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by claude@2026-06, 2026-06-11
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This pathology study reviewed 20 adnexal endometrioid carcinomas of the ovary or fallopian tube with sex cord-like morphology, assessing their histology, immunohistochemistry, and available genomic data in patients aged 32–78 years. Tumors showed a range of sex cord-like patterns and often had fibromatous stroma and background endometriosis or endometrioid adenofibromas, with a key finding that PAX8 was positive in only 2/20 while SOX17 was positive in all and beta-catenin had aberrant nuclear staining in 17/20. Of the 10 sequenced cases, 9 had activating pathogenic CTNNB1 variants, and all sequenced tumors lacked mismatch repair gene, TP53, and POLE alterations; the main caveat is the small number of tumors with sequencing. This paper is centrally about endometriosis — it reports an observed association between these sex cord-like endometrioid carcinomas and concurrent endometriosis (6/20 cases).
Abstract
Sex cord-like morphology is now a well-known feature of occasional examples of endometrioid carcinoma of the ovary or fallopian tube. Recently, we have observed that these tumors are frequently negative for PAX8, prompting us to review their morphologic, immunohistochemical, and genomic spectrum. Twenty tumors (17 ovarian, 3 fallopian tube) with available tissue were identified in patients ranging from 32 to 78 (median 60) years. Sex cord-like patterns included cords/trabeculae (n=17), small tubular glands (n=16), and "granulosa-like" nests (n=12). In addition, 1 had ependymoma-like features with perivascular pseudorosettes and 3 had focal spindled cells. Conventional endometrioid glands were often sparse. All had conspicuous fibromatous stroma and 11 tumors had background endometrioid adenofibromas. Six tumors had associated endometriosis. PAX8 was positive in only 2/20 (diffuse in both), while SOX17 was positive in all (focal in 1, diffuse in 19). Beta-catenin showed aberrant nuclear staining in 17/20. Of the 10 sequenced tumors, 9 showed activating pathogenic variants in CTNNB1; each of these also showed nuclear beta-catenin staining. All lacked alterations in mismatch repair genes, TP53 , and POLE . In summary, adnexal endometrioid carcinomas with sex cord-like features are frequently PAX8-negative, which may result in diagnostic difficulty. However, SOX17 is typically positive and thus useful to establish the diagnosis. Most of these tumors are classified in the "no specific molecular profile" subgroup and have high rates of nuclear beta-catenin/ CTNNB1 alterations. Awareness of these morphologic and immunohistochemical associations may help avoid misclassification as sex cord-stromal or other neoplasms.
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Sex cord-like morphology is now a well-known feature of occasional examples of endometrioid carcinoma of the ovary or fallopian tube. Recently, we have observed that these tumors are frequently negative for PAX8, prompting us to review their morphologic, immunohistochemical, and genomic spectrum. Twenty tumors (17 ovarian, 3 fallopian tube) with available tissue were identified in patients ranging from 32 to 78 (median 60) years. Sex cord-like patterns included cords/trabeculae (n=17), small tubular glands (n=16), and “granulosa-like” nests (n=12). In addition, 1 had ependymoma-like features with perivascular pseudorosettes and 3 had focal spindled cells. Conventional endometrioid glands were often sparse. All had conspicuous fibromatous stroma and 11 tumors had background endometrioid adenofibromas. Six tumors had associated endometriosis. PAX8 was positive in only 2/20 (diffuse in both), while SOX17 was positive in all (focal in 1, diffuse in 19). Beta-catenin showed aberrant nuclear staining in 17/20. Of the 10 sequenced tumors, 9 showed activating pathogenic variants in CTNNB1; each of these also showed nuclear beta-catenin staining. All lacked alterations in mismatch repair genes, TP53, and POLE. In summary, adnexal endometrioid carcinomas with sex cord-like features are frequently PAX8-negative, which may result in diagnostic difficulty. However, SOX17 is typically positive and thus useful to establish the diagnosis. Most of these tumors are classified in the “no specific molecular profile” subgroup and have high rates of nuclear beta-catenin/CTNNB1 alterations. Awareness of these morphologic and immunohistochemical associations may help avoid misclassification as sex cord-stromal or other neoplasms.
Adnexal Endometrioid Carcinomas With Sex Cord-Like Morphology are Frequently PAX8-Negative, SOX17-Positive, and Enriched for CTNNB1 Alterations
Plain Language SummaryThis study examined 20 cases of endometrioid carcinoma in the ovary or fallopian tube, focusing on their unique sex cord-like features and molecular profiles. These tumors often lack PAX8 expression, which can complicate diagnosis, but they consistently express SOX17, aiding identification. Most tumors showed nuclear beta-catenin staining and CTNNB1 mutations, but they are classified in the \u0022no specific molecular profile\u0022 subgroup. They did not have mutations in mismatch repair genes, TP53, or POLE. Recognizing these characteristics can prevent misdiagnosis as other types of tumors. The findings highlight the importance of SOX17 and beta-catenin in diagnosing these carcinomas.
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