{"title":"Ovarian Mesonephric-Like Adenocarcinoma Arising from an Endometriotic Cyst with an Identical <i>KRAS</i> Mutation in Both Components: A Case Report.","authors":"Chika Eto, Tsubasa Hiraki, Takuya Kawata, Nobutaka Takahashi, Yasuyuki Hirashima","doi":"10.1177/10668969251333437","DOIUrl":"10.1177/10668969251333437","url":null,"abstract":"<p><p>Mesonephric-like adenocarcinoma (MLA) is a rare tumor that occurs in the uterine corpus and ovary. It shares the morphological and immunohistochemical features of cervical mesonephric adenocarcinoma. Recent reports have suggested that a majority of ovarian MLAs are associated with endometriosis. Herein, we report the novel tumor of a 54-year-old Japanese woman with a history of bilateral ovarian endometriotic cysts who presented with abdominal distension. Pelvic magnetic resonance imaging revealed a 14-cm left ovarian cystic tumor with an intra-cystic solid mass. Histological examination revealed proliferation of columnar or cuboidal tumor cells forming round tubules, irregular tubular glands, fused glands, papillary structures, and solid nests.Additionally, associated endometriosis was observed in the cyst wall. The tumor cells were diffusely positive for GATA-binding protein 3 (GATA3) and negative for estrogen receptor. The tumor cells also showed wild-type p53, with retained expression of AT-rich interactive domain-containing protein 1A (ARID1) and phosphatase and tensin homolog (PTEN). Molecular analysis revealed an identical <i>KRAS</i> p.G12V mutation in the tumor and the endometriotic cyst. The present tumor elucidates the pathogenesis and molecular mechanisms of ovarian MLA and demonstrates that ovarian MLA is an endometriosis-associated neoplasm. Further investigation into the pathogenesis and molecular mechanisms of ovarian MLA may assist in identifying potential therapeutic targets for this rare condition.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1778-1783"},"PeriodicalIF":1.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143989640","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}
{"title":"Calcifying Nested Stromal-Epithelial Tumor (CNSET) of the Liver: A Case Report With Its Molecular Profile.","authors":"Hazal İzol Özmen, Nevra Dursun, Sibel Erdamar, Kamuran Tutuş, Nazli Gülsüm Akyel, İlgin Özden, Banu Yilmaz Özgüven","doi":"10.1177/10668969251338570","DOIUrl":"10.1177/10668969251338570","url":null,"abstract":"<p><p>Calcifying Nested Stromal-Epithelial Tumor (CNSET) of the liver is a hepatic neoplasm of uncertain lineage, primarily affecting young individuals. This case report aims to elucidate the clinical, histopathological, and molecular characteristics of CNSET, contributing to the sparse knowledge regarding this unusual entity. We present here a 17-year-old female patient diagnosed with CNSET, highlighting the tumor's radiologic appearance, surgical management, and postoperative findings, alongside a comprehensive review of existing literature on the subject.The patient was evaluated for obesity and discovered to have a liver mass through imaging studies. The tumor demonstrated distinctive MRI features and was surgically resected, with histopathology confirming the diagnosis of CNSET. Molecular analysis revealed <i>CTNNB1</i> and <i>TERT</i> promoter mutations.This case report underscores the importance of considering CNSET in differential diagnoses of liver tumors in young patients and adds valuable insights into its molecular underpinnings, emphasizing the need for further research to understand its pathogenesis and optimize management strategies.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1795-1800"},"PeriodicalIF":1.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143996451","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}
{"title":"Hepatic Undifferentiated Carcinoma With Osteoclast Like Giant Cell With Dominant Intraductal Growth: A Case Report With Literature Review.","authors":"Yu-Chien Ko, Tse-Ching Chen","doi":"10.1177/10668969251334752","DOIUrl":"10.1177/10668969251334752","url":null,"abstract":"<p><p>Undifferentiated carcinoma with osteoclast-like giant cells is extremely rare in the liver. Here, we report a tumor with dominant intraductal growth. The unique growth pattern has never been reported before. A 73-year-old female patient presented intermittent right upper quadrant abdominal pain accompanied by fever and general weakness for 1 month. Imaging study revealed an intraductal lesion in the dilated large bile duct. Histologically, the tumor showed predominantly undifferentiated carcinoma with osteoclast-like giant cells, associated with high-grade biliary intraepithelial neoplasia and minor foci of adenocarcinoma. A <i>KRAS</i> codon 61 mutation was detected, supporting the epithelial origin of undifferentiated carcinoma with osteoclast-like giant cells. This tumor not only adds to the limited documented instances of undifferentiated carcinoma with osteoclast-like giant cells in the liver but also demonstrates that the development of an undifferentiated component might occur in a low stage tumor, which is typically considered a late stage in tumor progression.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1784-1790"},"PeriodicalIF":1.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143989864","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}
Benjamin L Petrykowski, Conner Thompson, Tiffany G Baker, Cynthia T Welsh
{"title":"Clival Chordoma Metastatic to Lymph Node: Brachyury Staining Insights.","authors":"Benjamin L Petrykowski, Conner Thompson, Tiffany G Baker, Cynthia T Welsh","doi":"10.1177/10668969251334728","DOIUrl":"10.1177/10668969251334728","url":null,"abstract":"<p><p>Clival chordomas are rare bone tumors involving the primary intracranial central nervous system. These tumors reoccur and invade locally but metastasis is uncommon. We present a 54-year-old woman with a 10-year recurrence of a conventional chordoma originating from the clivus with lymph node metastasis that was confirmed with brachyury. Based on our review of the literature, there have been only 3 confirmed patients with clival chordoma metastasis to lymph nodes and this is the first account of a conventional chordoma lymph node metastasis being confirmed with brachyury immunohistochemistry.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1791-1794"},"PeriodicalIF":1.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143980587","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}
{"title":"Morphologically In Situ Solid Papillary Carcinoma of the Breast With Lymph Node and Lung Metastasis: A Rare Case Report and Literature Review.","authors":"Sayed Matar, Seyed Reza Taha, Fouad Boulos","doi":"10.1177/10668969251339807","DOIUrl":"10.1177/10668969251339807","url":null,"abstract":"<p><p>Solid papillary carcinoma (SPC) is rare, accounting for less than 1% of all breast cancers. According to the 5th edition of the WHO Classification of Breast Tumors (2019), SPC is divided into invasive (ISPC) and in situ (SPC in situ) subtypes. ISPC is characterized by an irregular or jigsaw pattern lacking myoepithelial cells, while SPC in situ is well-circumscribed, regardless of myoepithelial cell presence. Metastasis is infrequently reported, with most patients involving lymph nodes and arising from ISPC. Metastases from SPC in situ without myoepithelial cells have rarely been reported. We present a 66-year-old woman with a 28 × 13 mm hypoechoic breast mass detected on ultrasound. Microscopic examination revealed well-circumscribed nests of SPC, positive for ER, PR, and synaptophysin, with a Ki-67 proliferation index of 10%. HER2 was negative. Myoepithelial markers, including p63, smooth muscle actin, keratin 5/6, p40, and CD10, were all negative, confirming the absence of a myoepithelial layer. Axillary lymph node dissection revealed metastasis in three of eighteen lymph nodes, with the metastatic foci demonstrating the same well-circumscribed SPC architecture as the primary tumor in the breast. Additionally, a lung biopsy exhibited metastatic SPC with identical histomorphology and immunohistochemical profile to the primary tumor. Although rare, SPC presenting a well-circumscribed morphology, and classified as in situ according to the WHO, should be approached with caution when myoepithelial cells are absent, as the in situ histomorphology does not necessarily exclude the potential for metastasis.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1801-1806"},"PeriodicalIF":1.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144078075","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}
Shirali Gokharu, Obaidur Rehman, Arpan Gandhi, Sima Das
{"title":"Rare Palpebral Conjunctival Myxoma With Histopathological and Immunohistochemical Evidence.","authors":"Shirali Gokharu, Obaidur Rehman, Arpan Gandhi, Sima Das","doi":"10.1177/10668969251343831","DOIUrl":"10.1177/10668969251343831","url":null,"abstract":"<p><p>Conjunctival myxoma is a rare benign tumor which may mimic other common lesions of the conjunctiva. We describe a lesion in a 52-year-old man, initially diagnosed as a conjunctival granuloma, arising from the palpebral conjunctiva. Intraoperatively, anterior orbital extension was noted via a connecting stalk. The lesion was excised completely under local anesthesia. The histopathological analysis and immunohistochemistry confirmed a diagnosis of conjunctival myxoma. Systemic evaluation was normal. At 3-month follow up, the patient remained healthy without any recurrence. Due to life-threatening syndromic associations, histopathological confirmation and systemic evaluation are mandatory when dealing with potential examples of conjunctival myxoma.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1852-1855"},"PeriodicalIF":1.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144199115","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}
Martina E Hale, Precious C Oyem, Zachary D Burke, Nathan W Mesko, Scott E Kilpatrick, Lukas M Nystrom
{"title":"The Impact of Margin Status on the Oncologic Outcomes of Surgically Treated Dedifferentiated Liposarcoma of the Extremities.","authors":"Martina E Hale, Precious C Oyem, Zachary D Burke, Nathan W Mesko, Scott E Kilpatrick, Lukas M Nystrom","doi":"10.1177/10668969251343110","DOIUrl":"10.1177/10668969251343110","url":null,"abstract":"<p><p><b><i>Background</i>.</b> Dedifferentiated liposarcoma (DDLPS) is an aggressive subtype of liposarcoma that can arise within an atypical lipomatous tumor/well-differentiated liposarcoma (ALT/WDLs). The primary goal of treatment for DDLPS is wide-margin resection, but it remains unclear if ALT/WDLs surrounding DDLPS also necessitates wide-margin resection for optimal local control. <b><i>Methods</i>.</b> Patients treated surgically for a biopsy showing DDLPS at a single institution were identified. Margin status for both DDLPS and ALT/WDLs were categorized into one of the following categories: wide, marginal or intralesional. All patients were categorized as either \"wide throughout\" or \"wide on DDLPS, marginal on ALT/WDLs\". Chi-square tests were used to compare margin status and oncologic outcome. <b><i>Results</i>.</b> Eighteen patients met the inclusion criteria for the study. Twelve patients had surgical margins wide on DDLPS/marginal on ALT/WDLs, and 6 patients had surgical margins wide throughout. Two patients with surgical margins wide on DDLPS/marginal on ALT/WDLs developed local recurrence compared with no patients with margins wide throughout (<i>P</i> = .289). Six patients with surgical margins wide on DDLPS/marginal on ALT/WDLs developed metastases compared to 1 patient with margins wide throughout (<i>P</i> = .289). <b><i>Conclusions</i>.</b> Compared with patients with wide margins throughout, patients with margins wide on DDLPS/marginal on ALT/WDLs had more local recurrences, new metastases, and death from disease. While no statistically significant difference was found between subgroups for these comparisons, cohort size limits our ability to conclude that no clinical difference exists. This study forms the basis for a future, larger, multi-institutional study to improve external validity and power to identify clinically relevant differences.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1767-1773"},"PeriodicalIF":1.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144225468","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}
Nisha Modi, Amit Varma, Rajesh Patidar, Vrushali Gadre, Srushti Karmarkar
{"title":"Differential Expression of TLE1 in Small Round Cell Tumors: A Proposed Solution for Challenging Differentials Impacting Treatment Strategies.","authors":"Nisha Modi, Amit Varma, Rajesh Patidar, Vrushali Gadre, Srushti Karmarkar","doi":"10.1177/10668969251339810","DOIUrl":"10.1177/10668969251339810","url":null,"abstract":"<p><p><i>Introduction.</i> Small round cell tumors (SRCTs) represent a heterogeneous group of neoplasms with overlapping histological features but varying origins, prognoses, and treatments. TLE1 is a well-established marker for synovial sarcoma (SS). However, TLE1's diagnostic utility is limited by its expression in a broad range of tumor types, reducing its specificity for SS. This study explores TLE1 expression across SRCTs and proposes an immunohistochemical algorithm to enhance diagnostic accuracy. <i>Methods.</i> This retrospective, single-center observational study, conducted from 2019 to 2024, reviewed pathology records for SRCTs. TLE1 staining was evaluated using the immunoreactive score system, categorized as follows: 0 (negative), 1+ (weak), 2+ (moderate), and 3+ (strong). <i>Results.</i> A total of 301 SRCTs were evaluated with diverse distribution: neuroendocrine neoplasms (21%, n = 63), extraskeletal Ewing sarcoma (15%, n = 45), lymphoblastic lymphoma (11%, n = 34), and poorly differentiated synovial sarcoma (PDSS) (8%, n = 24). TLE1 expression of 3 + was most frequently observed in PDSS (75%, 18/24). Other tumors with TLE1 3 + included extraskeletal Ewing sarcoma, extracutaneous malignant melanoma, neuroendocrine neoplasms, rhabdomyosarcoma, and endometrial stromal sarcoma. TLE1 2 + was noted in lymphoblastic lymphoma and desmoplastic SRCT, while TLE1 1 + was seen in some gastrointestinal stromal tumors. The remaining specimens were negative for TLE1. These findings led to a diagnostic framework for SRCTs based on TLE1 expression patterns. <i>Conclusions.</i> Although TLE1 is a key marker for SS, its expression in other tumors can present diagnostic challenges. Integrating clinical features, histological assessment, and a panel of immunohistochemistry markers is essential for accurate diagnosis and effective management of SRCTs.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1742-1751"},"PeriodicalIF":1.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144012481","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}
{"title":"Simple Nephrectomy for Non-Functioning Kidney: An Institutional Experience with Non-Neoplastic and Incidentally Detected Neoplastic Lesions.","authors":"Maitrayee Roy, Akshay Bali, Ayushi Kediya, Vishesh Dhawan, Vijay Shrawan Nijhawan","doi":"10.1177/10668969251339803","DOIUrl":"10.1177/10668969251339803","url":null,"abstract":"<p><p><i>Introduction.</i> Chronic kidney diseases are attributed to a variety of etiologies, including diabetes mellitus, hypertension, vascular diseases, cystic kidney diseases, chronic tubulo-interstitial diseases, chronic pyelonephritis, and glomerular diseases. Simple nephrectomy is indicated when the loss of renal function in a unilateral non-functional kidney is permanent in a persistently symptomatic patient. Uncommonly, a non-functional kidney may develop neoplasm unsuspected pre-operatively and be diagnosed only during histopathological examination of nephrectomy specimen. <i>Material and methods.</i> We examined 275 simple nephrectomies performed between 2019 and 2024 in patients with non-functional kidneys confirmed by DMSA scan. All nephrectomies done for diagnosed or even suspected tumors by pre-operative imaging were excluded from the study. <i>Results.</i> Chronic pyelonephritis (79.3%) was the most common histopathological diagnosis, followed by xanthogranulomatous pyelonephritis (8.7%) and granulomatous pyelonephritis (5.4%). In five (1.8%) patients, microscopy revealed non-specific chronic interstitial inflammation and interstitial fibrosis. One patient (0.4%) was diagnosed with large solitary pyelogenic cyst and renal nocardiosis. Eleven patients (4%) were diagnosed with neoplasms. Clear cell renal cell carcinoma was the most common diagnosis (27.2%), followed by urothelial carcinomas of renal pelvis (18.2%), one patient each of collecting duct carcinoma, squamous cell carcinoma of renal pelvis, and multilocular cystic renal neoplasm of low malignant potential. Three patients had papillary adenomas. <i>Conclusion.</i> Diligent histopathological examination of simple nephrectomy is crucial in establishing the final diagnosis with important further therapeutic implications. This is especially important in neoplastic pathologies developing in non-functional kidneys that may rarely be unsuspected by pre-operative imaging and diagnosed only during histopathological evaluation.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1759-1766"},"PeriodicalIF":1.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144142121","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}