{"title":"Response to Expanding Insights Into PRAME Expression in Merkel Cell Carcinoma.","authors":"Shyam S Raghavan","doi":"10.1097/PAS.0000000000002410","DOIUrl":"https://doi.org/10.1097/PAS.0000000000002410","url":null,"abstract":"","PeriodicalId":7772,"journal":{"name":"American Journal of Surgical Pathology","volume":"49 6","pages":"637-638"},"PeriodicalIF":4.5,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143962298","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Steven Christopher Smith, Julio A Diaz-Perez, Mark Cameron Mochel, Steven D Billings, Leopoldo Fernandez, Andrew S Poklepovic
{"title":"A High-grade PML::JAK1 Fusion Sarcoma.","authors":"Steven Christopher Smith, Julio A Diaz-Perez, Mark Cameron Mochel, Steven D Billings, Leopoldo Fernandez, Andrew S Poklepovic","doi":"10.1097/PAS.0000000000002326","DOIUrl":"10.1097/PAS.0000000000002326","url":null,"abstract":"","PeriodicalId":7772,"journal":{"name":"American Journal of Surgical Pathology","volume":" ","pages":"633-635"},"PeriodicalIF":4.5,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142567796","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ivan J Stojanov, Kelly Yi Ping Liu, Christina McCord, Julia Yu-Fong Chang, Yi-Ping Wang, Chia-Cheng Li, Lingxin Zhang, Victoria L Woo, Elizabeth M Philipone, Paras B Patel, Kelly R Magliocca, Iona Leong, Hemlata Shirsat, Vincent Cracolici, Christopher C Griffith, William H Westra, Emilija Todorovic, Elizabeth A Bilodeau, William C Faquin, Lynn N Hoang, Ilena S Yim, Natyra Haxhiavdija, Martial Guillaud, Brandon M Veremis, Yen Chen Kevin Ko
{"title":"Consensus in Oral Epithelial Dysplasia Classification: A Comparative Analysis of H&E-stained Sections With and Without p53/p16 Immunohistochemistry.","authors":"Ivan J Stojanov, Kelly Yi Ping Liu, Christina McCord, Julia Yu-Fong Chang, Yi-Ping Wang, Chia-Cheng Li, Lingxin Zhang, Victoria L Woo, Elizabeth M Philipone, Paras B Patel, Kelly R Magliocca, Iona Leong, Hemlata Shirsat, Vincent Cracolici, Christopher C Griffith, William H Westra, Emilija Todorovic, Elizabeth A Bilodeau, William C Faquin, Lynn N Hoang, Ilena S Yim, Natyra Haxhiavdija, Martial Guillaud, Brandon M Veremis, Yen Chen Kevin Ko","doi":"10.1097/PAS.0000000000002385","DOIUrl":"10.1097/PAS.0000000000002385","url":null,"abstract":"<p><p>Diagnosis and classification of oral epithelial dysplasia (OED) is critical to identifying and prognosticating patients at risk of squamous cell carcinoma (SCC). However, conventional 3-tiered and 2-tiered grading systems suffer from poor inter-pathologist agreement, and SCC may arise from all grades of OED. This study evaluated pathologist agreement in OED classification as p53 wildtype, p53 abnormal, and HPV-associated based on recent evidence demonstrating the utility of p53/p16 immunohistochemistry (IHC) in this setting and increased risk of p53 abnormal OED progression to SCC, regardless of histologic grade. Fifty digital biopsy specimens were evaluated for diagnosis by 18 subspecialty-trained pathologists, with OED graded utilizing 3-tiered, 2-tiered, and p53 wildtype/p53 abnormal/HPV-associated schemata. Cases were reviewed first without and subsequently with p53/p16 IHC. The cohort consisted of 8 cases of p53 wildtype, 24 cases of p53 abnormal, and 18 cases of HPV-associated OED. Inter-pathologist agreement in OED grading according to 3-tiered (κ=0.32) and 2-tiered (κ=0.39) systems by H&E was poor, but fair-to-good (κ=0.59) in classification as p53 wildtype/p53 abnormal/HPV-associated by H&E and IHC. Classification of OED as p53 wildtype, p53 abnormal, or HPV-associated using p53/p16 IHC outperformed conventional grading in this cohort enriched for p53 abnormal OED, which required correct interpretation of p53 IHC, historically deemed challenging. Routine use of IHC also identifies a wider histologic spectrum of HPV-associated OED than is currently appreciated. More work is needed to determine the efficacy of this classification system in predicting patient outcomes and in guiding management decisions in real-world cohorts.</p>","PeriodicalId":7772,"journal":{"name":"American Journal of Surgical Pathology","volume":" ","pages":"601-609"},"PeriodicalIF":4.5,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143810340","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Andrew P Norgan, Elizabeth Ann L Enninga, Bohdana Fedyshyn, Matthew Wolf, Jeffery A Goldstein, Elisheva D Shanes
{"title":"Enterovirus Placentitis is an Underrecognized Cause of Placental Pathology.","authors":"Andrew P Norgan, Elizabeth Ann L Enninga, Bohdana Fedyshyn, Matthew Wolf, Jeffery A Goldstein, Elisheva D Shanes","doi":"10.1097/PAS.0000000000002378","DOIUrl":"10.1097/PAS.0000000000002378","url":null,"abstract":"<p><p>The placenta is susceptible to infection by a number of viral pathogens, including severe acute respiratory syndrome coronavirus 2, which is associated with poor fetal outcomes. The histologic pattern of injury, termed severe acute respiratory syndrome coronavirus 2 placentitis, is characterized by a triad of increased perivillous fibrin deposition, intervillous histiocytes, and trophoblast necrosis. While the etiology of massive perivillous fibrin deposition (MPVFD) is mostly unknown, previous case reports of MPVFD in association with maternal Enterovirus (ENT) suggest that a subset of these cases are a consequence of undiagnosed viral infection. We evaluated 46 placentas collected between 2011 and 2022 with a diagnosis of MPVFD (n = 41) or chronic histiocytic intervillositis (CHI; n = 4). Combining methods of pan-viral metagenomic sequencing and targeted viral PCR, we detected Enterovirus DNA in 8 of 45 (18%) MPVFD and/or CHI cases. Seven of these positive cases were from MPVFD, and 1 was associated with a CHI diagnosis. Enterovirus A species (n = 7) were commonly identified, whereas one case had Enterovirus B. Histologic evaluation of these cases, including immunohistochemical staining for CD68, demonstrated increased intervillous histiocytes in Enterovirus -positive MPVFD cases in comparison with Enterovirus- negative, as well as evidence of trophoblast necrosis. Thus, we favor the terminology Enterovirus placentitis to describe this pathology. Overall, these findings suggest that Enterovirus is an underrecognized etiology of histologic MPVFD and, possibly, CHI. Further study to evaluate the recurrence risk of Enterovirus placentitis in comparison to MPVFD may help inform future fertility planning in patients with these diagnoses.</p>","PeriodicalId":7772,"journal":{"name":"American Journal of Surgical Pathology","volume":" ","pages":"594-600"},"PeriodicalIF":4.5,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143646793","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Grace Neville, Kyle M Devins, Marisa R Nucci, Jaclyn C Watkins
{"title":"Somatic Malignancies Arising in Ovarian Mature Cystic Teratomas: A Multi-Institutional Study of 40 Cases Highlighting Outcomes and Novel Malignancies.","authors":"Grace Neville, Kyle M Devins, Marisa R Nucci, Jaclyn C Watkins","doi":"10.1097/PAS.0000000000002384","DOIUrl":"10.1097/PAS.0000000000002384","url":null,"abstract":"<p><p>Somatic malignancy arising in ovarian mature cystic teratoma (MCT) is a relatively rare phenomenon with an estimated incidence ranging from 0.17% to 5.5%. Most previous studies have been limited by small sample sizes, hindering more precise estimates of incidence as well as providing limited prognostic information. We aimed to conduct a large-scale, multi-institutional study to better define incidence, discuss prognosis, and report occurrences of unusual malignancies arising in MCT. The pathology archives of the Massachusetts General Hospital and Brigham and Women's Hospital were searched for all cases of MCT arising between 2006 and 2021. The pathology reports were reviewed for the presence of somatic malignancy arising within MCT. Cases harboring somatic malignancy were re-reviewed by a gynecologic pathologist, with documentation of a number of histomorphologic variables, including surface involvement, lymphovascular invasion, and tumor size. Sociodemographic variables, adjuvant chemotherapy, disease recurrence/progression, and survival were extrapolated from the medical record. Among 2416 cases of MCT, 40 cases of somatic malignancy were identified. Tumors included squamous cell carcinoma (SCC, n=21), papillary thyroid carcinoma (PTC, n=7), sebaceous carcinoma (n=2), neuroendocrine carcinoma (n=2), and other rarer types. The mean age of patients was 49 years (range: 17.7 to 69.7 y). Follow-up data was available for 20 patients (range: 3 to 196 mo, mean: 80.5 mo). Eleven were ovarian confined without surface involvement; 9 were AJCC stage pT1C or higher at the time of diagnosis. Of ovarian confined tumors without surface involvement, only 1 recurred (a follicular variant of papillary thyroid carcinoma) with bone metastases found 72 months after initial diagnosis. Four additional cases, all of which were stage 1C or higher at initial diagnosis, recurred after initial resection, including 2 cases with SCC, 1 melanoma case, and 1 adenocarcinoma ex-Goblet cell carcinoid case. Tumors that recurred tended to have a large malignant component (range: 4 to 23 cm, mean: 16.8 cm). When cases received in consultation were excluded, the overall incidence of incidental somatic malignancy arising in MCT was 0.54% (13 of 2389 cases). Somatic malignancy in MCT is rare, and outcomes largely depend on the stage at initial diagnosis, and possibly, the size of the malignant portion of the tumor. Poor outcomes were noted across multiple histologies. Patients diagnosed with early-stage disease (stage IA) generally had a favorable prognosis, whereas those with advanced-stage disease (stage IC or higher) faced higher risks of recurrence and mortality. Nevertheless, some low-stage patients experienced recurrence, highlighting the need for long-term follow-up for all patients. More aggressive management strategies should be tailored on a case-by-case basis. The focality of residual MCT, in some cases, underscores the need for a thorough sampling of ovarian somatic ","PeriodicalId":7772,"journal":{"name":"American Journal of Surgical Pathology","volume":" ","pages":"527-538"},"PeriodicalIF":4.5,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143603111","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Carina A Dehner, George Jour, Maximilian Gassenmaier, Michael Michal, Nicolas de Saint Aubain, David J Papke, Brandon Umphress, Aofei Li, Mark M Tanner, Eduardo Calonje, Thomas Brenn, Christopher D M Fletcher, Thomas Mentzel, Klaus Busam, Konstantinos Linos
{"title":"Cutaneous Hemangioma With Epithelioid Features Harboring TPM3/4::ALK Fusions : A Distinct Entity or a Molecular Variant of Epithelioid Hemangioma?","authors":"Carina A Dehner, George Jour, Maximilian Gassenmaier, Michael Michal, Nicolas de Saint Aubain, David J Papke, Brandon Umphress, Aofei Li, Mark M Tanner, Eduardo Calonje, Thomas Brenn, Christopher D M Fletcher, Thomas Mentzel, Klaus Busam, Konstantinos Linos","doi":"10.1097/PAS.0000000000002380","DOIUrl":"10.1097/PAS.0000000000002380","url":null,"abstract":"<p><p>Vascular neoplasms with epithelioid cytomorphology encompass a wide spectrum of benign and malignant lesions, including epithelioid hemangioma (EH), cutaneous epithelioid angiomatous nodule (CEAN), epithelioid hemangioendothelioma (EHE), and epithelioid angiosarcoma (EAS). Recently, the first case of a cutaneous hemangioma with epithelioid features harboring a TPM3::ALK fusion was reported. Herein, we report 4 additional cases, including 1 case with an alternate TPM4::ALK fusion, and expand on the clinicopathologic and molecular genetic features of these unusual vascular lesions. Including the previously reported case, 5 tumors occurred in 4 male and 1 female patients with a median age of 14 years (range: 2 to 38 y) and involved the shoulder region (2), the lower extremity (1), trunk (1), and head and neck (1). Clinical follow-up (3 patients; 60%) showed no evidence of disease at the last follow-up (median: 5 mo; range: 1 to 16 mo). Histologically, all tumors showed highly similar morphologic features, including an epidermal collarette, well-formed vascular channels composed of epithelioid endothelial cells with intracytoplasmic vacuoles, and admixed inflammatory cells. Immunohistochemically, all tumors were positive for vascular markers such as ERG and CD31, along with strong and diffuse cytoplasmic expression of ALK. RNA sequencing revealed recurrent TPM3 exon 8 :: ALK exon 20 (4) and TPM4 exon 7 :: ALK exon 20 fusions (1). We conclude that cutaneous hemangiomas with epithelioid features harboring TPM3/4::ALK fusions show consistent morphologic, immunophenotypic, and molecular genetic features. It remains to be determined whether this neoplasm represents a distinct entity or a molecular variant of epithelioid hemangioma.</p>","PeriodicalId":7772,"journal":{"name":"American Journal of Surgical Pathology","volume":" ","pages":"610-619"},"PeriodicalIF":4.5,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143603597","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pedram Argani, Sintawat Wangsiricharoen, Maria Tretiakova, Yajuan J Liu, Sara M Falzarano, Katrina Collins, Fadi Brimo, John M Gross, Ezra Baraban, Andres Matoso, Kristina Wakeman, Christopher Corless, Tanaya Neff, Benjamin F Smith, Ali Abdel Satir, Abbas Agaimy, Cristina R Antonescu, Gregory W Charville, Ankur R Sangoi
{"title":"MEIS1::NCOA1 Primitive Spindle Cell Sarcoma of the Kidney : Report of 7 Cases of a Distinctive Clinicopathologic Entity.","authors":"Pedram Argani, Sintawat Wangsiricharoen, Maria Tretiakova, Yajuan J Liu, Sara M Falzarano, Katrina Collins, Fadi Brimo, John M Gross, Ezra Baraban, Andres Matoso, Kristina Wakeman, Christopher Corless, Tanaya Neff, Benjamin F Smith, Ali Abdel Satir, Abbas Agaimy, Cristina R Antonescu, Gregory W Charville, Ankur R Sangoi","doi":"10.1097/PAS.0000000000002386","DOIUrl":"10.1097/PAS.0000000000002386","url":null,"abstract":"<p><p>Primitive sarcomas harboring the MEIS1::NCOA2 gene fusion were originally described in the kidney in 2018, and subsequently reported in other organs. These variably cellular neoplasms feature monomorphic primitive plump spindle cells forming nodules and whorls in addition to nondescript fascicular, solid, and storiform patterns. They lack skeletal muscle differentiation in contrast to the primarily intraosseous rhabdomyosarcomas that harbor the same gene fusion. We describe 7 new primary primitive renal sarcomas with MEIS1::NCOA1 gene fusions. Although their morphology overlaps with that described in MEIS1::NCOA2 renal sarcoma, 3 of the 7 cases contained adipose tissue. The majority had intimately admixed entrapped cystic epithelial elements and demonstrated patchy immunoreactivity for estrogen receptor and nuclear labeling for WT1 protein, leading to the differential diagnosis of malignant mixed epithelial stromal tumor (MEST) in 4 cases and metanephric stromal tumor in one. The neoplasms demonstrate a broad spectrum of clinicopathologic features ranging from a bland low-grade neoplasm that metastasized 9 years after diagnosis to a high-grade sarcoma with multiple recurrences, ultimately leading to patient death in under 1 year. In summary, MEIS1::NCOA1 primitive sarcomas overlap with the previously described MEIS1::NCOA2 primitive renal sarcomas and represent a distinctive renal neoplasm that can be mistaken for malignant MEST. Grade ranges from low to high but even low-grade neoplasms require long-term clinical follow-up.</p>","PeriodicalId":7772,"journal":{"name":"American Journal of Surgical Pathology","volume":" ","pages":"620-632"},"PeriodicalIF":4.5,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143750685","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Wipawi Klaisuban, Chuying Su, Matthew Koslow, Jay H Ryu, Henry D Tazelaar, Sarah M Jenkins, Eunhee S Yi
{"title":"Revisit of Histopathology in Rheumatoid Lung Nodules.","authors":"Wipawi Klaisuban, Chuying Su, Matthew Koslow, Jay H Ryu, Henry D Tazelaar, Sarah M Jenkins, Eunhee S Yi","doi":"10.1097/PAS.0000000000002374","DOIUrl":"10.1097/PAS.0000000000002374","url":null,"abstract":"<p><p>Diagnosing pulmonary rheumatoid nodules (RN) is challenging because they are rare and share clinical, radiologic, and histopathologic features with other necrotizing granulomatous diseases such as infectious granulomas (IG) and granulomatosis with polyangiitis (GPA). Herein, we revisit the histopathologic features of RN and the findings in the adjacent lung tissue, in comparison with IG and GPA, to identify distinguishing features. Twenty-eight cases with surgically resected RN evaluated at our institution (1991 to 2024), 33 IG (10 mycobacterial infection, 10 histoplasmosis, 13 coccidiomycosis), and 10 GPA cases were included in the study. All available slides with H&E and special stains were reviewed for various histologic parameters within the nodules and in surrounding lung tissue. Many histopathologic features of RN overlap with the necrotizing granulomas seen in infections and GPA. However, some findings in the adjacent lung tissue showed significant differences. The lack of airspace granulomas/non-necrotizing granuloma and the absence of necrotizing vasculitis in the setting of RN can help to differentiate between infection cases and GPA, respectively. Given their considerable overlap, correlation with clinical context, serologic and microbiologic studies, as well as careful evaluation of special stains is crucial in the diagnosis of RN.</p>","PeriodicalId":7772,"journal":{"name":"American Journal of Surgical Pathology","volume":" ","pages":"588-593"},"PeriodicalIF":4.5,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143673164","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Rajal B Shah, Gladell P Paner, Liang Cheng, Angelo M De Marzo, Cristina Magi-Galluzzi, Murali Varma, Ming Zhou, Ali Amin, Mahul B Amin, Manju Aron, Isabela W Cunha, Jonathan I Epstein, Samson W Fine, Aiman Haider, Kenneth A Iczkowski, James G Kench, Lakshmi P Kunju, Sambit K Mohanty, Rodolfo Montironi, George J Netto, Chin-Chen Pan, Priya Rao, John R Srigley, Guido Sauter, Puay Hoon Tan, Toyonori Tsuzuki, Theodorus H van der Kwast, Geert J van Leenders, Glen Kristiansen
{"title":"Genitourinary Pathology Society and International Society of Urological Pathology White Paper on Defining Indolent Prostate Cancer.","authors":"Rajal B Shah, Gladell P Paner, Liang Cheng, Angelo M De Marzo, Cristina Magi-Galluzzi, Murali Varma, Ming Zhou, Ali Amin, Mahul B Amin, Manju Aron, Isabela W Cunha, Jonathan I Epstein, Samson W Fine, Aiman Haider, Kenneth A Iczkowski, James G Kench, Lakshmi P Kunju, Sambit K Mohanty, Rodolfo Montironi, George J Netto, Chin-Chen Pan, Priya Rao, John R Srigley, Guido Sauter, Puay Hoon Tan, Toyonori Tsuzuki, Theodorus H van der Kwast, Geert J van Leenders, Glen Kristiansen","doi":"10.1097/PAS.0000000000002425","DOIUrl":"https://doi.org/10.1097/PAS.0000000000002425","url":null,"abstract":"<p><p>A significant subset of well-differentiated prostatic acinar neoplasms with invasive histologic features will not spread outside of the prostate, become symptomatic, or shorten a patient's life even if the tumor is left untreated. Overdiagnosis and overtreatment of these indolent prostate cancers (PCa) remain a significant health care problem despite the improved risk assessment and uptake in acceptance of conservative management. While detection of indolent PCa on an entirely resected prostate is possible, recognition of indolent PCa on a needle biopsy (NBX) cannot be reliably made as Grade Group 1 (GG1) PCa diagnosis on NBX is not always identical to one from radical prostatectomy due to a variety of reasons. Further, some of the initially diagnosed GG1 PCas on NBX and carefully monitored on active surveillance (AS) are later reclassified with higher grades. At the same time, other GG1 PCas never progressed on long-term follow-up while receiving no therapy. The overarching goal of this white paper by the 2 leading uropathology organizations, Genitourinary Pathology Society (GUPS) and International Society of Urological Pathology (ISUP), is to help identify a path toward a more meaningful multidisciplinary solution addressing the pervasive problem of overdiagnosis of indolent PCa and its downstream negative effects. Herein, GUPS and ISUP jointly release statements that address why recognition of indolent PCa cannot be reliably made in NBX and why various contemporary multidisciplinary approaches are needed to help improve the detection of indolent PCa in NBX.</p>","PeriodicalId":7772,"journal":{"name":"American Journal of Surgical Pathology","volume":" ","pages":""},"PeriodicalIF":4.5,"publicationDate":"2025-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144186266","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kyle M Devins, Rachelle P Mendoza, Maryam Shahi, Mariachristina Ghioni, Rofieda Alwaqfi, Sabrina Croce, Anna Pesci, Joana Ferreira, Ana Felix, Iñigo Espinosa, Damiano Arciuolo, Gian F Zannoni, Esther Oliva
{"title":"Low-Grade Endometrial Stromal Sarcoma: Clinicopathologic and Prognostic Features in a Cohort of 102 Tumors.","authors":"Kyle M Devins, Rachelle P Mendoza, Maryam Shahi, Mariachristina Ghioni, Rofieda Alwaqfi, Sabrina Croce, Anna Pesci, Joana Ferreira, Ana Felix, Iñigo Espinosa, Damiano Arciuolo, Gian F Zannoni, Esther Oliva","doi":"10.1097/PAS.0000000000002428","DOIUrl":"https://doi.org/10.1097/PAS.0000000000002428","url":null,"abstract":"<p><p>Low-grade endometrial stromal sarcomas (LG-ESS) are the second most common malignant uterine mesenchymal tumors, but in contrast to the more common leiomyosarcomas, they are often characterized by a prolonged and relatively indolent course. However, a subset of patients experience significant morbidity or die of disease, and it is difficult to predict which tumors will behave aggressively, with most published studies limited in either the number of tumors or the depth of pathologic parameters evaluated. Thus, we studied the clinicopathologic features of LG-ESS in 102 patients ranging from 21 to 74 (median: 47) years. All were treated with hysterectomy and staged according to both the FIGO 2018 system (stage IA=22, IB=36, I-not otherwise specified=5, II=16, III=13, IV=10) and the FIGO 1988 system (stage I=62, II=1, III=17, IV=22). Tumors measured 1.2-49 (median: 7) cm. Microscopically, 69 involved the endometrium while 33 were centered in the myometrium. Thirteen showed only minimal infiltration of the myometrium while the rest displayed the typical extensive myometrial permeation. The cervical stroma was involved in 18, the uterine serosa in 27, and the parametrium in 22. Conventional morphology resembling proliferative endometrial stroma was seen in 95, fibroblastic appearance in 35, smooth muscle differentiation in 23, sex cord-like differentiation in 21, stromal hyalinization in 21, and myxoid stroma in 9. Less common features included glandular differentiation resembling adenomyosis (n=5), pseudopapillary pattern (n=1), deciduoid appearance (n=2), adipocytic differentiation (n=2), multinucleated cells (n=2), and rhabdomyoblastic differentiation (n=1). Mitoses ranged from <1 to 20 per 10 high-power fields (median=3). Lymphovascular invasion and infarct-type necrosis were present in 64 and 23, respectively. Follow-up was available in all patients ranging from 16 to 358 (median: 79) months. Forty-six received adjuvant treatment as hormonal therapy (n=34), radiation (n=4), radiation and hormonal therapy (n=4), chemotherapy (n=3), or chemotherapy and radiation (n=1). Three patients had persistent unresected tumor following surgery, and an additional 34 had recurrences at intervals of 3 to 272 (median: 79) months, including 2 tumors with minimal infiltration. At last follow-up, 75 patients were alive with no evidence of disease, 14 were alive with disease, and 9 died of disease at intervals of 16 to 167 (median=70) months. Four died of unrelated causes without recurrence. Five-year recurrence-free survival (RFS) and disease-specific survival (DSS) were 80% and 94%, while 10-year RFS and DSS were 51% and 87%, respectively. On statistical analysis, cervical stromal involvement (P=0.018) and myxoid stroma (P<0.001) were associated with shorter recurrence-free survival. Tumors lacking a conventional component had worse disease-specific survival (P=0.048). All other clinical and morphologic features, including stage, were not significantly associa","PeriodicalId":7772,"journal":{"name":"American Journal of Surgical Pathology","volume":" ","pages":""},"PeriodicalIF":4.5,"publicationDate":"2025-05-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144155568","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}