Case Reports in Pathology最新文献

{"title":"A Rare Finding of Pulmonary Epithelioid Hemangioendothelioma.","authors":"Riona Park, Curtis Ober, Calista Sha, Hailey Lau, Isabella Sha, Alain Borczuk, Paul C Lee","doi":"10.1155/crip/1830357","DOIUrl":"10.1155/crip/1830357","url":null,"abstract":"<p><p>Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor with variable presentation and malignancy. These highly uncommon tumors may arise from the liver, soft tissues, bones, and, more infrequently, from the lungs. Given its rarity, treatment approach is not unanimous, with ongoing debate regarding the relative benefits of surgical resection and radiation therapy. In support of growing efforts to standardize treatment of these tumors, we present our findings of a rare pulmonary EHE, incidentally detected in the left lung of a 65-year-old, nonsmoking female patient with a history of diabetes, hypertension, and renal cell carcinoma (RCC) in remission for 8 years after surgical resection. A surgical route was recommended, and left robot-assisted thoracoscopic wedge resections were performed. Final pathological assessment confirmed two pulmonary EHE tumors, one resected from the left upper lobe (LUL) and another from the left lower lobe (LLL) of the lung. Her postoperative course was uneventful, and at 13 months postresection, there has been no evidence of recurrence.</p>","PeriodicalId":45638,"journal":{"name":"Case Reports in Pathology","volume":"2026 ","pages":"1830357"},"PeriodicalIF":0.4,"publicationDate":"2026-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13673118/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149216962","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Recurrent Thoracic Intradural Extramedullary Calcifying Pseudoneoplasm.","authors":"Masayuki Shintaku, Takeshi Matsui, Takahiro Fukuzaki, Masayuki Ishihara, Katsunori Uchida, Koji Tsuta","doi":"10.1155/crip/6088106","DOIUrl":"10.1155/crip/6088106","url":null,"abstract":"<p><p>A case of calcifying pseudoneoplasm of the neuraxis (CAPNON) that arose as an intradural mass of the thoracic spinal cord of a 53-year-old man, a motorcycle mechanic, is reported. The mass showed regrowth after the subtotal removal and was resected again 8 years later. Both primary and regrowth lesions consisted of calcified round nodules of the myxochondroid matrix rimmed by polygonal or short spindle cells. Fibrous tissue showing chronic inflammatory cell infiltration intervened between the nodules. Lamellar or woven bone tissue was also observed. In the regrowth lesion, the myxochondroid matrix was reduced in amount and partly replaced by fibrous tissue. Cells rimming the myxochondroid matrix were immunoreactive to epithelial membrane antigen and somatostatin receptor Type 2A, supporting the meningothelial origin. The pathogenesis of CAPNON remains obscure, but it is a nonneoplastic, probably reactive disorder to some unknown causes, and its close histopathological resemblance to tumoral calcinosis of somatic soft tissue suggests that both disorders share a common pathogenesis. Meningothelial cells surrounding myxochondroid nodules in the present lesions probably represent reactive meningothelial hyperplasia and reflect the locoregional specificity of the lesions. In the present case, some chronological changes of histopathological findings could be observed by comparing the specimens of two operations. Occupational mechanical overload to the vertebral column for a long term might have contributed to the pathogenesis of CAPNON.</p>","PeriodicalId":45638,"journal":{"name":"Case Reports in Pathology","volume":"2026 ","pages":"6088106"},"PeriodicalIF":0.4,"publicationDate":"2026-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13669533/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149217012","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Transformation to Plasmablastic Lymphoma Following CD19-Directed CAR T Cell Therapy for Refractory Diffuse Large B Cell Lymphoma: A Case Report.","authors":"Sinan G Khudhur, Ishaq A Asghar, Yousif M Abdelmoneim, Liuyan Jiang, Yao-Shan Fan, Aziza Nassar","doi":"10.1155/crip/6303492","DOIUrl":"10.1155/crip/6303492","url":null,"abstract":"<p><p>Plasmablastic lymphoma is a rare, aggressive large B cell lymphoma characterized by plasmablastic morphology, terminal B cell (plasma cell) differentiation, frequent loss of conventional B cell markers, and poor clinical outcomes. Although classically associated with immunodeficiency and Epstein-Barr virus (EBV) infection, plasmablastic lymphoma has also been described as an uncommon mechanism of relapse after CD19-directed chimeric antigen receptor T cell (CAR-T) therapy. We report a 69-year-old man with a remote history of chronic lymphocytic leukemia/small lymphocytic lymphoma and subsequent refractory diffuse large B cell lymphoma (DLBCL) who developed rapidly progressive, CD19-negative plasmablastic lymphoma approximately 1 month after CD19-directed CAR-T therapy. Although DLBCL was of a nongerminal-center phenotype with expression of pan-B markers including CD19 and lack of CD138, the post-CAR-T transformed lymphoma demonstrated acquisition of plasmablastic morphology and CD138 expression, loss of pan-B markers and negative for EBV by in situ hybridization and HHV8 by immunostaining. Fluorescence in situ hybridization (FISH) on the plasmablastic lymphoma showed a <i>MYC::IGH</i> fusion and low-level MYC copy gain that were absent in the antecedent DLBCL. Disease dissemination was later confirmed on left pleural fluid cytology a few days before the patient passed away. This case highlights EBV and HHV8 negative plasmablastic transformation as a rare but important post-CAR-T relapse pattern and underscores the need for repeat biopsy, comprehensive paired immunophenotyping, and molecular studies in patients with atypical progression after cellular therapy.</p>","PeriodicalId":45638,"journal":{"name":"Case Reports in Pathology","volume":"2026 ","pages":"6303492"},"PeriodicalIF":0.4,"publicationDate":"2026-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13669536/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149217000","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Postmortem Presentation and Review of Danon Disease: A Rare Metabolic Disorder.","authors":"Bryanna Rather Eguh, Antonio Farinhas, Shilpika Bagh, Rachael Connington, Stephanie Klinesmith","doi":"10.1155/crip/1525653","DOIUrl":"https://doi.org/10.1155/crip/1525653","url":null,"abstract":"<p><p>Danon disease is a rare, X-linked dominant inherited lysosomal storage disorder that involves a mutation in the lysosomal-associated membrane Protein 2 gene. Mutation in this gene results in severe disease clinical manifestations that may include skeletal and cardiac myopathies, retinopathies, and intellectual disabilities. This case report explores postmortem findings observed in a 25-year-old male donor who succumbed to heart failure secondary to Danon disease. With no transplant history, postmortem findings include hypertrophy, extensive fibrosis, and vacuolization in cardiomyocytes compared with expected anatomical findings in an unaffected patient. This case report is aimed at deepening the understanding of the clinical manifestations of Danon disease, raise awareness, and explore diagnostic approaches and differentials to help identify additional treatment options that may improve patient outcomes.</p>","PeriodicalId":45638,"journal":{"name":"Case Reports in Pathology","volume":"2026 ","pages":"1525653"},"PeriodicalIF":0.4,"publicationDate":"2026-10-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13659043/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149185076","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Adult Low-Grade Glioma With <i>MEF2D::NTRK1</i> Fusion: A Case Report and Literature Review.","authors":"Xiuyi Pan, Linmao Zheng, Xiaoxue Yin, Mengxin Zhang, Ting Yu, Zhengzheng Su, Xueqin Chen, Ni Chen, Yuyan Wei","doi":"10.1155/crip/6015225","DOIUrl":"10.1155/crip/6015225","url":null,"abstract":"<p><p>Low-grade glioma (LGG) is a malignancy that is rare in adult central nervous system (CNS). Clinical management decisions are increasingly guided by molecular alterations rather than the histological subtype alone. Neurotrophic tropomyosin receptor kinase 1 (<i>NTRK1</i>) is constitutively activated in multiple tumor types. TRK inhibitors, targeting NTRK fusion proteins, have exhibited notable therapeutic efficacy in some <i>NTRK</i> gene fusion tumors. NTRK fusions with diverse partners have been reported in CNS tumors. Nevertheless, <i>MEF2D::NTRK1</i> fusion cases were only reported in high-grade glioneuronal neoplasms and infantile-type hemispheric glioma. We report a 33-year-old patient with LGG morphological features, but presented a diffuse invasive growth pattern involving the corpus callosum, the third ventricle, the entire anterior cranial base and the suprasellar cistern. Histology showed oligodendrocyte-like cells within an abundant mucinous matrix, with scattered astrocytes and no dense collagen, Rosenthal fibers or glomeruloid microvascular. The nucleoli were inconspicuous and mitotic figure was rare. Next-generation sequencing (NGS) showed it harbored a <i>MEF2D::NTRK1</i> fusion and 22q loss with no additional mutations identified. DNA methylation clustered with pilocytic astrocytoma (PA) and diffuse leptomeningeal glioneuronal tumor (DLGNT). However, according to the tumor's growth pattern, morphological features, and molecular characteristics, it does not match to PA or DLGNS. This report provides a comprehensive description of the clinical, radiographic, histopathological, immunohistochemical, and molecular characteristics of <i>MEF2D::NTRK1</i> fused LGG with the aim of enhancing the clinical and pathological understanding of <i>MEF2D::NTRK1</i> fused LGG. Patient in this case had benefited from <i>NTRK</i>-targeted therapeutic intervention.</p>","PeriodicalId":45638,"journal":{"name":"Case Reports in Pathology","volume":"2026 ","pages":"6015225"},"PeriodicalIF":0.4,"publicationDate":"2026-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13643359/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149138705","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Two Cases of NUT Carcinoma of the Lung: Same Name With Different Appearance: A Case Report.","authors":"Eriko Atsumi, Hidenori Kawasaki, Isoko Owan, Futoshi Higa, Hajime Aoyama, Hirofumi Matsumoto","doi":"10.1155/crip/9648746","DOIUrl":"10.1155/crip/9648746","url":null,"abstract":"<p><p>NUT carcinoma is a poorly differentiated tumor defined by nuclear protein in testis (<i>NUTM1</i>) gene rearrangement. Histologically, it is characterized by a primitive small round cell proliferation with abrupt squamous differentiation, although the extent of squamous differentiation varies among cases. Inconspicuous squamous epithelium may result in misdiagnosis as lymphoma or other small round cell tumors. In this study, we present two cases of NUT carcinoma: (1) a man in his 20s with a right hilar and middle lobe mass, in whom a lymph node biopsy was performed followed by autopsy; and (2) a woman in her 30s with a left lung mass that was surgically resected. Both cases showed primitive small round cell proliferation; however, they differed in both morphological and immunohistochemical features. Morphologically, the appearance and extent of squamous differentiation varied between the two cases. The first case exhibited abrupt squamous epithelium intermingled with the round cell component, whereas the second exhibited tiny foci of squamous epithelium, with the remainder composed of primitive round cells. Regarding immunohistochemistry, the first case showed diffuse keratin positivity, whereas the second showed almost no staining. Based on these results, the first case was considered typical NUT carcinoma, whereas the second was difficult to diagnose due to limited squamous epithelium. Although the key diagnostic feature of NUT carcinoma is reported to be abrupt squamous differentiation, many cases do not exhibit this feature, which may represent a diagnostic pitfall. There are also pitfalls in immunohistochemistry: keratin, which confirms epithelial origin, may be negative; p40, which highlights squamous differentiation, may also be negative; and TTF-1 and neuroendocrine markers may be positive. This report compares these two cases morphologically and immunohistochemically and highlights the diagnostic challenges of NUT carcinoma with scant squamous components. Additionally, related literature is reviewed, and key diagnostic points, including immunohistochemistry, are discussed.</p>","PeriodicalId":45638,"journal":{"name":"Case Reports in Pathology","volume":"2026 ","pages":"9648746"},"PeriodicalIF":0.4,"publicationDate":"2026-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13640534/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149116250","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Plasmablastic Richter Transformation of Chronic Lymphocytic Leukemia With Aberrant Epithelial Marker Expression Presenting as a Pleural Effusion: A Diagnostic Challenge.","authors":"Jamie Rogers, Jacky Zahedha Akhter, Marilin Rosa, Sara Niyazi, Dietrich Werner-Idiaquez, Javier Pinilla-Ibarz, Ariel Grajales-Cruz, Lynn Moscinski, Christopher B Ryder","doi":"10.1155/crip/5618071","DOIUrl":"https://doi.org/10.1155/crip/5618071","url":null,"abstract":"<p><p>Richter transformation (RT) is a well-known complication of chronic lymphocytic leukemia (CLL), and it is usually characterized by high-grade transformation to diffuse large B-cell lymphoma or, less commonly, to classic Hodgkin lymphoma. Rare reports describe RT presenting as plasmablastic lymphoma or myeloma. In this scenario, complete clinicopathological evaluation is critical to correctly classify the atypical transformation, and demonstration of a clonal relationship with precursor CLL is essential to confirm RT. Herein, we present a case of CLL with plasmablastic RT diagnosed on pleural effusion. The rarity of this phenomenon, compounded by aberrant expression of markers of epithelial differentiation, posed a significant diagnostic challenge. The unusual presentation in our case emphasizes the need for a comprehensive approach integrating clinical, morphologic, phenotypic, and molecular data to avoid misclassification and to guide appropriate clinical management.</p>","PeriodicalId":45638,"journal":{"name":"Case Reports in Pathology","volume":"2026 ","pages":"5618071"},"PeriodicalIF":0.4,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13628566/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149116230","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Correction to 'Advanced Lymphoblastic Lymphoma, NOS in Leukaemic Phase Presenting With Sudden and Fatal CNS Haemorrhage'.","authors":"","doi":"10.1155/crip/9856983","DOIUrl":"https://doi.org/10.1155/crip/9856983","url":null,"abstract":"<p><p>[This corrects the article DOI: 10.1155/crip/5845893.].</p>","PeriodicalId":45638,"journal":{"name":"Case Reports in Pathology","volume":"2026 ","pages":"9856983"},"PeriodicalIF":0.4,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13618568/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149076064","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Rare Case of a Non-Invasive Vaginal Urothelial Carcinoma Demonstrating a Clonal Relationship With a Metachronous Non-Invasive Bladder Urothelial Carcinoma.","authors":"Cassandra Hill, Noman Ghazanfar, Andy Chuang, Robert Gordon Hislop, Ketan Rathod, Jamie Wilson, Ghulam Mustafa Nandwani","doi":"10.1155/crip/4360431","DOIUrl":"10.1155/crip/4360431","url":null,"abstract":"<p><p>Non-invasive vaginal urothelial carcinomas (UCs) are extremely rare and are almost always associated with a non-invasive bladder UC. The pathogenesis of these rare vaginal lesions is uncertain. They may represent a primary vaginal tumour or they may arise from a urinary tract UC, in which tumour cells \"seed\" by non-invasive mechanisms. We describe a case of a non-invasive vaginal UC, occurring 6 years following treatment of a non-invasive bladder UC, managed with an anterior pelvic exenteration and followed up over 5 years. Molecular analysis using a genome-wide single nucleotide polymorphism (SNP) microarray to assess copy number variations and regions of copy number neutral loss of heterozygosity (CN-LOH) was performed on both tumours. These results evidence a clonal relationship between these two tumours indicating that the vaginal tumour is the result of non-invasive tumour \"seeding\" from the original bladder lesion. To our knowledge this is the first case report to demonstrate clonality between metachronous non-invasive bladder and vaginal UCs.</p>","PeriodicalId":45638,"journal":{"name":"Case Reports in Pathology","volume":"2026 ","pages":"4360431"},"PeriodicalIF":0.4,"publicationDate":"2026-09-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13584327/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148991857","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"<i>DEK::AFF2</i> Fusion-Associated Sinonasal Carcinoma With Intracranial and Orbital Involvement.","authors":"Katelyn Elizabeth Golden, Josh Barlow, Hong Yu, David J Hernandez, Fouad El-Dana, Neda Zarrin-Khameh, Songlin Zhang, Li Liang","doi":"10.1155/crip/7248935","DOIUrl":"10.1155/crip/7248935","url":null,"abstract":"<p><p>Sinonasal carcinomas encompass a molecularly heterogeneous group of malignancies. <i>DEK::AFF2</i> fusion-associated carcinoma is a recently recognized subtype of nonkeratinizing squamous cell carcinoma characterized by deceptively bland morphology yet clinically aggressive behavior. We report the case of a 67-year-old woman who presented with a rapidly enlarging sinonasal mass with extensive local invasion involving both the intracranial compartment and orbit. Histologically, the tumor was composed of cytologically bland cells with monotonous nuclei and exhibited a combination of exophytic and endophytic growth patterns that closely mimicked a sinonasal papilloma. Although the tumor shows predominantly squamous differentiation, focal extracellular mucin was also present. The brain-invasive foci demonstrated tumor cells in sheets and nests floating within pools of mucin, a pattern focally reminiscent of mucinous adenocarcinoma. RNA sequencing identified a <i>DEK::AFF2</i> fusion with breakpoints at <i>DEK</i> exon 7 and <i>AFF2</i> exon 6, confirming the diagnosis. The tumor demonstrated positive PD-L1 expression with a combined positive score (CPS) of 40 and a low tumor mutational burden (TMB) of 0.5 mutations/Mb. This case highlights the diagnostic challenges posed by <i>DEK::AFF2</i> fusion-associated carcinoma, underscores the importance of molecular profiling for accurate classification, and demonstrates the aggressive clinical behavior of this rare entity.</p>","PeriodicalId":45638,"journal":{"name":"Case Reports in Pathology","volume":"2026 ","pages":"7248935"},"PeriodicalIF":0.4,"publicationDate":"2026-09-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13576631/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148978214","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}