International Journal of Surgical Pathology最新文献

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Unusual Tumor in an Unusual Localization: Sclerosing Extramedullary Hematopoietic Tumor in Breast With Literature Review. 异常定位的异常肿瘤:乳腺硬化性髓外造血肿瘤并文献复习。
IF 0.9 4区 医学
International Journal of Surgical Pathology Pub Date : 2026-10-08 DOI: 10.1177/10668969261486328
Istem Kose, Cheryl Xin, Joshua Greenwald, David Yau, Tiannan Wang
{"title":"Unusual Tumor in an Unusual Localization: Sclerosing Extramedullary Hematopoietic Tumor in Breast With Literature Review.","authors":"Istem Kose, Cheryl Xin, Joshua Greenwald, David Yau, Tiannan Wang","doi":"10.1177/10668969261486328","DOIUrl":"https://doi.org/10.1177/10668969261486328","url":null,"abstract":"<p><p>BackgroundSclerosing extramedullary hematopoietic tumor (SEMHT) is a rare entity that can occur in patients with myeloproliferative neoplasms. It has been reported in various localizations including retroperitoneum, mesentery, liver, colon, lymph nodes, thyroid, and orbit. It is considered as late manifestation of the underlying myeloid neoplasm. It is clinically important to distinguish from extramedullary hematopoiesis, as well as mesenchymal tumors.PresentationA 81-year-old female patient with a history of left breast ductal carcinoma in situ who underwent breast lumpectomy followed by radiation was diagnosed with polycythemia vera the following year. Two years later, two indistinct masses were incidentally identified in the patient's right breast. Breast biopsy showed effaced normal breast architecture, sclerotic/fibrotic background with scattered markedly atypical large cells with large, hyperchromatic nuclei. The immunohistochemical studies on the biopsy were inconclusive and diagnosis of atypical mesenchymal neoplasm with recommended resection was rendered. The resection specimen showed similar morphology. Immunohistochemistry stains were performed, CD61 and FVIII proved the large, atypical cells were atypical megakaryocytes. Glycophorin A stained the erythroid cells, and myeloperoxidase was positive in myeloid cells, identifying this as a SEMHT.Discussion<b>:</b> SEMHT is an uncommon tumor, exceptionally rare in breast. The main differential diagnoses include extramedullary hematopoiesis, metaplastic carcinoma of the breast, pseudo angiomatous stromal hyperplasia (PASH), fibroblastic/myofibroblastic tumors of the breast, mesenchymal tumors.ConclusionSEMHT is a rare entity that can present in any body sites. To recognize this entity in the breast tissue along patient's history to avoid diagnostic pitfall is essential for clinical guidance.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"10668969261486328"},"PeriodicalIF":0.9,"publicationDate":"2026-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149136570","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
Intracranial Mesenchymal Tumor With FET::CREB Fusion Involving Lateral Ventricle: A Case Report With Review of Literature. 颅内间充质肿瘤伴FET::CREB融合累及侧脑室1例并文献复习。
IF 0.9 4区 医学
International Journal of Surgical Pathology Pub Date : 2026-10-08 DOI: 10.1177/10668969261483645
Sunil Pasricha, Garima Durga, Anila Sharma, Himanshi Dewan, Ishwar C Premsagar, Rupinder Singh, Meenakshi Kamboj, Divya Bansal, Prerna Chadha, Gurudutt Gupta, Anurag Mehta
{"title":"Intracranial Mesenchymal Tumor With <i>FET::CREB</i> Fusion Involving Lateral Ventricle: A Case Report With Review of Literature.","authors":"Sunil Pasricha, Garima Durga, Anila Sharma, Himanshi Dewan, Ishwar C Premsagar, Rupinder Singh, Meenakshi Kamboj, Divya Bansal, Prerna Chadha, Gurudutt Gupta, Anurag Mehta","doi":"10.1177/10668969261483645","DOIUrl":"https://doi.org/10.1177/10668969261483645","url":null,"abstract":"<p><p>Intracranial mesenchymal tumor, <i>FET::CREB</i> fusion-positive is a provisional tumor type in the 2021 World Health Organization (WHO) classification of central nervous system tumors, typically seen in children and young adults. Herein, we describe a patient with intracranial mesenchymal tumor, <i>FET::CREB</i> fusion-positive arising at a rare site, that is, lateral ventricle in a young girl with complaints of headache and diplopia. The mass was excised and sent for histopathological examination wherein, a possibility of intracranial myxoid mesenchymal tumor with <i>FET::CREB</i> fusion was suggested. Subsequently, the diagnosis was confirmed on demonstration of <i>EWSR1</i> gene rearrangement by break apart fluorescence in situ hybridization (FISH). Owing to its rarity, there are very few published reports in the literature and hence their clinical course is not well known. On review of the world literature, it is documented to be a locally aggressive tumor with a high recurrence rate. It is imperative that pathologists are aware of the full clinicopathologic spectrum of this rare entity, as the differential diagnosis can be challenging.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"10668969261483645"},"PeriodicalIF":0.9,"publicationDate":"2026-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149136552","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
A Pathogenic BCOR Mutation Favors the Diagnosis of Borderline Phyllodes Tumor in a Fibroepithelial Lesion With Inconclusive Morphology and Early Recurrence. 一种致病性BCOR突变有利于在形态不确定和早期复发的纤维上皮病变中诊断交界性叶状瘤。
IF 0.9 4区 医学
International Journal of Surgical Pathology Pub Date : 2026-10-07 DOI: 10.1177/10668969261489624
Pablo García Fernández, Mirela M Roman, Fabrizio De Vietro, Alicia Gonzalez Antelo, Christine Galant, Mieke R Van Bockstal
{"title":"A Pathogenic <i>BCOR</i> Mutation Favors the Diagnosis of Borderline Phyllodes Tumor in a Fibroepithelial Lesion With Inconclusive Morphology and Early Recurrence.","authors":"Pablo García Fernández, Mirela M Roman, Fabrizio De Vietro, Alicia Gonzalez Antelo, Christine Galant, Mieke R Van Bockstal","doi":"10.1177/10668969261489624","DOIUrl":"https://doi.org/10.1177/10668969261489624","url":null,"abstract":"<p><p>Phyllodes tumors (PTs) are rare fibroepithelial neoplasms of the breast. PTs may pose significant diagnostic challenges, especially when they lack the classic leaf-like architecture and resemble cellular fibroadenomas. Accurate distinction between these entities is clinically important because of differences in management and recurrence risk. We report a 21-year-old woman who developed an early local recurrence of a fibroepithelial lesion initially diagnosed as a cellular complex fibroadenoma. The recurrence demonstrated stromal hypercellularity, multifocal infiltrative growth, and non-atypical smooth muscle differentiation without characteristic leaflike architecture, rendering morphological classification inconclusive. Next-generation sequencing demonstrated a pathogenic frameshift mutation in the <i>BCOR</i> gene. When integrated with the patient's clinical history and the lesion's morphology, these molecular findings supported a diagnosis of borderline PT. This report emphasizes the importance of integrating histopathological, clinical, and molecular data in the evaluation of fibroepithelial lesions with ambiguous morphology. Molecular alterations, such as <i>BCOR</i> mutations, may provide valuable diagnostic support, in particular in fibroadenoma-like PTs.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"10668969261489624"},"PeriodicalIF":0.9,"publicationDate":"2026-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149127468","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
IDH-Mutant Astrocytomas in Patients Older than 55 Years: A Retrospective Analysis. 55岁以上患者的idh突变星形细胞瘤:回顾性分析
IF 0.9 4区 医学
International Journal of Surgical Pathology Pub Date : 2026-10-07 DOI: 10.1177/10668969261489616
Rasha Alfattal, Keng Lam, Hanim I Ozkizilkaya, Chirag B Patel, Daniel H Wang, Jason T Huse, Leomar Y Ballester, Maria A Gubbiotti
{"title":"IDH-Mutant Astrocytomas in Patients Older than 55 Years: A Retrospective Analysis.","authors":"Rasha Alfattal, Keng Lam, Hanim I Ozkizilkaya, Chirag B Patel, Daniel H Wang, Jason T Huse, Leomar Y Ballester, Maria A Gubbiotti","doi":"10.1177/10668969261489616","DOIUrl":"https://doi.org/10.1177/10668969261489616","url":null,"abstract":"<p><p>Diffuse gliomas are the most common malignant brain tumors in adults, stratified by isocitrate dehydrogenase (<i>IDH1/IDH2</i>) mutation status. While the median age for central nervous system (CNS) World Health Organization (WHO) grade 2 to 3 IDH-mutant astrocytomas is 38 years, older patients of >55 years predominantly exhibit IDH-wildtype gliomas and confer a poorer prognosis. To examine the clinicopathologic spectrum of IDH-mutant astrocytomas in older adults, we retrospectively reviewed all astrocytic tumors diagnosed at our institution between 2021 and 2024 in patients >55 years. Twenty-two IDH-mutant astrocytomas (median age: 63 years; range: 56-79; male:female = 9:13) were identified and compared descriptively to a historical cohort of 179 IDH-mutant astrocytomas in younger patients. Grade distribution among older patients paralleled that of younger adults: CNS WHO grade 2 (55%), grade 3 (18%), and grade 4 (27%). Magnetic resonance imaging (MRI) showed T2/FLAIR mismatch sign in 25% of grade 2 tumors and in none of the higher grades; 50% of grade 2 tumors demonstrated contrast enhancement. IDH1 p.R132H accounted for 95% of mutations, with 1 p.R132G variant. ATRX loss was seen in 71% and strong p53 immunoreactivity in 83% of evaluable tumors. Copy-number analysis (<i>n</i> = 10) revealed <i>CDKN2A/B</i> homozygous deletion in 10% and heterozygous loss in 20%<i>.</i> Despite their rarity, IDH-mutant astrocytomas in older adults are more prevalent than historically appreciated and maintain imaging and molecular profiles akin to tumors in younger adults. Accordingly, IDH1 p.R132H-specific immunohistochemistry remains a cost-effective first-line screening tool irrespective of age.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"10668969261489616"},"PeriodicalIF":0.9,"publicationDate":"2026-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149127489","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
Aberrant Desmin Expression in a Middle Ear Neuroendocrine Tumor: Expanding the Immunophenotypic Spectrum. 特异蛋白在中耳神经内分泌肿瘤中的异常表达:扩大免疫表型谱。
IF 0.9 4区 医学
International Journal of Surgical Pathology Pub Date : 2026-10-07 DOI: 10.1177/10668969261486382
Diya Roy, Suvradeep Mitra, Amanjit Bal, Ramandeep Virk
{"title":"Aberrant Desmin Expression in a Middle Ear Neuroendocrine Tumor: Expanding the Immunophenotypic Spectrum.","authors":"Diya Roy, Suvradeep Mitra, Amanjit Bal, Ramandeep Virk","doi":"10.1177/10668969261486382","DOIUrl":"https://doi.org/10.1177/10668969261486382","url":null,"abstract":"<p><p>Middle ear neuroendocrine tumors (NETs) are uncommon neoplasms that exhibit both epithelial and neuroendocrine differentiation. Aberrant desmin expression in middle ear NET has not been documented previously and may create a diagnostic pitfall due to morphologic overlap with myogenic and rhabdoid tumors. A 52-year-old man presented with right ear pain, fullness, and facial deviation. Imaging revealed a soft-tissue mass involving the middle ear cavity and the mastoid air cells, with ossicular erosion. Histopathology showed an infiltrative neoplasm arranged in nests, trabeculae, cords, and pseudoacini composed of monomorphic cells with plasmacytoid/rhabdoid morphology. Immunohistochemically, the tumor cells showed diffuse positivity for KRT, synaptophysin, chromogranin, and diffuse strong desmin expression, while myogenin, MYOD1, p63, calponin, TTF1, CDX2, and GATA3 were negative. SMARCB1/INI1 expression was retained, and the Ki67 index was <2%. A diagnosis of middle ear NET with aberrant desmin positivity was rendered. The patient remained disease-free 18 months following surgical excision. The index patient expands the immunophenotypic spectrum of middle ear NET by documenting diffuse aberrant desmin expression. Awareness of this unusual finding is important to avoid misdiagnosis as rhabdomyosarcoma or other rhabdoid neoplasms. Accurate diagnosis requires careful integration of morphologic and immunohistochemical features, and long-term follow-up remains essential despite the generally indolent behavior of middle ear NETs.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"10668969261486382"},"PeriodicalIF":0.9,"publicationDate":"2026-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149127481","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
Extrapulmonary Retroperitoneal Lymphangioleiomyomatosis Associated With Endosalpingiosis: A Case Report. 肺外腹膜后淋巴管油松肌瘤病合并输卵管内肿大1例报告。
IF 0.9 4区 医学
International Journal of Surgical Pathology Pub Date : 2026-10-07 DOI: 10.1177/10668969261489623
Ahmed R Alshamasi, Tafeeq Tawfeeq, Khalid Balawi, Khaleel I Al-Obaidy, Zainab I Alruwaii
{"title":"Extrapulmonary Retroperitoneal Lymphangioleiomyomatosis Associated With Endosalpingiosis: A Case Report.","authors":"Ahmed R Alshamasi, Tafeeq Tawfeeq, Khalid Balawi, Khaleel I Al-Obaidy, Zainab I Alruwaii","doi":"10.1177/10668969261489623","DOIUrl":"https://doi.org/10.1177/10668969261489623","url":null,"abstract":"<p><p>BackgroundLymphangioleiomyomatosis (LAM) is an uncommon multisystem disorder characterized by proliferation of perivascular epithelioid cells and classified within the perivascular epithelioid cell tumor (PEComa) family. Although classically involving the lung, extrapulmonary manifestations have been documented, most frequently within the retroperitoneum and abdominopelvic lymphatics. Endosalpingiosis is a benign Müllerian lesion defined by the presence of ectopic glands lined by fallopian tube-type ciliated epithelium in the absence of accompanying endometrial-type stroma. The coexistence of extrapulmonary LAM and endosalpingiosis within the same lesion is exceedingly rare, with only a limited number of examples reported in the literature.Patient presentationA 40-year-old woman presented with abdominal distension and discomfort. Imaging demonstrated a 16-cm multilocular cyst in the right retroperitoneal space. Surgical excision was performed, and histological examination revealed a cyst with a proliferation of spindle to epithelioid smooth muscle-like cells arranged around lymphatic channels and admixed with lymphoid aggregates, consistent with LAM. The cyst wall was focally lined by ciliated columnar epithelium morphologically identical to that of fallopian tube, without associated endometrial-type stroma, in keeping with endosalpingiosis. The LAM cells exhibited positivity for PMEL (HMB-45) and desmin, whereas the endosalpingiosis component demonstrated positivity for pan-keratin and PAX8 immunostains.ConclusionExtrapulmonary LAM may rarely present as a cystic retroperitoneal lesion and coexist with endosalpingiosis, posing a diagnostic challenge. Recognition of this pattern is important to ensure correct classification and appropriate evaluation for systemic involvement.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"10668969261489623"},"PeriodicalIF":0.9,"publicationDate":"2026-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149127535","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
Diagnostic Value of Immunohistochemical Markers GATA3, Cyclin D1, KRT20, and GLUT1 in Renal Neoplasms. 免疫组化标志物GATA3、Cyclin D1、KRT20和GLUT1在肾肿瘤中的诊断价值。
IF 0.9 4区 医学
International Journal of Surgical Pathology Pub Date : 2026-10-01 Epub Date: 2026-06-25 DOI: 10.1177/10668969261442121
Muhammad Abrar Barakzai, Shoukat Ali Arain, Nasir Ali Afsar, Yara Obaeda Alsouss, Turki Al-Hussain
{"title":"Diagnostic Value of Immunohistochemical Markers GATA3, Cyclin D1, KRT20, and GLUT1 in Renal Neoplasms.","authors":"Muhammad Abrar Barakzai, Shoukat Ali Arain, Nasir Ali Afsar, Yara Obaeda Alsouss, Turki Al-Hussain","doi":"10.1177/10668969261442121","DOIUrl":"10.1177/10668969261442121","url":null,"abstract":"<p><p>BackgroundThis study assessed the potential utility of GATA3, cyclin D1, KRT20, and GLUT1 immunohistochemistry (IHC) markers for diagnosing renal epithelial tumors.MethodsA retrospective analysis was conducted on all eligible specimens (<i>n</i> = 95) diagnosed at our institution between 2014 and 2024. IHC was done and the staining intensity and distribution of selected IHC markers were assessed.ResultsAmong 95 specimens, 26% were clear cell renal cell carcinoma, 21% were chromophobe RCC, and 17% were papillary RCC. Less frequent subtypes included TFE3-rearranged RCC, clear cell papillary renal cell tumor (CCPRT), papillary renal neoplasm with reverse polarity (PRNRP), and eosinophilic solid and cystic RCC (ESC-RCC). Clear cell RCC and CCPRT were consistently positive for cyclin D1 and GLUT1, and negative for GATA3/KRT20. CCPRT showed strong, diffuse GLUT1, unlike the limited staining in papillary RCC. PRNRP was positive for GATA3, cyclin D1, and GLUT1, and negative for KRT20. While chromophobe RCC exhibited variable GATA3 and GLUT1 expression with weak to moderate cyclin D1 positivity, oncocytomas were strong cyclin D1 positive, with negative GATA3, KRT20, and GLUT1. All 3 ESC-RCC specimens were KRT20 positive.ConclusionsOur findings validate many previously reported observations and characterize adjunctive patterns of these less commonly used markers across renal tumor subtypes. It suggests GATA3 as a potential diagnostic tool for PRNRP, while GLUT1 demonstrates differential expression patterns for papillary RCC and CCPRT, aiding in distinguishing papillary renal tumors. Similarly, cyclin D1 appears to be a valuable adjunct in differentiating oncocytoma from chromophobe RCC. Also, KRT20 shows high specificity for ESC-RCC.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1759-1766"},"PeriodicalIF":0.9,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148316920","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
Metaplastic Breast Carcinoma Hidden Behind a Melanocytic Facade: A Case Report with Literature Review on Metaplastic Breast Carcinoma with Melanocytic Differentiation. 隐藏在黑素细胞外表面的化生性乳腺癌:伴黑素细胞分化的化生性乳腺癌1例报告并文献复习。
IF 0.9 4区 医学
International Journal of Surgical Pathology Pub Date : 2026-10-01 Epub Date: 2026-05-14 DOI: 10.1177/10668969261440473
Vandna Bharati, Ridhi Sood, Prachi Bansal, Ashutosh Mishra, Ruchi Rathore, Sandeep Mathur
{"title":"Metaplastic Breast Carcinoma Hidden Behind a Melanocytic Facade: A Case Report with Literature Review on Metaplastic Breast Carcinoma with Melanocytic Differentiation.","authors":"Vandna Bharati, Ridhi Sood, Prachi Bansal, Ashutosh Mishra, Ruchi Rathore, Sandeep Mathur","doi":"10.1177/10668969261440473","DOIUrl":"10.1177/10668969261440473","url":null,"abstract":"<p><p>Melanocytic differentiation in breast carcinomas is extremely rare. It often leads to diagnostic dilemmas and should be distinguished from melanoma and pigmented breast lesions, which have significantly different treatment implications and prognoses. The pathogenesis of these tumors is unclear with limited follow-up data. We present one such tumor arising in a female patient in her late 20s, where a final diagnosis of mixed metaplastic carcinoma with melanocytic differentiation and invasive breast carcinoma of no special type was made based on morphological and immunohistochemical evidence. This report discusses the differential diagnosis, insights into the pathogenesis and nomenclature of these tumors, along with a literature review of prior case reports.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1881-1888"},"PeriodicalIF":0.9,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147929619","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 Renal Sclerosing Epithelioid Fibrosarcoma With EWSR1::CREB3L1 Fusion: A Diagnostic Pitfall With a Comprehensive Review of Reported Patients. 原发性肾硬化上皮样纤维肉瘤合并EWSR1::CREB3L1融合:一个诊断缺陷与已报道患者的综合回顾
IF 0.9 4区 医学
International Journal of Surgical Pathology Pub Date : 2026-10-01 Epub Date: 2026-05-08 DOI: 10.1177/10668969261444687
Ayoub Nahal, Jessica Jahoda, Waleed Hassen, Mohamed Aziz
{"title":"Primary Renal Sclerosing Epithelioid Fibrosarcoma With <i>EWSR1::CREB3L1</i> Fusion: A Diagnostic Pitfall With a Comprehensive Review of Reported Patients.","authors":"Ayoub Nahal, Jessica Jahoda, Waleed Hassen, Mohamed Aziz","doi":"10.1177/10668969261444687","DOIUrl":"10.1177/10668969261444687","url":null,"abstract":"<p><p>Sclerosing epithelioid fibrosarcoma (SEF) is a rare malignant soft tissue sarcoma with morphologic and molecular overlap with low-grade fibromyxoid sarcoma (LGFMS). Although most commonly arising in deep soft tissues, primary involvement of the kidney is exceptionally uncommon and represents a significant diagnostic pitfall. We report a primary renal SEF in a 22-year-old woman, characterized by classic histomorphology, diffuse MUC4 immunoreactivity, and definitive molecular confirmation by next-generation sequencing demonstrating an <i>EWSR1::CREB3L1</i> fusion. Radiologic evaluation revealed a solid renal mass suspicious for malignancy, prompting partial nephrectomy. Histologically, the tumor was composed of epithelioid and spindle cells embedded in a densely sclerotic stroma with characteristic filigree collagen. Accurate recognition of renal SEF is critical, as it may be misdiagnosed as sarcomatoid renal cell carcinoma, sclerosing clear cell sarcoma of the kidney, epithelioid angiomyolipoma, or other primary renal neoplasms. In addition, a comprehensive review of the literature was performed. Including recently reported tumors identified within molecularly characterized series, a total of 19 patients with primary renal SEF have been documented to date. Notably, within the genitourinary tract, available data suggest a relative predilection for renal involvement. This tumor confirms the broad anatomic spectrum of SEF and underscores the essential role of MUC4 immunohistochemistry and molecular testing in the evaluation of renal tumors with epithelioid cytology and prominent stromal sclerosis.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1925-1932"},"PeriodicalIF":0.9,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147837452","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
Distinguishing Inverted Sinonasal Papilloma and Respiratory Epithelial Adenomatoid Hamartoma: A Single Institution Case Series. 鉴别鼻窦内翻性乳头状瘤和呼吸道上皮性腺瘤样错构瘤:单一机构病例系列。
IF 0.9 4区 医学
International Journal of Surgical Pathology Pub Date : 2026-10-01 Epub Date: 2026-05-14 DOI: 10.1177/10668969261446657
Austin R Swisher, Samuel E Razmi, Michael T Sramek, Annica C Eells, Pedro L Gomes, Alessandra Schmitt, Amar Miglani, Devyani Lal, Michael J Marino
{"title":"Distinguishing Inverted Sinonasal Papilloma and Respiratory Epithelial Adenomatoid Hamartoma: A Single Institution Case Series.","authors":"Austin R Swisher, Samuel E Razmi, Michael T Sramek, Annica C Eells, Pedro L Gomes, Alessandra Schmitt, Amar Miglani, Devyani Lal, Michael J Marino","doi":"10.1177/10668969261446657","DOIUrl":"10.1177/10668969261446657","url":null,"abstract":"<p><p>Inverted sinonasal papilloma (ISP) and respiratory epithelial adenomatoid hamartoma (REAH) are sinonasal lesions with overlapping clinical, radiographic, and histologic features, making accurate diagnosis challenging. ISP is a benign but aggressive tumor with a propensity for local destruction, recurrence, and malignant transformation, whereas REAH is a hamartomatous lesion often arising in the olfactory cleft. We present four patients with lesions initially suspected to be ISP based on intraoperative frozen section but ultimately diagnosed as REAH on permanent pathology. All patients had polypoid sinonasal masses, frequently involving or adjacent to the olfactory cleft. Intraoperative frozen sections were often inconclusive or favored ISP, reflecting the difficulty of distinguishing these entities. Histologic analysis revealed that epithelial thickness served as a key morphologic feature differentiating ISP from REAH, with REAH exhibiting ciliated glandular proliferation and ISP characterized by hyperplastic epithelium with features of columnar and squamous differentiation. These patients underscore the diagnostic pitfalls of small biopsies and frozen sections, and highlight the importance of permanent specimens and collaboration with experienced pathologists. Accurate differentiation is critical, as management strategies differ substantially: ISP requires aggressive resection and long-term surveillance, while REAH may be treated with limited excision and minimal follow-up.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1775-1781"},"PeriodicalIF":0.9,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147941379","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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