{"title":"SMARCB1-Deficient Pulmonary Mesenchymal Tumor Without Malignant Histological Features.","authors":"Nozomi Nakajima, Kohei Fukuoka, Seshiru Nakazawa, Yoichi Ohtaki, Nozomi Matsumura, Ayako Yamazaki, Hideaki Yokoo, Akihiko Yoshida, Sumihito Nobusawa","doi":"10.1111/pin.70025","DOIUrl":"https://doi.org/10.1111/pin.70025","url":null,"abstract":"<p><p>Complete loss of nuclear SMARCB1 expression was originally described as a hallmark of malignant rhabdoid tumors, typically occurring in the kidney, soft tissue, and central nervous system (CNS). Generally, SMARCB1 deficiency is associated with malignant histopathological appearance, except for some rare tumors. Herein, we present a case of hitherto undescribed SMARCB1-deficient pulmonary mesenchymal tumor without rhabdoid features or malignant histopathology involving a 62-year-old male patient. Histologically, the tumor demonstrated a moderately cellular proliferation of monomorphic spindle cells arranged in short fascicles or a storiform pattern with intervening collagenous stroma. The mitotic activity was lower than that of typical SMARCB1-deficient tumors, and rhabdoid features and necrosis were absent. Nuclear SMARCB1 expression was lost, and a part of SMARCB1 was revealed to be homozygously deleted. DNA methylation analysis demonstrated that this case was not clustered with other well-known SMARCB1-deficient tumors.</p>","PeriodicalId":19806,"journal":{"name":"Pathology International","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144079460","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Proposal for a New Classification of Rare Cancers Adopting Updated Histological Tumor Types.","authors":"Ryoko Rikitake, Yasushi Yatabe, Yoko Yamamoto, Tatsunori Shimoi, Shintaro Iwata, Yasushi Goto, Yu Mizushima, Akira Kawai, Takahiro Higashi","doi":"10.1111/pin.70021","DOIUrl":"https://doi.org/10.1111/pin.70021","url":null,"abstract":"<p><p>Several classifications have been proposed to define rare cancers; however, the pathophysiological understanding of tumors evolves rapidly. We propose a New Classification of Rare Cancer (NCRC) using the updated International Classification of Diseases for Oncology 3.2 coding system and World Health Organization Classification of Tumors 5th edition. We applied patient data recorded in the National Cancer Registry of Japan to the new classification, setting a cut-off of a crude incidence rate of 6 cases/100 000/year to define rare cancers, and developed a list of rare cancers in Japan from 2016 to 2019. The NCRC system identified various rare cancers, comprising 20.0% of all cancer diagnoses in this period. To examine this classification system's performance, we compared rare/non-rare labeling of cancers by the Surveillance of Rare Cancers in Europe (RARECARENet) project and NCRC system. Compared with cases using the RARECARENet classification in Europe, 69 351 cases/year (6.8%) switched status with our classification, with 45 293 and 232 109 cases (4 years) switching from rare and non-rare, respectively. Major differences included diffuse large B-cell lymphomas, some thyroid cancers, oral cavity and lip cancers, and squamous cell carcinoma of the uterine cervix. As the NCRC includes newly classified tumor entities, it warrants validation using other diverse cohorts.</p>","PeriodicalId":19806,"journal":{"name":"Pathology International","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144079458","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}
Ayuna Sugai, Kosuke Miyai, Keiichi Ito, Susumu Matsukuma, Kimiya Sato
{"title":"Kidney Injury Molecule-1 Expression in Pathological T1b Clear Cell Renal Cell Carcinoma: A Putative Biomarker of High Immune-Inflamed Status and Recurrence.","authors":"Ayuna Sugai, Kosuke Miyai, Keiichi Ito, Susumu Matsukuma, Kimiya Sato","doi":"10.1111/pin.70024","DOIUrl":"https://doi.org/10.1111/pin.70024","url":null,"abstract":"<p><p>Kidney injury molecule-1 (KIM-1) is a potential prognostic marker of advanced-stage clear cell renal cell carcinoma (ccRCC) and is associated with tumor immunogenicity. Little is known about its role in early-stage ccRCC, especially in pathological T1b (pT1b) disease, which shows a higher recurrence rate than pT1a disease. Resected specimens from 112 pT1b ccRCC cases were reviewed and immunohistochemically analyzed for KIM-1 expression. High membranous KIM-1 expression was defined as H score ≥ 140, based on the immunoreactive intensity and area, and cytoplasmic expression in ≥ 10% of cancer cells was considered as high cytoplasmic KIM-1 expression. KIM-1 expression status was compared with clinicopathological variables, including tumor-associated immune cell (TAIC) status. Among the 112 cases, high membranous and cytoplasmic KIM-1 expression was observed in 30 (27%) and 38 (34%) cases, respectively. High membranous KIM-1 expression was significantly associated with a higher nuclear grade, tumor necrosis, hot TAIC status, and shorter recurrence-free survival (RFS) and cancer-specific survival, whereas high cytoplasmic expression was only related to a higher nuclear grade. Multivariate Cox regression analysis revealed that high membranous KIM-1 expression and tumor necrosis were independent predictors of shorter RFS. Our results indicate that membranous KIM-1 expression could be a biomarker for predicting postnephrectomy recurrence in pT1b ccRCC.</p>","PeriodicalId":19806,"journal":{"name":"Pathology International","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144027715","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Gastric Neuroendocrine Tumor With Pancreatic Acinar Cell Differentiation in the Background of Atrophic Gastritis: A Possible Variant of Type 1 ECL-Cell NET-A Case Report.","authors":"Tomoko Norose, Nobuyuki Ohike, Misato Tsukada, Yoshiya Sugiura, Hirotaka Koizumi, Yusuke Nakamoto, Keisuke Tateishi, Shinya Mikami, Junki Koike","doi":"10.1111/pin.70022","DOIUrl":"https://doi.org/10.1111/pin.70022","url":null,"abstract":"<p><p>A gastric neuroendocrine tumor (NET) with pancreatic acinar cell differentiation is extremely rare. We report the case of an 87-year-old woman with a submucosal tumor in the gastric body on a background of atrophic gastritis. She also had Sjögren's syndrome. Initially 17.8 × 6.5 mm, the tumor enlarged over 10 years, leading to wedge resection. The resected mass (45 × 40 × 30 mm) was solid with a pale yellow to gray-white cut surface. Histologically, it showed trabecular or solid nests of epithelial cells with round nuclei and eosinophilic cytoplasm. Immunohistochemistry showed positivity for CKAE1/3, VMAT2, neuroendocrine markers, and pancreatic acinar markers. Ki-67 index was 11.2%. The tumor co-expressed PDX1 and ARX and showed loss of menin and ATRX. These findings support a diagnosis of gastric ECL-cell NET G2 arising in autoimmune gastritis, with secondary pancreatic acinar differentiation. This tumor may represent a variant of type 1 gastric NET.</p>","PeriodicalId":19806,"journal":{"name":"Pathology International","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144037637","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Identification of Target Genes Using Innovative Screening Systems.","authors":"Keisuke Sugita, Morito Kurata","doi":"10.1111/pin.70019","DOIUrl":"https://doi.org/10.1111/pin.70019","url":null,"abstract":"<p><p>Advances in cancer biology have been achieved by the identification of oncogenes and tumor suppressor genes through the remarkable progression of next-generation sequencing. New techniques, such as single-cell analysis, help uncover cancer progression and heterogeneity. Reverse genetic screenings, including methods like random mutagenesis via retroviruses, transposons, RNA interference, and CRISPR, are useful for exploring gene functions and their roles in cancer. Especially in random mutagenesis, CRISPR screening and its modifications have recently emerged as powerful tools due to their comprehensiveness and simplicity in inducing genetic mutations. Initially, CRISPR screening focused on analyzing biological phenotypes in a cell population. It has since evolved to incorporate advanced techniques, such as combining single-cell and spatial analyses. These developments enable the investigation of cell-cell and spatial interactions, which more closely mimic In Vivo microenvironments, offering deeper insights into complex biological processes. These approaches allow for the identification of essential genes involved in cancer survival, drug resistance, and tumorigenesis. Together, these technologies are advancing cancer research and therapeutic development.</p>","PeriodicalId":19806,"journal":{"name":"Pathology International","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-05-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144009584","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Human Papillomavirus-Related Adenosquamous Carcinoma of the Anorectum With Pagetoid Spread.","authors":"Kenta Seki, Taiki Hashimoto, Hiroshi Nagata, Dai Ogata, Shunsuke Tsukamoto, Hiroyuki Takamaru, Yukihide Kanemitsu, Shigeki Sekine","doi":"10.1111/pin.70018","DOIUrl":"https://doi.org/10.1111/pin.70018","url":null,"abstract":"<p><p>Adenosquamous carcinoma is an uncommon type of cancer that comprises malignant squamous and glandular components. We present a case of human papillomavirus (HPV)-positive adenosquamous carcinoma of the anorectum, which exhibited extensive pagetoid spread, in a 70-year-old woman. The tumor had spread from the lower rectum to the perianal skin and was removed through a combined endoscopic and transanal surgical procedure. Histological examination revealed three morphologically distinct components: adenocarcinoma in the lower rectum, squamous intraepithelial neoplasia with a minor invasive squamous cell carcinoma component in the anal canal, and pagetoid spread of adenocarcinoma extending to the perianal skin. HPV18 DNA and diffuse p16 expression were detected in all three components, suggesting the integration of HPV and a histogenetic relationship among these morphologically distinct components. This case indicates that HPV-associated adenosquamous carcinoma also occurs in the anal canal, similar to the uterine cervix, and may present as secondary Paget's disease.</p>","PeriodicalId":19806,"journal":{"name":"Pathology International","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-05-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143973353","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A Prominent Trabecular/Nested Growth Pattern in Mucoepidermoid Carcinoma May be Formed Primarily by the Fourth Cell Type, Namely Cells With a Columnar Cell Lineage.","authors":"Shinnichi Sakamoto, Fumio Ide, Miyako Hoshino, Michiko Nishimura, Kentaro Kikuchi","doi":"10.1111/pin.70016","DOIUrl":"https://doi.org/10.1111/pin.70016","url":null,"abstract":"","PeriodicalId":19806,"journal":{"name":"Pathology International","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-04-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143974814","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A Potential Case of Tubulocystic Ductal Carcinoma of the Pancreas.","authors":"Rena Uno, Yoh Zen, Tomonori Tanaka, Hirochika Toyama, Takumi Fukumoto, Keitaro Sofue, Tomoo Itoh","doi":"10.1111/pin.70008","DOIUrl":"https://doi.org/10.1111/pin.70008","url":null,"abstract":"<p><p>We present a case of pancreatic ductal carcinoma with a microcystic appearance. A 64-year-old woman was found to have a pancreatic mass during a routine medical checkup. The tumor was well-circumscribed and multicystic; thus, she was followed up for suspected serous cystadenoma. However, the tumor gradually enlarged in the following 2.5 years; subsequently, she underwent a Whipple procedure. Grossly, the cut surface of the tumor was honeycomb-like with small cysts. Histologically, the cysts were ductal structures lined by a relatively bland, cuboidal or columnar epithelium with mildly enlarged nuclei. No intracytoplasmic mucus was observed. The presence of stromal invasion confirmed the diagnosis of ductal carcinoma. KRAS was wild type. Postoperative course was uneventful, with no recurrence to date (followup period: 5.5 years postsurgery). The present case did not meet any known subtypes of pancreatic ductal carcinoma. The tumor resembled a large duct variant, which typically shows a microcystic appearance. However, unlike the present case, the large duct type usually consists of mucus-rich neoplastic cells. A recent study on cholangiocarcinoma proposed a novel tubulocystic subtype characterized by microcystic neoplastic glands and adenofibromatous stroma, which is morphologically similar to the present case. The present case may correspond to a pancreatic counterpart of tubulocystic cholangiocarcinoma.</p>","PeriodicalId":19806,"journal":{"name":"Pathology International","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-04-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144006940","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}