. Am J Pathol. 2000;156(1):339–345. doi:https://doi.org/10.1016/S0002-9440(10)64735-9

Watanabe

Soeda

Endo

, et al. Rare hereditary gynecological cancer syndromes. Int J Mol Sci. 2022;23(3):1563. doi:https://doi.org/10.3390/ijms23031563

Mascaro

Aboelnasr

Masood

Yague

Moran

El-Bahrawy

. Exploring the histogenesis of STK11 adnexal tumour using electron microscopy. Virchows Arch. 2024;485(4):683-690. doi:https://doi.org/10.1007/s00428-024-03763-2

Estevez-Diz

Bonadio

Carvalho

. Everolimus plus anastrozole for female adnexal tumor of probable Wolffian origin (FATWO) with STK11 mutation. Gynecol Oncol Rep. 2021;37:100838. doi:https://doi.org/10.1016/j.gore.2021.100838

Zhang

, et al. Detection and analysis of common pathogenic germline mutations in Peutz-Jeghers syndrome. World J Gastroenterol. 2021;27(39):6631–6646. doi:https://doi.org/10.3748/wjg.v27.i39.6631

Sharma

Slack

Parra-Herran

, et al. STK11 Adnexal tumor in an adolescent female: diagnostic pitfalls of a recently described entity. Pediatr Dev Pathol. 2023;26(5):486–493. doi:https://doi.org/10.1177/10935266231176681

10.

Skoulidis

Goldberg

Greenawalt

, et al. STK11/LKB1 Mutations and PD-1 inhibitor resistance in KRAS-mutant lung adenocarcinoma. Cancer Discov. 2018;8(7):822–835. doi:https://doi.org/10.1158/2159-8290.CD-18-0099

Supplementary Material

Please find the following supplemental material available below.

For Open Access articles published under a Creative Commons License, all supplemental material carries the same license as the article it is associated with.

For non-Open Access articles published, all supplemental material carries a non-exclusive license, and permission requests for re-use of supplemental material or any part of supplemental material shall be sent directly to the copyright owner as specified in the copyright notice associated with the article.

0.00 MB

0.03 MB

0.02 MB