Annals of Diagnostic Pathology最新文献

{"title":"Clinicopathological features of SMARCB1/INI1-deficient medullary-like renal cell carcinoma: Report of 3 cases and literature review","authors":"Wenwen Luo ,&nbsp;Yaqi Zheng ,&nbsp;Wenqing Huang ,&nbsp;Ye Jiang ,&nbsp;Bojin Su ,&nbsp;Nana Zhang","doi":"10.1016/j.anndiagpath.2026.152621","DOIUrl":"10.1016/j.anndiagpath.2026.152621","url":null,"abstract":"<div><div>SMARCB1/INI1-deficient medullary-like renal cell carcinoma (MLRCC), also used to be known as unclassified renal cell carcinoma with medullary phenotype (RCCU-MP), is an extremely rare and highly aggressive kidney tumor. The 2022 World Health Organization (WHO) classification proposes that high-grade renal cell carcinomas exhibiting morphological and immunophenotypic features identical to renal medullary carcinoma, but without evidence of sickle cell trait or disease, should be diagnosed as SMARCB1/INI1-deficient medullary-like renal cell carcinoma(MLRCC) and classified as a subtype of SMARCB1-deficient renal medullary carcinoma (RMC). To date, only 15 cases of MLRCC have been reported in the literature, primarily as case reports. This study presents three additional cases of MLRCC, all without a history of hemoglobinopathy or relevant family history. Histopathologically, all tumors exhibited nested, cord-like, and reticular/microcystic growth patterns that closely resemble those of classical renal medullary carcinoma, yet differ in epidemiological characteristics such as age and ethnic distribution. The tumor cells showed significant atypia with high-grade nuclear features and one case displayed focal rhabdoid morphology. Associated findings included necrosis, extensive stromal fibrosis, myxoid change, and a prominent inflammatory infiltrate with aggregates. Immunohistochemically, all three cases were positive for CK and Vimentin, and showed a complete loss of SMARCB1/INI1 protein expression. Currently, no standard treatment regimen has been established for this disease. Given its highly aggressive nature, early and accurate diagnosis is crucial. Pathologists must integrate clinical history, morphological characteristics, immunophenotype for a comprehensive diagnosis.</div></div>","PeriodicalId":50768,"journal":{"name":"Annals of Diagnostic Pathology","volume":"82 ","pages":"Article 152621"},"PeriodicalIF":1.4,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146159182","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":"Percutaneous renal mass biopsies with no viable lesional cells – Recognizing different histologic patterns can help predict nondiagnostic vs. true negative biopsy and guide clinical management","authors":"Anisha Jacob,&nbsp;Richard Simon,&nbsp;Tatjana Antic,&nbsp;Jung Woo Kwon","doi":"10.1016/j.anndiagpath.2026.152607","DOIUrl":"10.1016/j.anndiagpath.2026.152607","url":null,"abstract":"<div><div>Percutaneous renal mass biopsy (RMB) is increasingly used for diagnosis and management of renal masses. Nondiagnostic RMBs are not uncommon, with large series reporting nondiagnostic RMB rates to be 10%–21%, and they can trigger challenging clinical scenarios and confusions for clinicians. Therefore, this study aims to evaluate whether specific histologic patterns seen in RMBs without lesional cells are associated with clinical outcomes and whether such findings could help guide the management. 539 consecutive RMBs performed between 2005 and 2024 were retrospectively identified in this study. Out of the 539 cases, sixty-four (12%) were nondiagnostic, defined as no viable tumor cells. Six of the sixty-four cases were excluded (5 cases being repeat biopsies of the previous nondiagnostic biopsies; 1 case lost to follow up). Histological patterns were categorized, and clinical outcomes were assessed via electronic medical records. Upon review, seven histological patterns were identified. Patterns 1–4 (benign parenchyma, chronic changes, tumor necrosis, hyalinized stroma) were associated with malignancy in 31/42 cases (74%). Patterns 5–7 (xanthogranulomatous pyelonephritis, crescentic glomerulonephritis, blood clots/macrophages/fibrosis/amorphous material) were associated with malignancy in only 2/16 cases (12.5%). There was a statistically significant difference by chi-square test (p &lt; 0.000005) between Patterns 1–4 and Patterns 5–7. In summary, certain histological features in nondiagnostic RMBs can help predict nondiagnostic vs. true negative biopsy and guide subsequent clinical decision-making. Recognition of these histologic patterns could aid pathologists in appropriately classifying nondiagnostic vs. true negative RMBs, improving patient management, and potentially reducing the risk of missed malignancy.</div></div>","PeriodicalId":50768,"journal":{"name":"Annals of Diagnostic Pathology","volume":"82 ","pages":"Article 152607"},"PeriodicalIF":1.4,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145929056","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":"Pulmonary intravascular mononuclear cell accumulation in cases of mechanical asphyxia due to external neck compression","authors":"Anna Laura Santunione ,&nbsp;Jessika Camatti ,&nbsp;Silvia Corradi ,&nbsp;Enrico Silingardi ,&nbsp;Rossana Cecchi","doi":"10.1016/j.anndiagpath.2025.152600","DOIUrl":"10.1016/j.anndiagpath.2025.152600","url":null,"abstract":"<div><div>Pulmonary intravascular mononuclear cell accumulations have been described in mechanical asphyxia, but their diagnostic value and independence from demographic or post-mortem factors remain uncertain. This study assessed the frequency of these accumulations in asphyxial deaths due to external neck compression compared with non-asphyxial deaths, and evaluated whether the association persists after adjustment for potential confounders.</div><div>Lung tissue samples from 31 external neck-compression deaths and 151 non-asphyxial controls were examined histologically. A subset underwent immunohistochemical phenotyping. Univariable comparisons were performed using χ² and Mann–Whitney <em>U</em> tests. A multivariable logistic regression model—including age, sex, post-mortem interval (PMI), and body mass index (BMI)—was used to evaluate the independence of the association.</div><div>Intravascular mononuclear accumulations were observed in 41.9 % of neck-compression deaths versus 17.2 % of controls (<em>p</em> &lt; 0.01; unadjusted OR 3.47, 95 % CI 1.52–7.96). In the multivariable model, external neck compression remained independently associated with the presence of intravascular accumulations (adjusted OR 2.93, 95 % CI 1.14–7.52; <em>p</em> = 0.025), while age, sex, PMI, and BMI showed no significant effect. Immunohistochemistry confirmed that accumulations consisted of mature mononuclear cell subsets.</div><div>Pulmonary intravascular mononuclear accumulations occur significantly more often in asphyxial deaths involving external neck compression, and this association persists after adjustment for key demographic and post-mortem variables. Although not specific to mechanical asphyxia, these accumulations represent a practical ancillary marker that may support the diagnosis of neck-compression vitality, especially in cases with limited external findings.</div></div>","PeriodicalId":50768,"journal":{"name":"Annals of Diagnostic Pathology","volume":"81 ","pages":"Article 152600"},"PeriodicalIF":1.4,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145790135","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":"Clinicopathological characterization of gastric amphicrine carcinoma: A case series","authors":"Dazhou Li ,&nbsp;Xiaodong Lin ,&nbsp;Wenqing Huang","doi":"10.1016/j.anndiagpath.2025.152584","DOIUrl":"10.1016/j.anndiagpath.2025.152584","url":null,"abstract":"<div><div>Gastric amphicrine carcinoma (GAC) is a rare malignancy exhibiting dual neuroendocrine and exocrine differentiation. Claudin 18.2 expression patterns in GAC remain poorly characterized. We retrospectively analyzed four GAC cases diagnosed between 2020 and 2024 were retrospectively analyzed. Diagnosis required expression of at least two neuroendocrine markers and intracellular mucin, with amphicrine components comprising &gt;30 % of tumor. Immunohistochemical evaluation included neuroendocrine markers, HER2, and Claudin 18.2. All patients were males aged 52–72 years (mean: 61.5 years). Tumors were located in gastric antrum (<em>n</em> = 2), cardia (<em>n</em> = 1), and body (n = 1), ranging from 4.0 to 4.5 cm. Three morphological patterns were identified: sheet-like/nested, tubular, and signet-ring trabecular. All cases expressed synaptophysin, with variable expression of chromogranin A (3/4), INSM1 (2/4), and CD56 (3/4). HER2 was negative in all cases. Claudin 18.2 expression correlated with morphological patterns: moderate to strong staining in sheet-like/nested areas (3/4) and tubular structures (3/4), but absent in signet-ring trabecular components (2/4). Follow-up demonstrated hepatic metastasis (<em>n</em> = 1) and death (n = 1) at 6 months, while two patients remained alive without recurrence. GAC may demonstrate distinctive morphology-dependent Claudin 18.2 expression, suggesting potential implications for targeted therapy selection and molecular subtyping. The morphological heterogeneity and aggressive clinical behavior underscore the importance of accurate diagnosis through comprehensive integrated assessment.</div></div>","PeriodicalId":50768,"journal":{"name":"Annals of Diagnostic Pathology","volume":"81 ","pages":"Article 152584"},"PeriodicalIF":1.4,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145617909","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":"Diagnostic value of dystrophin immunostaining for histopathologic diagnosis of uterine smooth muscle tumors","authors":"Fatemeh Nili ,&nbsp;Kiana Anousha ,&nbsp;Soheila Sarmadi ,&nbsp;Fereshteh Ameli","doi":"10.1016/j.anndiagpath.2025.152604","DOIUrl":"10.1016/j.anndiagpath.2025.152604","url":null,"abstract":"<div><div>Uterine smooth muscle tumors are a heterogeneous group of mesenchymal neoplasms with diagnostic challenges and overlapping histopathologic features. Recently, the molecular or immunohistochemical evaluation of dystrophin in the diagnosis of mesenchymal tumors with muscle differentiation has gained attention. In this retrospective study, the immunohistochemical expression of dystrophin was examined in 105 cases of uterine smooth muscle neoplasms, including 71 cases of leiomyoma (LM) and its variants, 6 cases of smooth muscle tumor of uncertain malignant potential (STUMP), and 28 cases of leiomyosarcoma (LMS). After thorough analysis, dystrophin expression was positive in 83.3 % of STUMP cases and 96.7 % of leiomyoma cases. In contrast, only 8 cases of LMS (28.6 %) expressed dystrophin. A significant difference in dystrophin expression was noted between STUMP and LMS, as well as LMS and LM and its variants. The median H-score in LM was significantly higher than in leiomyoma variants, STUMP, and LMS. In conclusion, dystrophin expression may be useful in distinguishing uterine LM, LM variants, and STUMP from LMS.</div></div>","PeriodicalId":50768,"journal":{"name":"Annals of Diagnostic Pathology","volume":"81 ","pages":"Article 152604"},"PeriodicalIF":1.4,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145883326","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":"Clinicopathological features of hepatic Langerhans cell histiocytosis: report of ten cases and review of the literature","authors":"Qian-Qian Chen ,&nbsp;Chun-kui Shao ,&nbsp;Yi-wang Zhang ,&nbsp;Jian-ning Chen ,&nbsp;Hai-feng Li ,&nbsp;Qiong Liang","doi":"10.1016/j.anndiagpath.2025.152586","DOIUrl":"10.1016/j.anndiagpath.2025.152586","url":null,"abstract":"<div><div>The aim was to evaluate the clinical and histopathological characteristics of hepatic Langerhans cell histiocytosis (LCH). Data from 10 patients with hepatic LCH were collected. The clinicopathological features and immunophenotypes of hepatic LCH were assessed. The study included 6 males and 4 females (median age: 20.5 years). Clinical manifestations and imaging findings were non-specific. Six cases had multisystem involvement, while 4 had isolated hepatic LCH. All lesions were located in the portal area. Histologically, Langerhans cells (LCs) with characteristic nuclear grooves infiltrated the bile duct epithelium. Although one case initially presented as sclerosing cholangitis (SC) without detectable LCs, the patient's history of cutaneous LCH provided a crucial diagnostic clue, and Langerhans cell (LC) foci were later confirmed in the explanted liver. Immunohistochemically, these tumor cells were positive for CD1a, S100, and CD207. BRAF V600E mutations were detected in 30 % (3/10) of cases. Five cases displayed unique morphological patterns: 2 exhibited sclerosing cholangitis, 1 resembled inflammatory myofibroblastic tumor (IMT) or EBV-positive inflammatory follicular dendritic cell sarcoma (EBV+ IFDCS), and 2 showed chronic suppurative cholangitis with abscess formation. Hepatic LCH exhibits diverse morphological features. Bile duct epithelial injury with eosinophil infiltration and a history of extrahepatic LCH are important diagnostic clues. Hematoxylin-eosin staining combined with immunohistochemistry (CD1a, S100, CD207) is essential for definitive diagnosis. LCH should be differentiated from hepatic parasitic infections, primary SC, EBV+ IFDCS, and IMT.</div></div>","PeriodicalId":50768,"journal":{"name":"Annals of Diagnostic Pathology","volume":"81 ","pages":"Article 152586"},"PeriodicalIF":1.4,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145566259","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":"Spread through air spaces (STAS) in lung adenocarcinoma: Prognostic impact of morphologic patterns, density, and extent","authors":"Sinem Eser Polat Ünal ,&nbsp;Canan Sadullahoğlu ,&nbsp;Hacer Boztepe Yeşilçay ,&nbsp;Şencan Akdağ","doi":"10.1016/j.anndiagpath.2025.152598","DOIUrl":"10.1016/j.anndiagpath.2025.152598","url":null,"abstract":"<div><div>Spread through air spaces (STAS) has emerged as a distinct invasion pattern in lung adenocarcinoma, but the prognostic implications of its morphologic features remain incompletely defined. In this study, we analyzed STAS presence, morphologic subtypes, density, and extent with clinicopathologic parameters and survival. We analyzed 184 surgically resected lung adenocarcinomas. We histopathologically examined the presence of STAS, STAS morphological subtypes (solid nests, micropapillary clusters, and single-cell spread), STAS extension, STAS density and evaluated the relationship of the findings with clinicopathological parameters. STAS was detected in 67.9 % of the tumors. The most common STAS subtype was solid (53.6 %), followed by micropapillary (30.4 %) and single-cell (16.0 %) subtypes. STAS density was categorized as low (1–4 tumor cell clusters) or high (≥5 clusters) at ×200 magnification, and STAS extent was classified as limited (≤3 alveolar spaces) or extensive (&gt;3 spaces). High-density STAS was observed in 56 %, low-density STAS in 44 %; limited-STAS in 57.6 %, and extensive-STAS in 42.4 %. STAS positivity was significantly associated with predominant tumor pattern (<em>p</em> = 0.002), tumor grade (<em>p</em> &lt; 0.001), pleural invasion (<em>p</em> = 0.004), lymphovascular invasion (LVI) (<em>p</em> &lt; 0.001), recurrence (p &lt; 0.001), metastasis (p &lt; 0.001), pN (<em>p</em> = 0.003), overall survival (OS) (p &lt; 0.001) and recurrence-free survival (DFS) (p &lt; 0.001). Among morphologic subtypes, significant correlations were found with predominant tumor pattern (p &lt; 0.001), grade (<em>p</em> = 0.001) and LVI (<em>p</em> = 0.005). We found STAS density to show a significant difference in OS (<em>p</em> = 0.009). Extensive STAS correlated with surgical resection type (<em>p</em> = 0.002), necrosis (<em>p</em> = 0.049), pN (<em>p</em> = 0.033), OS (<em>p</em> = 0.016) and DFS (<em>p</em> = 0.037). Our study shows that STAS and its features have prognostic significance with clinically meaningful differences in lung adenocarcinomas. These results highlight the potential clinical relevance of STAS subtype, density, and extent in risk stratification.</div></div>","PeriodicalId":50768,"journal":{"name":"Annals of Diagnostic Pathology","volume":"81 ","pages":"Article 152598"},"PeriodicalIF":1.4,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145571866","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":"“Letter to the editor: Comparative analysis of MIB1 and SP6 antibodies for Ki-67 assessment in breast carcinoma with focus on the influence of molecular subtyping”","authors":"Alishba Irfan","doi":"10.1016/j.anndiagpath.2025.152599","DOIUrl":"10.1016/j.anndiagpath.2025.152599","url":null,"abstract":"<div><div>This letter evaluates the study by Milev and Ivanov (2025) comparing MIB1 and SP6 antibodies for Ki-67 assessment in breast carcinoma and outlines key methodological limitations that weaken the reliability of their conclusions. The use of a single, non blinded pathologist without interobserver or intraobserver reproducibility assessment raises concerns about scoring subjectivity. The absence of formal agreement analyses, such as Bland Altman plots, further limits confidence in the reported concordance between the two clones. Additionally, the study lacks a predefined primary endpoint, sample size justification, and power calculation, reducing its interpretive strength. Addressing these issues would enhance reproducibility, analytical validity, and the translational relevance of Ki-67 evaluation.</div></div>","PeriodicalId":50768,"journal":{"name":"Annals of Diagnostic Pathology","volume":"81 ","pages":"Article 152599"},"PeriodicalIF":1.4,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145684727","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":"The diagnostic utility and frequency of CD56 expression in plasma cell myeloma","authors":"Midori Imai ,&nbsp;Asami Nishikori ,&nbsp;Tomoka Haratake ,&nbsp;Midori Filiz Nishimura ,&nbsp;Rio Yamada ,&nbsp;Syoma Kato ,&nbsp;Mizuha Tabe ,&nbsp;Hiroyuki Yanai ,&nbsp;Hidetaka Yamamoto ,&nbsp;Yasuharu Sato","doi":"10.1016/j.anndiagpath.2025.152587","DOIUrl":"10.1016/j.anndiagpath.2025.152587","url":null,"abstract":"<div><div>Plasma cell myeloma (PCM) is a hematological malignancy characterized by systemic proliferation of neoplastic plasma cells within the bone marrow. Diagnosis requires clinical findings and immunohistochemical staining, including CD138, CD79a, cyclin D1, immunoglobulin κ (Igκ), and λ (Igλ). However, CD79a and cyclin D1 have limited sensitivity and specificity, and Igκ/Igλ assessment is often difficult due to overstaining. Therefore, more reliable antibodies are needed to accurately diagnose PCM. In this study, we examined the diagnostic utility of CD56 expression in PCM. We retrospectively performed immunostaining for CD138, CD56, CD79a, cyclin D1, Igκ, and Igλ in bone marrow samples from 116 patients with PCM.</div><div>CD56 expression was observed in 85/116 cases (73.3 %), CD79a was downregulated in 46/116 cases (39.7 %), and cyclin D1 expression was observed in 42/116 cases (36.2 %). The expression of CD56 was significantly higher than that of CD79a and cyclin D1 (both <em>p</em> &lt; 0.001). The combination of two antibodies resulted in the highest detection rate when combining CD56 and CD79a (105/116, 90.5 %), which was significantly higher than the detection rates of CD56 and cyclin D1 (93/116, 80.2 %) and CD79a and cyclin D1 (75/116, 64.7 %) (both <em>p</em> &lt; 0.001). In contrast, lymphoplasmacytic lymphoma and marginal zone lymphoma lacked CD56 and cyclin D1 expression. Furthermore, in cases where light chain restriction was undetectable (11/116, 9.5 %), all could be diagnosed as PCM based on CD56, CD79a, and cyclin D1. Among these, CD56 showed the highest detection rate (8/11, 72.7 %).</div><div>These findings highlight CD56 as a helpful marker for PCM diagnosis and support further clinical research.</div></div>","PeriodicalId":50768,"journal":{"name":"Annals of Diagnostic Pathology","volume":"81 ","pages":"Article 152587"},"PeriodicalIF":1.4,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145532983","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":"From the archives of MD Anderson Cancer Center: Paraneoplastic autoimmune multiorgan syndrome (PAMS) associated with stroma-rich Castleman disease","authors":"Doaa Alqaidy ,&nbsp;Hong Fang ,&nbsp;Cesar A. Moran ,&nbsp;L. Jeffrey Medeiros ,&nbsp;Annikka Weissferdt","doi":"10.1016/j.anndiagpath.2025.152602","DOIUrl":"10.1016/j.anndiagpath.2025.152602","url":null,"abstract":"<div><div>Paraneoplastic autoimmune multiorgan syndrome (PAMS) is a rare, often fatal condition that occurs in association with a range of neoplasms, most commonly lymphoproliferative disorders. PAMS often presents as paraneoplastic pemphigus, an autoimmune bullous disease affecting the skin and mucosal surfaces and bronchiolitis obliterans, a small airway disease that can result in respiratory failure and death. We describe a 39-year-old woman with an incidentally discovered paratracheal mass that was followed clinically. Seven years later, she developed ulcerative oral lesions followed by progressive dyspnea. Biopsy of the oral lesions showed intraepithelial clefting and interface mucositis. Immunofluorescence and serologic studies revealed IgG and complement deposition within intercellular epithelial spaces and along the basement membrane zone, together with circulating anti-plakin antibodies, establishing a diagnosis of paraneoplastic pemphigus. Resection of the paratracheal mass revealed lymphoid tissue with follicular and interfollicular stromal changes, atretic germinal centers and thickened mantle zones, consistent with unicentric Castleman disease, stroma-rich hyaline vascular variant. Wedge resection of the lung showed focal bronchiolar scarring supporting the clinical impression of bronchiolitis obliterans. The patient was treated with steroids and rituximab and recently underwent bilateral lung transplantation due to progressive respiratory symptoms. This report underscores a rare and potentially life-threatening complication of Castleman disease and presents an updated review of the literature. Bronchiolitis obliterans, in particular, has been identified as a marker of poor prognosis in patients with PAMS and lung transplantation often represents the only viable treatment option once the underlying neoplasm has been controlled.</div></div>","PeriodicalId":50768,"journal":{"name":"Annals of Diagnostic Pathology","volume":"81 ","pages":"Article 152602"},"PeriodicalIF":1.4,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145883327","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}