International Journal of Gynecological Pathology最新文献

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SOX2 Can Support a Diagnosis of PiMHEC With Rare to Absent Ghost Cells and Can Distinguish PiMHEC From Its Most Problematic Mimickers. SOX2可以支持罕见或缺失鬼细胞的PiMHEC诊断,并可以将PiMHEC与最问题的模仿者区分开来。
IF 2.1 4区 医学
International Journal of Gynecological Pathology Pub Date : 2026-11-01 Epub Date: 2026-07-24 DOI: 10.1097/PGP.0000000000001197
Jin Xu, Paul S Weisman
{"title":"SOX2 Can Support a Diagnosis of PiMHEC With Rare to Absent Ghost Cells and Can Distinguish PiMHEC From Its Most Problematic Mimickers.","authors":"Jin Xu, Paul S Weisman","doi":"10.1097/PGP.0000000000001197","DOIUrl":"10.1097/PGP.0000000000001197","url":null,"abstract":"<p><p>Pilomatrix-like high-grade endometrioid carcinoma (PiMHEC) is an aggressive variant of endometrioid adenocarcinoma characterized by divergent pilomatrical differentiation. While classic cases are usually recognizable, the diagnosis can be challenging when ghost cells are rare or absent, or when encountering mimickers that share overlapping features. We investigated the utility of SOX2 immunohistochemistry as a diagnostic marker for PiMHEC. SOX2 expression was evaluated in 26 cases of PiMHEC (24 endometrial, 2 ovarian). We also analyzed problematic mimickers, including non-PiMHEC FIGO grade 3 endometrioid carcinomas (EMCA) with aberrant beta-catenin expression (n=4) and undifferentiated/dedifferentiated EMCA (UD-EMCA) with aberrant beta-catenin expression (n=7). In addition, a tissue microarray (TMA) of 84 EMCA cases (FIGO grade 1-3 and UD-EMCA) was screened. All 26 PiMHEC cases (100 % ) showed robust SOX2 positivity. In contrast, all non-PiMHEC FIGO grade 3 EMCAs with aberrant beta-catenin expression were negative for SOX2. While 2 of 7 UD-EMCAs with aberrant beta-catenin expression showed very focal SOX2 expression, these were easily distinguished from PiMHEC by their lack of cytokeratin expression. In the TMA cohort, only 5 of 84 cases (all FIGO grade 3) showed SOX2 positivity; these cases all had membranous beta-catenin expression, had no histologic features of PiMHEC and followed an indolent clinical course. SOX2 is a useful marker for confirming a diagnosis of PiMHEC, especially when ghost cells are inconspicuous. It effectively differentiates PiMHEC from its FIGO grade 3 mimickers with aberrant beta-catenin expression.</p>","PeriodicalId":14001,"journal":{"name":"International Journal of Gynecological Pathology","volume":" ","pages":"437-446"},"PeriodicalIF":2.1,"publicationDate":"2026-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148584321","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Bizarre But Benign: A Case Report of an EHHADH::RAD51B Rearranged Endometrial Polyp With Bizarre Stromal Cells. 奇异但良性:EHHADH::RAD51B重排子宫内膜息肉伴奇异间质细胞一例报告。
IF 2.1 4区 医学
International Journal of Gynecological Pathology Pub Date : 2026-10-22 DOI: 10.1097/PGP.0000000000001222
Jeanne Camille Cardenas, Christopher Felicelli
{"title":"Bizarre But Benign: A Case Report of an EHHADH::RAD51B Rearranged Endometrial Polyp With Bizarre Stromal Cells.","authors":"Jeanne Camille Cardenas, Christopher Felicelli","doi":"10.1097/PGP.0000000000001222","DOIUrl":"https://doi.org/10.1097/PGP.0000000000001222","url":null,"abstract":"<p><p>Endometrial polyps with bizarre stromal cells are a rare histological variant of endometrial polyps characterized by striking nuclear atypia that can mimic features of malignant mesenchymal neoplasms, particularly Müllerian adenosarcoma (MA). Despite their alarming cytological appearance, these lesions typically follow a benign clinical course, making an accurate diagnosis essential to avoid unnecessary aggressive surgical interventions. Here, we present the case of a 53-yr-old woman with a 3-mo history of postmenopausal bleeding. Imaging revealed an 18 mm endometrial lesion with circumferential Doppler flow. Subsequent hysteroscopic resection and histopathological examination identified an endometrial polyp containing atypical, enlarged, and hyperchromatic stromal spindle cells with characteristic \"smudged\" chromatin. Mitotic activity was negligible, and there was no evidence of the leaf-like architecture or periglandular cuffing typical of MA. Immunohistochemical profiling showed diffuse positivity for CD10 and HMGA2, p53 wild-type expression, and a low Ki-67 proliferation index (<5%). Molecular analysis via FusionPlex Next Generation Sequencing revealed an EHHADH::RAD51B fusion, a novel gene fusion that expands the limited body of molecular data on endometrial polyps with bizarre stromal cells.</p>","PeriodicalId":14001,"journal":{"name":"International Journal of Gynecological Pathology","volume":" ","pages":""},"PeriodicalIF":2.1,"publicationDate":"2026-10-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149244374","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Novel Somatic Co-occurrence of IDH1 and TENT5C Mutations in Juvenile Granulosa Cell Tumor With Sertoli Elements Occurring in Ollier Disease. 在肝脏疾病中发生的支持因子的幼年颗粒细胞瘤中IDH1和TENT5C突变的新体细胞共现
IF 2.1 4区 医学
International Journal of Gynecological Pathology Pub Date : 2026-10-19 DOI: 10.1097/PGP.0000000000001220
Nasser El Laham, Maxime Fastrez, Nicky D'Haene, Jean-Christophe Noel
{"title":"Novel Somatic Co-occurrence of IDH1 and TENT5C Mutations in Juvenile Granulosa Cell Tumor With Sertoli Elements Occurring in Ollier Disease.","authors":"Nasser El Laham, Maxime Fastrez, Nicky D'Haene, Jean-Christophe Noel","doi":"10.1097/PGP.0000000000001220","DOIUrl":"https://doi.org/10.1097/PGP.0000000000001220","url":null,"abstract":"<p><p>We report a rare ovarian juvenile granulosa cell tumor with Sertoli differentiation occurring in a woman with Ollier disease and concurrent somatic IDH1 R132C and TENT5C mutations. Histology showed a predominantly juvenile granulosa cell tumor with a minor Sertoli-like component, and immunohistochemistry confirmed sex cord-stromal differentiation. The case is notable for the identification of TENT5C, a noncanonical poly(A) polymerase with emerging tumor suppressor relevance. Although not established as a recurrent driver in ovarian sex cord-stromal tumors, TENT5C alterations may represent an additional molecular event contributing to tumor biology alongside IDH1-associated mosaicism.</p>","PeriodicalId":14001,"journal":{"name":"International Journal of Gynecological Pathology","volume":" ","pages":""},"PeriodicalIF":2.1,"publicationDate":"2026-10-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149223922","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Vulvovaginal Myxoid Epithelioid Smooth Muscle Tumor With NCOA2 Rearrangement: Case Report and Literature Review. 外阴阴道黏液样上皮样平滑肌肿瘤伴NCOA2重排1例并文献复习。
IF 2.1 4区 医学
International Journal of Gynecological Pathology Pub Date : 2026-10-12 DOI: 10.1097/PGP.0000000000001221
Lingxuan Li, Ying Shao, Xiaofei Zhang, Bingjian Lu
{"title":"Vulvovaginal Myxoid Epithelioid Smooth Muscle Tumor With NCOA2 Rearrangement: Case Report and Literature Review.","authors":"Lingxuan Li, Ying Shao, Xiaofei Zhang, Bingjian Lu","doi":"10.1097/PGP.0000000000001221","DOIUrl":"https://doi.org/10.1097/PGP.0000000000001221","url":null,"abstract":"<p><p>Vulvovaginal myxoid epithelioid smooth muscle tumor with NCOA2 rearrangement is an emerging, rare mesenchymal neoplasm of the female genital tract. We reported a 56-yr-old Chinese woman with a slow-growing, tender mass in the right labium majus. The tumor was composed of epithelioid and spindle cells with smooth muscle differentiation arranged in short fascicles or small nests with prominent myxoid stroma and distinct microcystic formation. The tumor cells were strongly positive for smooth muscle actin, desmin, and caldesmon, negative for estrogen receptor, and had a low Ki-67 labeling index (~1%). Fluorescence in situ hybridization indicated NCOA2 rearrangement, but no MEF2D rearrangement. Our case expands the spectrum of this rare entity with regard to negative estrogen receptor and highlights the value of molecular testing for accurate diagnosis.</p>","PeriodicalId":14001,"journal":{"name":"International Journal of Gynecological Pathology","volume":" ","pages":""},"PeriodicalIF":2.1,"publicationDate":"2026-10-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149167378","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Diagnostically Confusing Aspects of the Physiologic Ovarian Follicle: A Report of 57 Cases and Brief Review of Selected Other Non-neoplastic Ovarian Lesions. 生理性卵巢卵泡的诊断混淆:附57例报告及其他非肿瘤性卵巢病变的简要回顾。
IF 2.1 4区 医学
International Journal of Gynecological Pathology Pub Date : 2026-09-28 DOI: 10.1097/PGP.0000000000001217
Negar Taheri, Robert H Young, Esther Oliva, Kyle M Devins
{"title":"Diagnostically Confusing Aspects of the Physiologic Ovarian Follicle: A Report of 57 Cases and Brief Review of Selected Other Non-neoplastic Ovarian Lesions.","authors":"Negar Taheri, Robert H Young, Esther Oliva, Kyle M Devins","doi":"10.1097/PGP.0000000000001217","DOIUrl":"https://doi.org/10.1097/PGP.0000000000001217","url":null,"abstract":"<p><p>We performed a retrospective review of cases sent in consultation wherein physiological ovarian follicles, corpora lutea, or their constituent cells raised concern for malignancy based on architectural or cytologic features. The 57 cases were broadly divided into 4 groups. The largest (n=31) showed tangential sections through the granulosa (n=18) or theca (n=13) layers of developing follicles. The irregular appearance, high baseline mitotic activity (median: 20 mitoses/10 high-power fields), and occasional Call-Exner-like spaces (n=6) contributed to concern. The second group (n=20) consisted of corpora lutea in which tangential sectioning or partial involution imparted an unusually complex architecture; mitoses were prominent in 8 of these cases, and endocrine-type atypia was seen in 5. The third group consisted of 2 cases in which corpora albicantia formed an appreciable mass. The final group consisted of 4 cases where artifactually detached granulosa cells from developing follicles were present within vascular spaces, mimicking intravascular malignancy. Extensive sampling revealed no associated ovarian neoplasm. In discussing these unusual appearances of physiological granulosa and theca cells, we briefly review the histology of developing follicles and other benign proliferations that may mimic sex cord-stromal neoplasia. Awareness of these variations of normal histology is critical to prevent misdiagnosis of malignancy.</p>","PeriodicalId":14001,"journal":{"name":"International Journal of Gynecological Pathology","volume":" ","pages":""},"PeriodicalIF":2.1,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149074604","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Primary Vulvar HPV-independent Adenosquamous Carcinoma: A Case Series of a Rare Neoplasm With an Apparent Predilection for the Upper Vulva. 原发性外阴不依赖hpv的腺鳞癌:一例明显倾向于上外阴的罕见肿瘤。
IF 2.1 4区 医学
International Journal of Gynecological Pathology Pub Date : 2026-09-28 DOI: 10.1097/PGP.0000000000001206
Lisa Vermij, Tjalling Bosse, Maysa Al-Hussaini, W Glenn McCluggage
{"title":"Primary Vulvar HPV-independent Adenosquamous Carcinoma: A Case Series of a Rare Neoplasm With an Apparent Predilection for the Upper Vulva.","authors":"Lisa Vermij, Tjalling Bosse, Maysa Al-Hussaini, W Glenn McCluggage","doi":"10.1097/PGP.0000000000001206","DOIUrl":"https://doi.org/10.1097/PGP.0000000000001206","url":null,"abstract":"<p><p>Adenosquamous carcinoma is a rare malignancy described in a variety of body sites, including the uterine cervix. These tumors exhibit morphologically unequivocal areas of squamous and glandular differentiation and are typically associated with an aggressive clinical course. Primary adenosquamous carcinoma of the vulva, arising outside the Bartholin gland, has only been described in a few case reports, is poorly characterized and is currently not included in the 5th Edition WHO Classification of Female Genital Tumours. We report a series of 3 primary vulvar HPV-independent adenosquamous carcinomas, all arising in the upper vulva close to the junction with the vagina, in close proximity to the urethral meatus. All tumors showed surface ulceration, and there were no features of lichen sclerosus or vulvar intraepithelial neoplasia in the adjacent squamous epithelium. The squamous components ranged from 40% to 70% of the neoplasms. All tumors exhibited mutation-type p53 expression and negative/non-block-type staining for p16. Molecular testing confirmed a TP53 mutation in all tumors and showed an additional AMER1 mutation in one. Two patients presented with lymph node metastasis at the time of diagnosis or shortly thereafter. None of the patients died of their disease, with follow-up ranging from 2 to 81 mo. Our findings suggest that primary vulvar HPV-independent adenosquamous carcinoma is a distinct morphologic entity that warrants inclusion in future classifications of primary vulvar neoplasms.</p>","PeriodicalId":14001,"journal":{"name":"International Journal of Gynecological Pathology","volume":" ","pages":""},"PeriodicalIF":2.1,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149074642","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Repurposing Gastric Cancer Biomarkers for Mucinous Ovarian Carcinoma: ERBB2 and Claudin 18.2 as Therapeutic Targets. 重新利用胃癌生物标志物治疗黏液性卵巢癌:ERBB2和Claudin 18.2作为治疗靶点
IF 2.1 4区 医学
International Journal of Gynecological Pathology Pub Date : 2026-09-28 DOI: 10.1097/PGP.0000000000001219
Shuaijin Wang, Astrid De Boeck, Eun Young Kang, Nicola S Meagher, Kylie L Gorringe, Gregg S Nelson, Cheng Han Lee, Parham Minoo, Martin Köbel
{"title":"Repurposing Gastric Cancer Biomarkers for Mucinous Ovarian Carcinoma: ERBB2 and Claudin 18.2 as Therapeutic Targets.","authors":"Shuaijin Wang, Astrid De Boeck, Eun Young Kang, Nicola S Meagher, Kylie L Gorringe, Gregg S Nelson, Cheng Han Lee, Parham Minoo, Martin Köbel","doi":"10.1097/PGP.0000000000001219","DOIUrl":"https://doi.org/10.1097/PGP.0000000000001219","url":null,"abstract":"<p><p>Mucinous ovarian carcinomas (MOCs) have an upper-gastrointestinal cell phenotype (CK7+/SATB2-) similar to pancreatic ductal adenocarcinoma (PDAC) and gastric adenocarcinoma (GA). We aimed to develop an immunohistochemical panel that accurately discriminates MOC from GA. A secondary objective was to compare molecular and targetable tissue-based biomarkers across MOC, GA, and PDAC. Immunohistochemistry was performed on tissue microarrays representing 103 MOC, 265 PDAC, and 159 GA for markers of cell lineage (CK17, MEP1A, PAX8, CDX2, and HIK1083), key molecular alterations [TP53, Mismatch Repair deficiency (MMRd), CTNNB1, ARID1A, and SMAD4], as well as potential antibody targets (ERBB2 and Claudin 18.2). In situ hybridization was performed for Epstein-Barr virus-encoded RNA. A lasso-penalized nominal logistic regression model discriminated the 3 entities with low overall accuracy [77.8%, area under the curve (AUC)=0.91]. Discrimination from others was highest for PDAC (AUC=0.93) and MOC (AUC=0.92), but lowest for GA (AUC=0.87), and MOC was more similar to GA than to PDAC. MOC more frequently showed Claudin 18.2 PS2+ high (42%) and ERBB2 IHC3+ (25%) than GA (Claudin 18.2, 25%; ERBB2, 3%; MMRd, 19%) and PDAC (Claudin 18.2, 10%; ERBB2, 0%; MMRd, 2%). At least 1 potentially targetable alteration was present in 60% of MOC, 43% of GA, and 12% of PDAC. While our marker panel is of insufficient diagnostic accuracy to distinguish MOC from GA, based on the similarity between MOC and GA, consideration should be given to include MOC in clinical basket trials targeting ERBB2 and Claudin 18.2.</p>","PeriodicalId":14001,"journal":{"name":"International Journal of Gynecological Pathology","volume":" ","pages":""},"PeriodicalIF":2.1,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149074628","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Interobserver Variability in Diagnosing STIC With Frequent Underdiagnosis of Early High-grade Serous Carcinoma: Insights From a Multicenter Review From the CANSTIC Study. 诊断STIC伴早期高级别浆液性癌常漏诊的观察者间差异:来自CANSTIC研究多中心综述的见解。
IF 2.1 4区 医学
International Journal of Gynecological Pathology Pub Date : 2026-09-28 DOI: 10.1097/PGP.0000000000001218
Sarah E J Khan, Martin Köbel, Elisabeth Spénard, Lien Hoang, Katherine Grondin, Marcus Q Bernardini, Marjan Rouzbahman
{"title":"Interobserver Variability in Diagnosing STIC With Frequent Underdiagnosis of Early High-grade Serous Carcinoma: Insights From a Multicenter Review From the CANSTIC Study.","authors":"Sarah E J Khan, Martin Köbel, Elisabeth Spénard, Lien Hoang, Katherine Grondin, Marcus Q Bernardini, Marjan Rouzbahman","doi":"10.1097/PGP.0000000000001218","DOIUrl":"https://doi.org/10.1097/PGP.0000000000001218","url":null,"abstract":"<p><p>It has become widely accepted that most cases of tubo-ovarian high-grade serous carcinoma (HGSC) arise from precursor lesions in the fallopian tube called serous tubal intraepithelial carcinoma (STIC). This study evaluated the interobserver reproducibility of STIC diagnosis and its diagnostic boundaries from a multicentre Canadian cohort study. The multicentre CANSTIC study included patients with isolated STIC diagnosed between 1998 and 2020 across 15 Canadian centres. A blinded panel of 4 subspecialized gynecologic pathologists independently reviewed scanned slides (hematoxylin and eosin, p53, and Ki67) from 57 cases and met for a consensus meeting. Diagnoses were grouped as HGSC, STIC, and serous tubal intraepithelial lesion/other. The initial interobserver agreement for the diagnostic categories was moderate (Fleiss's kappa =0.495, 95% CI: 0.269-0.675; Cohen's kappa =0.492, 95% CI: 0.226-0.758, percent agreement 83.3%). A concordant diagnosis among all 4 reviewers was initially reached in 68% (39/57; 95% CI: 55.5%-79.0%), which increased to 91% (52/57; 95% CI: 81.1%-96.2%) after the consensus meeting. Consensus or majority (3 of 4 reviewers) was considered the final diagnosis; this resulted in STIC in 79% (45/57; 95% CI: 66.7-87.5), in agreement with the original diagnosis. Nine percent of cases were downgraded to serous tubal intraepithelial lesion/other (5/57; 95% CI: 1.8%-14.4%), while 12% were upgraded to HGSC (7/57; 95% CI: 6.1%-23.2%). Six patients developed HGSC clinically (11%; 95% CI: 4.9%-21.1%), of whom 3 had a review diagnosis of HGSC and 3 of STIC. This study shows suboptimal interobserver reproducibility for a diagnosis of STIC, despite consideration of ancillary p53/Ki67 testing. This study offers guidance to increase interobserver reproducibility in this challenging area, particularly in avoiding an underdiagnosis of HGSC.</p>","PeriodicalId":14001,"journal":{"name":"International Journal of Gynecological Pathology","volume":" ","pages":""},"PeriodicalIF":2.1,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149074609","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Unusual Variants of Endometrial Stromal Sarcoma. 子宫内膜间质肉瘤的异常变异。
IF 2.1 4区 医学
International Journal of Gynecological Pathology Pub Date : 2026-09-21 DOI: 10.1097/PGP.0000000000001216
Amr Abulaban, Andre Pinto
{"title":"Unusual Variants of Endometrial Stromal Sarcoma.","authors":"Amr Abulaban, Andre Pinto","doi":"10.1097/PGP.0000000000001216","DOIUrl":"https://doi.org/10.1097/PGP.0000000000001216","url":null,"abstract":"<p><p>Endometrial stromal sarcoma (ESS) is traditionally classified into low-grade (LGESS) and high-grade (HGESS) categories based on morphology, immunophenotype, and specific molecular alterations. Some tumors, however, defy these patterns, displaying unusual features that complicate diagnosis and prognostication. We performed a retrospective review of ESS diagnosed from 2022 to 2025 at a single institution, obtaining 4 cases with highly uncommon pathologic findings, including 3 with available molecular data. Key histologic, immunohistochemical, molecular, and clinical findings were recorded. These cases broaden the known spectrum of ESS, demonstrating that tumors with genetic hallmarks of LGESS can exhibit unusual patterns of differentiation, high-grade features, unconventional immunohistochemical findings, and rare molecular profiles.</p>","PeriodicalId":14001,"journal":{"name":"International Journal of Gynecological Pathology","volume":" ","pages":""},"PeriodicalIF":2.1,"publicationDate":"2026-09-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149016448","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Poor Prognosis and High Recurrence in NTRK3 Fusion Cervical Sarcoma: A Series of 3 Cases and Literature Review. NTRK3融合型宫颈肉瘤3例临床分析及文献复习
IF 2.1 4区 医学
International Journal of Gynecological Pathology Pub Date : 2026-09-16 DOI: 10.1097/PGP.0000000000001215
Wan Xiong, Junbo Hu, Zhigan Wang, Fang Yu, Qiongrong Chen
{"title":"Poor Prognosis and High Recurrence in NTRK3 Fusion Cervical Sarcoma: A Series of 3 Cases and Literature Review.","authors":"Wan Xiong, Junbo Hu, Zhigan Wang, Fang Yu, Qiongrong Chen","doi":"10.1097/PGP.0000000000001215","DOIUrl":"https://doi.org/10.1097/PGP.0000000000001215","url":null,"abstract":"<p><p>This study aimed to expand the morphologic and molecular spectrum of NTRK-rearranged uterine tumors by reporting 3 cases of NTRK3-fusion cervical sarcoma and conducting a literature review, thereby strengthening the understanding of their clinicopathologic features, differential diagnosis and treatments. We collected 3 cases of NTRK3-fusion cervical sarcoma and reviewed their clinical presentation, histomorphology, immunohistochemistry, fluorescence in situ hybridization (FISH) and next-generation sequencing (NGS) findings. Previously reported cases were identified from the English-language literature in PubMed, and the differential diagnosis of uterine spindle cell tumors was discussed. The tumors in all 3 cases were composed of uniform spindle cells with mild to moderate atypia and focal lymphocytic infiltration. Mitotic activity varied from 10 to 30 per 10 high-power fields. Immunohistochemically, all cases showed CD34 and pan-TRK positivity, and 2 cases showed patchy to diffuse S-100 staining. NGS identified NTRK3 fusions in all cases (SPECC1L::NTRK3, ETV6::NTRK3, KHDRBS1::NTRK3). All 3 patients experienced recurrence, and 2 remained stable after receiving TRK inhibitor therapy. Integration of our cases with 68 previously reported cases revealed that NTRK3-fusion uterine tumors tend to be larger, with higher mitotic counts, a higher proportion of cases with International Federation of Gynecology and Obstetrics (FIGO) stage above IB, higher recurrence rates, and increased disease-specific mortality. NTRK3-fusion cervical sarcomas represent a rare but clinically significant subset of NTRK-rearranged tumors characterized by their potential for recurrence and metastasis. The molecular identification of NTRK-rearranged neoplasms is highly significant, as patients may benefit from TRK inhibitor therapy.</p>","PeriodicalId":14001,"journal":{"name":"International Journal of Gynecological Pathology","volume":" ","pages":""},"PeriodicalIF":2.1,"publicationDate":"2026-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148982677","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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