{"title":"Preneoplastic and Neoplastic Biliary Diseases.","authors":"Tom Z Liang, Shefali Chopra","doi":"10.1097/PAP.0000000000000497","DOIUrl":"https://doi.org/10.1097/PAP.0000000000000497","url":null,"abstract":"<p><p>Preneoplastic and neoplastic biliary disease comprises biliary intraepithelial neoplasia (BilIN), intraductal papillary neoplasms, mucinous cystic neoplasms (MCNs), and cholangiocarcinoma and their variants. Correct recognition of these entities can be challenging, especially on small/needle biopsies, but is required to plan therapy and guide transplant in the setting of cirrhosis. Salient histologic features of these entities, along with ancillary use of immunostains and key molecular findings aiding in diagnosis, are discussed. Type 2 intraductal papillary neoplasm of the bile ducts is typically associated with an invasive malignancy and lack unique molecular features associated with the Type 1 intraductal papillary neoplasm, thus they are called \"papillary cholangiocarcinoma\" by some authors. Some of the cholangiocarcinoma variants, like enteroblastic and mucoepidermoid, are under-recognized and can pose diagnostic challenges. The tubulocystic and cholangioblastic variants are relatively recently described but are being increasingly recognized. The cholangioblastic variant has a novel NIBPL-NACC1 fusion described in the more recent larger series reported, making it a somewhat unique variant of cholangiocarcinoma. Nomenclature of the cholangioblastic variant is in evolution as is the link between adenofibroma and the tubulocystic variant. Correct recognition of these variant subtypes would aid in long-term studies to better determine the prognosis in these subtypes.</p>","PeriodicalId":7305,"journal":{"name":"Advances In Anatomic Pathology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143794411","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Hemangioblastoma-like Clear Cell Stromal Tumor of the Lung: A Comprehensive Review With Insights From a Case.","authors":"Jian Zeng, Peng Lushan, Qin Jing, Wei Du","doi":"10.1097/PAP.0000000000000482","DOIUrl":"https://doi.org/10.1097/PAP.0000000000000482","url":null,"abstract":"<p><p>Hemangioblastoma-like clear cell stromal tumor (HLCCST) of the lung is an exceptionally rare mesenchymal neoplasm that is generally considered benign. Current knowledge of this tumor remains limited. Histologically, HLCCST is defined by a hypervascular stroma with dilated blood vessels and is predominantly composed of epithelial-like cells arranged in solid sheets or nests with uniform morphology. Molecular genetic studies have identified YAP1::TFE3 gene fusions as a hallmark in most reported cases. To date, in addition to our case, a total of 19 HLCCST cases have been reported across 7 publications. Here, we present a comprehensive review of HLCCST, detailing its clinicopathologic features, key molecular alterations, and prognostic data of HLCCST. In addition, we emphasize the importance of accurate recognition and diagnosis of this rare tumor to ensure appropriate treatment and improved patient outcomes.</p>","PeriodicalId":7305,"journal":{"name":"Advances In Anatomic Pathology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143794409","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Hepatoblastoma: Comprehensive Review With Recent Updates.","authors":"Jingjing Jiao, Romil Saxena, Raffaella Morotti","doi":"10.1097/PAP.0000000000000495","DOIUrl":"https://doi.org/10.1097/PAP.0000000000000495","url":null,"abstract":"<p><p>Hepatoblastoma (HB), the most common primary malignant liver tumor of childhood, demonstrates remarkable histologic heterogeneity and can be classified into epithelial or mixed epithelial-mesenchymal subtypes. This review summarizes updates in histologic classification, molecular signatures, staging, and risk stratification of HB. The Children's Hepatic tumors International Collaboration represents an international effort to standardize the study of rare pediatric liver tumors; emphasis continues to remain on improving risk stratification by a combination of clinical, histologic, and molecular features to tailor treatment in a bid to reduce toxicity while maintaining or improving efficacy. Pure fetal HB is cured by complete resection without the need for adjuvant chemotherapy. Malignant rhabdoid tumors have been parsed out from small cell undifferentiated HBs by negative INI-1 staining on immunohistochemistry; these tumors require a distinct and more aggressive chemotherapeutic regimen. The significance of recently characterized \"blastema\" component in HB remains to be elucidated. Hepatocellular neoplasm, not otherwise specified, is a provisional diagnostic category for tumors exhibiting either intermediate or a combination of both HB and hepatocellular carcinoma histologic features. The Children's Hepatic tumors International Collaboration risk stratification algorithm includes age as an important discriminator of risk, in addition to AFP, metastasis, and PreTreatment EXTent of disease stage and its annotations.</p>","PeriodicalId":7305,"journal":{"name":"Advances In Anatomic Pathology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-04-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143771191","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A Primer on Bluesky (and PathSky) for Pathologists, Trainees, and Medical Students.","authors":"Casey P Schukow, Lavisha S Punjabi, Emma Khan","doi":"10.1097/PAP.0000000000000491","DOIUrl":"https://doi.org/10.1097/PAP.0000000000000491","url":null,"abstract":"<p><p>Social media (SoMe) has become an integral tool in modern pathology, facilitating education, research, mentorship, and professional networking. However, the evolving landscape of SoMe platforms presents both opportunities and challenges for pathologists. Bluesky, a decentralized platform launched publically in 2024 has gained significant traction among pathologists as an alternative to \"traditional,\" or more widely-used, platforms like Twitter/X. This narrative review explores the role of SoMe in pathology, introduces Bluesky and its pathology-focused community PathSky, and compares it with other platforms. In addition, practical guidance on joining Bluesky and engaging with PathSky is provided. By embracing innovative platforms like Bluesky, pathologists can enhance collaboration, education, and professional growth in the digital age.</p>","PeriodicalId":7305,"journal":{"name":"Advances In Anatomic Pathology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-03-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143699361","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Bone Matrix-forming Tumors.","authors":"Julio A Diaz-Perez, Andrew E Rosenberg","doi":"10.1097/PAP.0000000000000476","DOIUrl":"10.1097/PAP.0000000000000476","url":null,"abstract":"<p><p>Bone matrix-forming tumors are a group of neoplasms that exhibit differentiation toward any stage of osteoblast development. Their clinicopathologic features can resemble one another, yet their clinical management may vary significantly. Therefore, appropriate treatment requires accurate diagnosis, which can be challenging, especially with limited biopsy specimens. Recently, the driver genetic alterations underlying these neoplasms have been discovered, and their protein products can be targeted for diagnosis and therapy. Herein, we summarize the recent advances in our understanding of bone matrix-forming tumors and emphasize the integration of molecular genetics into their conventional clinicopathologic evaluation.</p>","PeriodicalId":7305,"journal":{"name":"Advances In Anatomic Pathology","volume":" ","pages":"168-179"},"PeriodicalIF":5.1,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142724688","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Cartilage Forming Tumors of the Skeleton.","authors":"Julio A Diaz-Perez, Andrew E Rosenberg","doi":"10.1097/PAP.0000000000000475","DOIUrl":"10.1097/PAP.0000000000000475","url":null,"abstract":"<p><p>Cartilage-forming tumors are a broad and diverse group of neoplasms frequently affecting the skeleton. Distinguishing between the members of this group is important because of significant differences in treatment and prognosis. Accurate diagnosis can be challenging because of similarities in their clinical, radiographic, and pathologic features. Immunohistochemistry and molecular tools are helpful in select instances. Therefore, careful evaluation and correlation of these features are essential in arriving at the correct diagnosis and appropriate patient management. This review provides an overview of the current literature, emphasizing helpful features in diagnosis.</p>","PeriodicalId":7305,"journal":{"name":"Advances In Anatomic Pathology","volume":" ","pages":"132-146"},"PeriodicalIF":5.1,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142714824","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Mesenchymal Tumors of the Tubular Gastrointestinal Tract (Non-GIST): The GI Pathologist's Approach.","authors":"Bence P Kővári, Gregory Y Lauwers","doi":"10.1097/PAP.0000000000000469","DOIUrl":"10.1097/PAP.0000000000000469","url":null,"abstract":"<p><p>Mesenchymal neoplasms of the gastrointestinal tract are rare compared with epithelial lesions. However, over the past few decades, the increasing volume of gastrointestinal endoscopy has expedited the recognition of several novel entities with varying clinical significance. Its spectrum extends from reactive changes and benign neoplasms to highly aggressive sarcomas. At the malignant end of the spectrum, the importance of correctly diagnosing these tumors is underscored by the specific therapeutic implications available for some tumor types (eg, tyrosine kinase inhibitors for gastrointestinal stromal tumors) that allow personalized treatments. Benign lesions frequently surface among routine polypectomy specimens, sometimes offering diagnostic challenges. However, precise classification is the only way to avoid prognostic uncertainty and overtreatment, and to recognize possible syndromic associations. Hereby, we offer a pragmatic review of the topic from the gastrointestinal pathologist's perspective, who, although more accustomed to epithelial neoplasms, can use an algorithmic approach to diagnose mesenchymal entities successfully.</p>","PeriodicalId":7305,"journal":{"name":"Advances In Anatomic Pathology","volume":" ","pages":"110-131"},"PeriodicalIF":5.1,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142714829","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Giant Cell-Rich Tumors of the Skeleton.","authors":"Julio A Diaz-Perez, Andrew E Rosenberg","doi":"10.1097/PAP.0000000000000477","DOIUrl":"10.1097/PAP.0000000000000477","url":null,"abstract":"<p><p>The accurate diagnosis of giant cell-rich tumors of bone is challenging, especially in limited tissue samples. This diverse group of neoplasms have similar and often ambiguous clinical presentations, radiologic features, and morphologic characteristics. During the last decade, the discovery of pathogenic recurrent genetic alterations has allowed the development of immunohistochemical surrogate markers and FISH assays that can help differentiate the entities of this broad group from one another. The correct diagnosis of these neoplasms is essential in the management of the affected patients.</p>","PeriodicalId":7305,"journal":{"name":"Advances In Anatomic Pathology","volume":" ","pages":"157-167"},"PeriodicalIF":5.1,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142724701","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Mesenchymal Tumors of the Human Body: A Targeted Practical Review (Part II).","authors":"Andrew Rosenberg, Andre Pinto","doi":"10.1097/PAP.0000000000000490","DOIUrl":"https://doi.org/10.1097/PAP.0000000000000490","url":null,"abstract":"","PeriodicalId":7305,"journal":{"name":"Advances In Anatomic Pathology","volume":"32 2","pages":"109"},"PeriodicalIF":5.1,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143447723","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Pediatric Mesenchymal Tumors.","authors":"Omar Aljuboori, Ali G Saad","doi":"10.1097/PAP.0000000000000480","DOIUrl":"10.1097/PAP.0000000000000480","url":null,"abstract":"<p><p>Vascular, fibrous/myofibroblastic, and myogenic tumors account for the majority of mesenchymal tumors in children. These tumors often show significant overlap in morphology and immunophenotype posing diagnostic difficulties and, thus, their classification remains challenging. Recent advances in immunohistochemistry have proved helpful in identifying a specific line of differentiation in some tumors, but other tumors remain difficult to classify. Molecular investigations have provided an existing tool to better understand the pathogenesis of some of these tumors and, in some cases like the EWING family of tumors, expanded the classification resulting in the emergence of previously unknown tumors. Some of these tumors are currently diagnosed according to their underlying molecular abnormality, such as CIC -rearranged sarcoma, BCOR -rearranged sarcoma, etc. This review focuses on the common mesenchymal neoplasms of the pediatric population with emphasis on the salient histologic features, immunoprofile, and molecular characteristics. For practical purposes, the latter are summarized in Supplemental Table 1, Supplemental Digital Content 1, http://links.lww.com/PAP/A46 .</p>","PeriodicalId":7305,"journal":{"name":"Advances In Anatomic Pathology","volume":" ","pages":"180-192"},"PeriodicalIF":5.1,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142817030","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}