International Journal of Surgical Pathology最新文献

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Hemangioma-Like Clear Cell Renal Cell Carcinoma Exhibiting Aggressive Behavior and High Stage: A Case Report. 血管瘤样透明细胞肾细胞癌表现为侵袭性晚期1例报告。
IF 0.9 4区 医学
International Journal of Surgical Pathology Pub Date : 2025-09-01 Epub Date: 2025-02-04 DOI: 10.1177/10668969241311495
Jiří Lenz, Květoslava Michalová, Luděk Fiala, Michal Tichý, František Tichý, Miroslav Kavka
{"title":"Hemangioma-Like Clear Cell Renal Cell Carcinoma Exhibiting Aggressive Behavior and High Stage: A Case Report.","authors":"Jiří Lenz, Květoslava Michalová, Luděk Fiala, Michal Tichý, František Tichý, Miroslav Kavka","doi":"10.1177/10668969241311495","DOIUrl":"10.1177/10668969241311495","url":null,"abstract":"<p><p>Clear cell renal cell carcinoma (CCRCC) displays a variety of architectural patterns, which are often intermingled. However, a predominant or purely multicystic growth with diffuse intracystic hemorrhage leading to hemangioma-like histomorphology, is extremely rare in CCRCC. In this article, we describe a CCRCC exhibiting a pure hemangioma-like architecture and aggressive behavior. The patient was a 73-year-old man with a tumor of the left kidney measuring 70 mm in the largest dimension. Histological examination of the nephrectomy specimen revealed a tumor composed entirely of blood filled spaces lined by a single layer of flattened or low cuboidal cells lacking high-grade features or voluminous clear cytoplasm. These cells showed diffuse immunohistochemical positivity for keratin AE1/AE3 and carbonic anhydrase 9 and focal positivity for PAX8, CD10, and alpha-methylacyl-CoA racemase. The tumor invaded the renal vein; no lymph nodes or extension of the tumor into the soft tissues of the hilum were detected (stage pT3a pNx). Using the Illumina TruSight Oncology 500 kit, a clinically significant c.3481dup, p.(Arg1161LysfsTer13) and c.2050del, p.(Gln684Asnfs4) mutations of the tet methylcytosine dioxygenase 2 (<i>TET2</i>) gene and c.296, p.(Pro99GlnfsTer60) mutation of the von Hippel-Lindau (<i>VHL</i>) gene were identified. The immunophenotype and molecular genetics of our tumor were consistent with CCRCC, suggesting that the unusual hemangioma-like growth pattern is most likely the result of extensive regressive changes. In contrast to all previously published reports, our study demonstrated that, despite the bland histological appearance, renal cell carcinomas with hemangioma-like features can invade the renal vein and follow an aggressive clinical course.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1459-1466"},"PeriodicalIF":0.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143189205","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
Penile Carcinoma Cuniculatum: A Case Report of an Extremely Rare Subtype of Squamous Cell Carcinoma. 阴茎鳞状癌:一极罕见的鳞状细胞癌亚型1例报告。
IF 0.9 4区 医学
International Journal of Surgical Pathology Pub Date : 2025-09-01 Epub Date: 2025-02-06 DOI: 10.1177/10668969251314121
Jiří Lenz, Květoslava Michalová, Luděk Fiala, Michal Tichý, František Tichý, Jana Němcová, Miroslav Kavka
{"title":"Penile Carcinoma Cuniculatum: A Case Report of an Extremely Rare Subtype of Squamous Cell Carcinoma.","authors":"Jiří Lenz, Květoslava Michalová, Luděk Fiala, Michal Tichý, František Tichý, Jana Němcová, Miroslav Kavka","doi":"10.1177/10668969251314121","DOIUrl":"10.1177/10668969251314121","url":null,"abstract":"<p><p>Carcinoma cuniculatum is a very rare subtype of well-differentiated squamous cell carcinoma affecting several anatomical locations, including the penis. In this paper, we describe a case report of penile carcinoma cuniculatum highlighting some unusual clinical features. The patient was 61-year-old with a cauliflower-like tumor of the glans penis of less than 1-year duration. Microscopic examination revealed a p16-negative well-differentiated squamous proliferation characterized by narrow and cystic spaces extending through the lesion. Carcinoma cuniculatum is characterized by a strong tendency to deep penetration, typically presenting in patients in their seventh to eighth decades and with a long duration of the disease. In contrast, the tumor presented herein occurred in a younger patient with a shorter disease duration and showed only shallow growth into the underlying structures. Since penile carcinoma cuniculatum is a local disease without metastatic potential (regardless of tumor size and disease duration), awareness of this entity is important, especially for distinguishing it from other more aggressive subtypes of squamous cell carcinoma, which require different therapeutic approaches.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1477-1483"},"PeriodicalIF":0.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143364647","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
Adenomatoid Tumor of the Liver with Prominent Admixed Cavernous Hemangioma-Like Vessels: An Exceptional Occurrence. 肝脏腺瘤样肿瘤伴有明显的海绵状血管瘤样血管:罕见病例
IF 0.9 4区 医学
International Journal of Surgical Pathology Pub Date : 2025-09-01 Epub Date: 2025-03-13 DOI: 10.1177/10668969251323930
Ruoji Zhou, Hanlin L Wang
{"title":"Adenomatoid Tumor of the Liver with Prominent Admixed Cavernous Hemangioma-Like Vessels: An Exceptional Occurrence.","authors":"Ruoji Zhou, Hanlin L Wang","doi":"10.1177/10668969251323930","DOIUrl":"10.1177/10668969251323930","url":null,"abstract":"<p><p>Adenomatoid tumors are benign neoplasms of mesothelial phenotype, commonly found in the genital tracts. Hepatic adenomatoid tumors are extremely uncommon and can present diagnostic challenges, particularly when mixed with other histologic elements. Here we report an adenomatoid tumor with prominent admixed cavernous hemangioma-like vessels in the liver of a 58-year-old woman. Grossly, the tumor appeared as a pedunculated lesion on the hepatic surface with cystic spaces filled with blood. Microscopic examination revealed numerous blood vessels of varying sizes intermingled with anastomosing tubular structures lined by cuboidal epithelioid cells that were immunohistochemically positive for mesothelial markers. Areas of typical cavernous hemangioma were also present within the same lesion. To our knowledge, this tumor represents the first documented instance of an adenomatoid tumor with prominent admixed cavernous hemangioma-like vessels in the liver. Our case report summarizes previously reported hepatic adenomatoid tumors and highlights their clinical, histopathologic, and immunohistochemical features. Given the various morphologic presentations, adenomatoid tumors can mimic other neoplasms. Their benign nature and excellent prognosis with complete surgical resection make accurate diagnosis crucial. Increased understanding and awareness of this uncommon tumor, combined with morphologic analysis and immunohistochemical studies, can help overcome diagnostic challenges to ensure accurate diagnosis and appropriate clinical management.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1505-1512"},"PeriodicalIF":0.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12276407/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143624751","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A Rare Lesion of Soft Tissue Malakoplakia Mimicking Neoplasm in the Thigh. 一种罕见的大腿软组织错构瘤病变。
IF 0.9 4区 医学
International Journal of Surgical Pathology Pub Date : 2025-09-01 Epub Date: 2025-02-04 DOI: 10.1177/10668969241311513
Asawari Arwikar, Divya Shetty, Anita Sharan
{"title":"A Rare Lesion of Soft Tissue Malakoplakia Mimicking Neoplasm in the Thigh.","authors":"Asawari Arwikar, Divya Shetty, Anita Sharan","doi":"10.1177/10668969241311513","DOIUrl":"10.1177/10668969241311513","url":null,"abstract":"<p><p>Malakoplakia, a rare acquired histiocytic inflammatory disease. It is an incidental finding on histopathology examination, as it does not have definite clinical manifestations. It typically affects the genitourinary tract but can also involve various anatomical sites. We present malakoplakia involving the left thigh, a rare extragenital manifestation. A 42-year-old male patient presented with insidious left thigh swelling, which gradually increased in size over a year, without associated pain or systemic symptoms. Imaging and fine needle aspiration cytology prompted a suspicion of malignancy. Wide resection was performed, and histopathological examination revealed characteristic features of malakoplakia, including Michaelis-Gutmann bodies and histiocytic infiltrates. Immunohistochemistry confirmed the histiocytic origin of the cells. Despite the absence of microbiological correlation, the patient remained asymptomatic with no recurrence at one-year follow-up. Malakoplakia poses diagnostic challenges due to its non-specific clinical and radiological features. Comprehensive histopathological evaluation is essential for accurate diagnosis, particularly in atypical locations. Our report underscores the importance of considering malakoplakia in the differential diagnosis of soft tissue masses, highlighting the need for further research to elucidate its pathogenesis and optimize management strategies.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1445-1449"},"PeriodicalIF":0.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143189199","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
Scrotal Malakoplakia in a Post-Lung Transplant Patient: A Case Report. 肺移植后阴囊斑疹1例。
IF 0.9 4区 医学
International Journal of Surgical Pathology Pub Date : 2025-09-01 Epub Date: 2025-02-20 DOI: 10.1177/10668969251316910
Neha Seth, Saroja Devi Geetha, Wayne Tam, Yani Zhao
{"title":"Scrotal Malakoplakia in a Post-Lung Transplant Patient: A Case Report.","authors":"Neha Seth, Saroja Devi Geetha, Wayne Tam, Yani Zhao","doi":"10.1177/10668969251316910","DOIUrl":"10.1177/10668969251316910","url":null,"abstract":"<p><p>Malakoplakia is a rare granulomatous disease characterized histologically by Michaelis-Gutmann bodies and sheets of macrophages with granular eosinophilic cytoplasm. While it most commonly affects the genitourinary tract, it can manifest in various locations, including cutaneous sites. This report details a rare example of scrotal malakoplakia in an 82-year-old man with a history of idiopathic pulmonary fibrosis, developing 7 months post-lung transplant, during ongoing chronic immunosuppressive therapy. Histopathological examination confirmed the diagnosis. This report highlights the importance of considering malakoplakia in the differential diagnosis of scrotal lesions, particularly in immunocompromised patients where the clinical presentation may be atypical.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1495-1498"},"PeriodicalIF":0.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143458033","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
Giant Cell Tumor of the Uterus: A Report and Review of the Literature. 子宫巨细胞瘤:一份报告和文献综述。
IF 0.9 4区 医学
International Journal of Surgical Pathology Pub Date : 2025-09-01 Epub Date: 2025-01-28 DOI: 10.1177/10668969241312402
João Martins Gama, James Trent Fry, Charles Matthew Quick
{"title":"Giant Cell Tumor of the Uterus: A Report and Review of the Literature.","authors":"João Martins Gama, James Trent Fry, Charles Matthew Quick","doi":"10.1177/10668969241312402","DOIUrl":"10.1177/10668969241312402","url":null,"abstract":"<p><p>Giant cell tumors are neoplasms that usually occur in the long bones of young adults. They can rarely present in the soft tissue and may display malignant behavior. Giant cell malignancies have previously been reported as tumors primary of the uterus but are exceptionally rare. Although uncommon, it is important to consider when a neoplasm with osteoclast-like giant cells is encountered. In this report, we describe a 70-year-old female patient with an unremarkable past history who presented with an enlarged uterus. Transvaginal ultrasound demonstrated multiple fibroids and a thickened endometrium. A biopsy diagnosis of a highgrade sarcoma was rendered and further refined in the surgical resection as malignant giant cell tumor of the uterus. In this case report, we describe the findings of this case and discuss the differential diagnosis of this entity.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1467-1472"},"PeriodicalIF":0.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143052491","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
YAP1::MAML2 Fusion Positive Epithelioid Soft Tissue Neoplasms: A Case Report and Review of the Literature. YAP1::MAML2融合阳性上皮样软组织肿瘤1例报告及文献复习。
IF 0.9 4区 医学
International Journal of Surgical Pathology Pub Date : 2025-09-01 Epub Date: 2025-03-13 DOI: 10.1177/10668969251323929
Claudia Grosse, Petar Noack, Alexandra Grosse
{"title":"<i>YAP1::MAML2</i> Fusion Positive Epithelioid Soft Tissue Neoplasms: A Case Report and Review of the Literature.","authors":"Claudia Grosse, Petar Noack, Alexandra Grosse","doi":"10.1177/10668969251323929","DOIUrl":"10.1177/10668969251323929","url":null,"abstract":"<p><p>In the recent literature soft tissue neoplasms which harbor a recurrent <i>YAP1::MAML2</i> fusion and have an epithelioid morphology with resemblance to myxoinflammatory fibroblastic sarcoma (MIFS) have been reported. In a recent series these tumors had a nodular appearance and central necrosis and were termed nodular necrotizing variants of MIFS. But their exact relationship to MIFS and their biological behavior seem to be unclear. We report a <i>YAP1::MAML2</i> fusion positive nodular and necrotizing tumor with a MIFS-like morphology and provide a review of the literature.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1513-1518"},"PeriodicalIF":0.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143624748","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
Intestinal Type Sinonasal Adenocarcinoma: A Clinicopathological Study of 48 Patients With Review of Literature. 肠型鼻腺癌48例临床病理分析并文献复习。
IF 0.9 4区 医学
International Journal of Surgical Pathology Pub Date : 2025-09-01 Epub Date: 2025-02-09 DOI: 10.1177/10668969241309936
Nasir Ud Din, Saba Akram, Muhammad Raza, Zubair Ahmad
{"title":"Intestinal Type Sinonasal Adenocarcinoma: A Clinicopathological Study of 48 Patients With Review of Literature.","authors":"Nasir Ud Din, Saba Akram, Muhammad Raza, Zubair Ahmad","doi":"10.1177/10668969241309936","DOIUrl":"10.1177/10668969241309936","url":null,"abstract":"<p><p>BackgroundIntestinal type sinonasal adenocarcinoma is a gland forming malignant tumor of sinonasal tract which is histologically and immunohistochemically similar to intestinal type adenocarcinomas. Intestinal type sinonasal adenocarcinoma has a well-recognized etiological association with occupational exposure to wood dusts.ObjectiveTo report the clinicopathological factors of intestinal type sinonasal adenocarcinoma and review the published literature.MethodsForty-eight tumors reported as intestinal type sinonasal adenocarcinoma were retrieved and clinicopathological features were noted.ResultsAge ranged from 22 to 79 (mean 44) years with 36 men and 12 women. Most patients were presented with nasal blockage and difficulty in breathing. Occupational exposure to dust was present in the majority of patients. Tumors were composed of papillae or acini lined by dysplastic intestinal type columnar epithelium with interspersed goblet cells. There were 13 well differentiated (low grade) and 35 poorly differentiated (high-grade) tumors. CDX2 and SATB2 were expressed by the tumor cells in most tumors. Follow-up was available in 24 patients (range 3 months to 10 years). Most of the patients received chemo and/or radiotherapy. Metastases occurred in 19 out of 24 patients. Brain metastases were very common. All patients with metastases died of their disease.ConclusionClinical and morphological features in our series were similar to published studies. It is important for pathologists to remember the association of these tumors with occupational exposure to wood dusts and to exclude metastases of intestinal adenocarcinomas when confronted by these tumors in the sinonasal tract.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1321-1333"},"PeriodicalIF":0.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143382420","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
SMARCB1-deficient Medullary-Like Renal Cell Carcinoma Without SMARCB1/INI1 Gene Deletion. 没有SMARCB1/INI1基因缺失的髓样肾细胞癌
IF 0.9 4区 医学
International Journal of Surgical Pathology Pub Date : 2025-09-01 Epub Date: 2025-02-21 DOI: 10.1177/10668969251316906
Yanning Zhang, Jianmin Zhao, Xiaojing Teng, Guangyong Chen
{"title":"SMARCB1-deficient Medullary-Like Renal Cell Carcinoma Without <i>SMARCB1/INI1</i> Gene Deletion.","authors":"Yanning Zhang, Jianmin Zhao, Xiaojing Teng, Guangyong Chen","doi":"10.1177/10668969251316906","DOIUrl":"10.1177/10668969251316906","url":null,"abstract":"<p><p>High-grade renal cell carcinoma with morphology and immunophenotypic features identical to renal medullary carcinoma, occurring in a patient without evidence of sickle cell trait or disease, is proposed to be diagnosed as renal cell carcinoma unclassified with medullary phenotype (RCCU-MP) and classified as a subtype of SMARCB1-deficient renal medullary carcinoma in the World Health Organization (WHO) 2022 edition. So far, about twelve tumors of RCCU-MP have been reported in the literature. Herein, we extend this observation to a tumor of RCCU-MP in a 39-year-old patient, morphologically similar to renal medullary carcinoma. SMARCB1 expression was absent by immunohistochemistry, but there was no evidence of hemoglobinopathy. The tumor cells were positive for keratin19, PAX8 and PAX2. GATA3, OCT3/4, and ALK were negative. Fluorescence in situ hybridization was conducted to detect the <i>SMARCB1</i> gene locus on chromosome 22 (22q11.23 region). The results showed no evidence of deletion or translocation involving the gene. Perirenal lymph node metastases were detected. The patient did not receive any treatment. Six months after surgery, she developed a local recurrence. In addition to <i>SMARCB1</i> gene deletion or translocation, other factors may be associated with the loss of INI1 (SMARCB1) protein. The presence of perirenal lymph node metastases and recurrence despite nephrectomy is indicative of the poor prognosis of this tumor. Further investigation of the relationship between the loss of SMARCB1 protein and the development of RCCU-MP might improve our understanding of the pathogenesis of this malignant tumor.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1499-1504"},"PeriodicalIF":0.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143467942","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
Perivascular Epithelioid Cell Tumor of the Lung With a Novel YAP1::TFE3 Fusion. 新型 YAP1::TFE3 融合的肺血管周围上皮样细胞瘤
IF 0.9 4区 医学
International Journal of Surgical Pathology Pub Date : 2025-09-01 Epub Date: 2025-03-13 DOI: 10.1177/10668969251323936
William MacDonald, Matthew R Avenarius, Jenna Aziz, Aaron Guo, Desmond M D'Souza, Swati Satturwar, Konstantin Shilo
{"title":"Perivascular Epithelioid Cell Tumor of the Lung With a Novel <i>YAP1::TFE3</i> Fusion.","authors":"William MacDonald, Matthew R Avenarius, Jenna Aziz, Aaron Guo, Desmond M D'Souza, Swati Satturwar, Konstantin Shilo","doi":"10.1177/10668969251323936","DOIUrl":"10.1177/10668969251323936","url":null,"abstract":"<p><p>Perivascular epithelioid cell tumor (PEComa) belongs to a family of rare mesenchymal neoplasms that share characteristic morphologic, immunohistochemical and molecular findings. In this report, we provide a detailed clinicopathological characterization of a PEComa incidentally discovered in the right lung of a 53-year-old woman. This tumor with epithelioid cell morphology and myomelanocytic differentiation demonstrated a <i>TFE3::YAP1</i> fusion by targeted RNA sequencing. While a subset of PEComas shows <i>TFE3</i> rearrangements, fusion with <i>YAP1</i> has not been systematically documented in this entity. Clear cell stromal tumor of the lung and epithelioid hemangioendothelioma characteristically display the <i>TFE3::YAP1</i> fusion; however, as currently defined, both lack myomelanocytic features. Here, we describe a novel <i>TFE3</i> fusion partner that further expands the spectrum of molecular alterations seen in PEComa.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1519-1525"},"PeriodicalIF":0.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12276402/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143624755","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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