Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society最新文献

{"title":"Novel Placental Functional Capacity Score: A Single Quantitative Score Estimating Placental Function and Its Association with Pregnancy Outcomes.","authors":"Linda M Ernst, Alexa Freedman, Sunitha Suresh, Andrew Franklin, Matthew Pellerite, Lauren Keenan-Devlin, Ann E B Borders, Gregory E Miller","doi":"10.1177/10935266261470991","DOIUrl":"https://doi.org/10.1177/10935266261470991","url":null,"abstract":"<p><strong>Background: </strong>Determining placental functional capacity from a typical pathology report can be difficult. Our goal was to create a new way to report on the functional capacity of the placenta, using much of the typical information obtained in a routine placental pathology assessment.</p><p><strong>Methods: </strong>Placental functional capacity score (PFCS), a continuous variable between 0 and 100, was defined by a combination of placental weight compared to that expected for gestational age, villous maturational abnormalities, percentage of parenchymal lesions, and presence of inflammation. We compared PFCS distributions in 3 existing placental pathology repositories, 2 with livebirths (n = 9783 and n = 575) and 1 with stillbirths (n = 149). Within the 2 livebirth cohorts, we compared PFCS by adverse pregnancy outcomes. Data were analyzed using SPSS and RStudio.</p><p><strong>Results: </strong>PFCS could be robustly applied in the 3 cohorts and could be calculated using simple formulas easily applied by software programs. Mean PFCS was 74.60 (SD 18.09) and 77.23 (SD 16.56) in the 2 liveborn cohorts, but was markedly reduced to 62.61 (SD 28.92) in the stillbirth cohort (p<.0001). Mean PFCS was significantly lower in preterm birth compared to term births, SGA infant versus non-SGA infant, and preeclampsia versus no preeclampsia, in both of the liveborn cohorts.</p><p><strong>Conclusion: </strong>It is feasible to calculate a continuous, quantitative PFCS from information collected in routine placental pathology examination, and PFCS is reduced in stillbirths compared to live birth, in preterm births compared to term births, and in SGA infants compared to non-SGA infants.</p>","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"10935266261470991"},"PeriodicalIF":1.3,"publicationDate":"2026-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148633518","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Association of First-Trimester Pan-Immune-Inflammation Value with Chronic Histiocytic Intervillositis and Villitis of Unknown Etiology.","authors":"Esra Karakus, Ali Furkan Yahsi, Kubra Katipoglu, Esengül Türkyılmaz","doi":"10.1177/10935266261469404","DOIUrl":"https://doi.org/10.1177/10935266261469404","url":null,"abstract":"<p><strong>Background: </strong>Chronic histiocytic intervillositis (CHI) and villitis of unknown etiology (VUE) are immune-mediated placental disorders leading to adverse pregnancy outcomes. This study investigated whether first-trimester pan-immune-inflammation value (PIV), a novel systemic inflammation marker, is associated with these conditions.</p><p><strong>Methods: </strong>This retrospective case-control study (October 2022-August 2025) included 163 placentas (25 controls, 63 CHI, 75 VUE). First-trimester complete blood counts (<14 weeks) were used to calculate PIV = (neutrophil × platelet × monocyte)/lymphocyte. Histopathological examination followed Amsterdam criteria. Multivariable logistic regression and ROC analyses were performed.</p><p><strong>Results: </strong>PIV was significantly higher in cases versus controls (503.1 vs 381.3, <i>P</i> = .048). Across groups, PIV was highest in CHI (661.6), intermediate in VUE (446.4), and lowest in controls (<i>P</i> < .001). CHI showed the worst perinatal outcomes: lowest gestational age (30.5 weeks), birth weight, and Apgar scores, and highest intrauterine fetal death (41.3%). After multivariable adjustment, first-trimester PIV remained independently associated with CHI (aOR 1.87, 95% CI 1.19-4.27, <i>P</i> = .005) but not with VUE (aOR 0.74, <i>P</i> = 0.103). Discriminatory performance was modest for CHI (AUC 0.677) and poor for VUE (AUC 0.434).</p><p><strong>Conclusion: </strong>Elevated first-trimester PIV is independently associated with CHI but not VUE, suggesting that systemic maternal inflammation may be detectable early in gestation, particularly in more severe placental immune pathology. PIV is not a standalone diagnostic test but may contribute to risk assessment when combined with clinical findings.</p>","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"10935266261469404"},"PeriodicalIF":1.3,"publicationDate":"2026-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148538110","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Fatal Pulmonary Air Emboli Associated with Pneumatosis Intestinalis.","authors":"Laura S Finn, Brittany DePasquale, Christine Camacho-Bydume","doi":"10.1177/10935266261466222","DOIUrl":"https://doi.org/10.1177/10935266261466222","url":null,"abstract":"<p><p>A 19-month-old patient died suddenly and unexpectedly ~11 months after hematopoietic stem cell transplantation (HSCT) for Hurler syndrome. Post-mortem examination revealed pulmonary air emboli that were attributed to extensive colonic pneumatosis intestinalis, an uncommon but reported complication of HSCT. Gas emboli are not just the result of trauma or decompression sickness but are more commonly iatrogenic in origin, resulting from the procedural-related introduction of air into the vascular system; pneumatosis intestinalis has rarely been associated with air emboli. Pathologists should be cognizant of unusual complications of HSCT and maintain a high index of suspicion for air embolization, even in a medically complex patient, to ensure optimal autopsy examination.</p>","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"10935266261466222"},"PeriodicalIF":1.3,"publicationDate":"2026-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148498634","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Mapping and Characterization of Isolated Lymphoid Follicles in Patients with Hirschsprung-Associated Enterocolitis.","authors":"Hanna Johannessen, Anders Telle Hoel, Remi André Karlsen, Hogne Røed Nilsen, Kristin Bjørnland, Espen Sønderaal Bækkevold, Frode Lars Jahnsen","doi":"10.1177/10935266261466230","DOIUrl":"https://doi.org/10.1177/10935266261466230","url":null,"abstract":"<p><p>Hirschsprung-associated enterocolitis (HAEC) is a severe complication of Hirschsprung's disease (HSCR), but its pathogenesis remains unclear. Gut-associated lymphoid tissue (GALT), including isolated lymphoid follicles (ILFs), plays a key role in mucosal immunity. This study investigated the presence, distribution, and phenotype of ILFs in colonic tissue from 35 HSCR patients and 12 controls. Histological and immunohistochemical analyses revealed mature ILFs in both ganglionic and aganglionic bowel, with no significant differences in densities between HSCR patients with and without HAEC. However, <b>patients that developed HAEC showed signs of enhanced B-cell activation in ILFs.</b> Patients with a stoma had significantly increased ILF density, suggesting that luminal content may influence ILF development. Despite the absence of enteric neurons, ILFs were structurally similar to those described in adults, indicating that ILF formation occurs independently of a functional enteric nervous system. These findings provide new insights into mucosal immunity in HSCR <b>and suggest a role for activation of the mucosal B-cell compartment in</b> HAEC pathogenesis.</p>","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"10935266261466230"},"PeriodicalIF":1.3,"publicationDate":"2026-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148451387","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Juvenile Myelomonocytic Leukemia Presenting with Unusual Immunonologic Manifestations: Report of a Case with Multiple Somatic Genetic Aberrations Including <i>KRAS</i> and Novel <i>ETV6</i> Mutations.","authors":"Kristina R Brannock, Benjamin Kaumeyer, Kathleen K Nicol, Lionel M L Chow, Hemalatha G Rangarajan, Samir B Kahwash","doi":"10.1177/10935266261466228","DOIUrl":"https://doi.org/10.1177/10935266261466228","url":null,"abstract":"<p><p>We report an unusual case of juvenile myelomonocytic leukemia (JMML) in a 4-year-old male who had persistent monocytosis and intermittent anemia and thrombocytopenia beginning at 1 year of age. Given a history of recurrent skin infections, the patient initially received an evaluation for a primary immune disorder, but workup was inconclusive. Bone marrow examination was performed to further evaluate persistent and progressive cytopenias. The findings fulfilled the criteria for JMML with peripheral blood monocytosis ≥ 1×10⁹/L, blast/promonocyte percentage of <20% in peripheral blood and bone marrow, clinical evidence of organ infiltration with hepatosplenomegaly, and absent <i>BCR::ABL1</i> fusion and <i>KMT2A</i> gene rearrangements. Additionally, next generation sequencing (NGS) studies performed on the bone marrow aspirate revealed multiple acquired somatic mutations, including a <i>KRAS</i> G12D mutation, meeting criteria for the diagnosis of JMML in the context of this case. A novel <i>ETV6</i> R369W mutation in the setting of JMML was also detected, which may explain the striking megakaryocytic dysplasia in the bone marrow beyond what is typically seen in JMML. This case showed interesting features overlapping with Ras-associated autoimmune leukoproliferative disorder (RALD), including monocytosis and recurrent infections. Furthermore, this case demonstrated a need for more definitive criteria for distinguishing JMML from RALD.</p>","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"10935266261466228"},"PeriodicalIF":1.3,"publicationDate":"2026-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148414565","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Histopathological Profiling of the Urethral Plate in Hypospadias Patients.","authors":"Gede Wirya Kusuma Duarsa, Desak Agung Istri Padma Putri, Putu Angga Risky Raharja, Ronald Sugianto, Pande Made Wisnu Tirtayasa","doi":"10.1177/10935266261461706","DOIUrl":"https://doi.org/10.1177/10935266261461706","url":null,"abstract":"<p><strong>Background: </strong>Hypospadias is a congenital anomaly characterized by ectopic placement of the urethral meatus on the ventral surface of the penis. Surgical manipulation of the urethral plate during urethroplasty induces tissue injury and wound-healing responses that may lead to fibrosis and altered tissue remodeling. However, histopathological differences of the urethral plate between distal and proximal hypospadias remain incompletely characterized.</p><p><strong>Methods: </strong>This retrospective analytical study included pediatric patients with hypospadias who underwent primary surgical repair between 2013 and 2023. Urethral plate specimens excised from the dartos fascia to the tunica albuginea were collected intraoperatively and examined histopathologically. Hematoxylin-eosin and Masson trichrome staining were used to assess fibrosis severity, collagen density, and inflammatory cell infiltration. Associations between histopathological parameters and hypospadias type were analyzed statistically.</p><p><strong>Results: </strong>A total of 96 patients met the inclusion and exclusion criteria and were included in the analysis. Fibrosis severity was significantly associated with hypospadias type (<i>P</i> = .028), with distal hypospadias demonstrating a higher risk of moderate to severe fibrosis compared with proximal hypospadias (risk ratio, 2.56; 95% CI, 1.098-5.97). In contrast, no significant differences were observed between distal and proximal hypospadias with respect to collagen density or inflammatory cell infiltration.</p><p><strong>Conclusions: </strong>Fibrosis severity of the urethral plate differs significantly between distal and proximal hypospadias, indicating that fibrotic remodeling represents a distinct histopathological feature associated with hypospadias severity. Collagen density and inflammatory cell presence did not differ significantly between groups. These findings may have implications for surgical planning and tissue handling in hypospadias repair.</p>","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"10935266261461706"},"PeriodicalIF":1.3,"publicationDate":"2026-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148414545","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Rhabdomyomatous Mesenchymal Hamartoma With Prominent Folliculosebaceous Hyperplasia in an Infant's Philtrum: A Case Report.","authors":"Mikiko Inada, Masaru Morita, Miki Izumi","doi":"10.1177/10935266261461665","DOIUrl":"https://doi.org/10.1177/10935266261461665","url":null,"abstract":"<p><p>Rhabdomyomatous mesenchymal hamartoma (RMH) is a rare congenital malformation. We report a case involving a 20-month-old girl who presented with a congenital midline philtral mass. Histopathological examination revealed mature striated muscle fibers interspersed with adipose tissue and prominent adnexal structures, including sebaceous glands originating from hair follicles. The lesion's prominent folliculosebaceous components initially suggested folliculosebaceous cystic hamartoma (FSCH). However, the presence of striated muscle hyperplasia, midline location, congenital onset, and the absence of characteristic cleft formation confirmed the diagnosis of RMH. The patient underwent complete surgical excision and was recurrence-free after 5 years of follow-up. This case highlights the importance of considering RMH in the differential diagnosis of congenital midline facial lesions, including those with adnexal components.</p>","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"10935266261461665"},"PeriodicalIF":1.3,"publicationDate":"2026-07-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148400480","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Heterotopic Salivary Gland in the Rectum Associated with Lumbosacral Lipomeningocele: A Dual Developmental Anomaly.","authors":"Rajarshi Debnath, Sayanta Bera, Aisharya Banerjee, Iqbal Khan, Keya Basu, Uttara Chatterjee","doi":"10.1177/10935266261466226","DOIUrl":"https://doi.org/10.1177/10935266261466226","url":null,"abstract":"<p><p>Heterotopic salivary glandular tissue within the rectum is exceptionally rare. To date, only 4 cases have been reported in literature and all in adult patients. Here we report a case of ectopic salivary glandular tissue in the rectum in a 12-year-old girl who also had a lumbosacral lipomeningocele. The coexistence of these 2 developmental anomalies; 1 endodermal and 1 neuroectodermal, has not been reported previously. The case is discussed with emphasis on embryologic correlation, review of literature, and a summary of prior published cases.</p>","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"10935266261466226"},"PeriodicalIF":1.3,"publicationDate":"2026-07-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148393169","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Assessing Normal Histologic Patterns in Perinatal Tissue: A Practical Guide.","authors":"Linda M Ernst, Erica Price, Beverly B Rogers","doi":"10.1177/10935266251413975","DOIUrl":"https://doi.org/10.1177/10935266251413975","url":null,"abstract":"<p><strong>Purpose: </strong>This review is intended to provide practical guidance to those who examine the tissues of fetuses and infants under the microscope. We focus on what is normal at various stages of development, and try to point out when caution should be used in interpreting histologic findings.</p><p><strong>Content: </strong>We present descriptions and images of normal developmental histology and its variations in the major human organs during fetal and neonatal life. The organs reviewed include the cardiovascular system, lungs, liver, pancreas, tubular gastrointestinal tract, kidneys, thymus, adrenal glands, gonads, thyroid gland, spleen, brain, bone, and skeletal muscle.</p>","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":"29 4","pages":"382-405"},"PeriodicalIF":1.3,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148379584","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Ancillary and Advanced Techniques for the Postmortem Examination.","authors":"Halit Pinar, Füsun Gündoğan","doi":"10.1177/10935266261428194","DOIUrl":"10.1177/10935266261428194","url":null,"abstract":"<p><strong>Purpose: </strong>This article describes how techniques such as advanced imaging, nucleic acid evaluation, integrated approaches, and data collection systems can facilitate the examination of a stillbirth or newborn, particularly when an autopsy is not possible.</p><p><strong>Content: </strong>The traditional autopsy remains the gold standard for investigating stillbirth and neonatal death. However, declining rates due to parental refusal, financial constraints, and cultural barriers highlight the need to widely adopt modern alternatives. This review examines emerging ancillary and advanced diagnostic techniques necessary to maximize diagnostic yield and ensure equitable access to comprehensive postmortem evaluations. Since imaging alone often misses critical microscopic pathologies, hybrid methods like Minimally Invasive Autopsy with Laparoscopically Assisted Sampling (MinImAL) are essential. MinImAL combines Postmortem Magnetic Resonance Imaging (PMMR) with image-guided tissue sampling to acquire specimens for histological and microbiological analysis. Complementary molecular techniques aid in identifying underlying genetic causes and informing recurrence risk. The success of these tests relies on optimized tissue selection. Systemic improvements include adopting standardized protocols, establishing Stillbirth Centers of Excellence, and using Artificial Intelligence (AI) for risk prediction. Importantly, integrating these advanced, less invasive methods is vital for addressing financial and racial health disparities, especially given the lack of Medicaid coverage for autopsy, thereby ensuring all bereaved families receive accurate and necessary diagnostic information.</p>","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":"29 4","pages":"431-437"},"PeriodicalIF":1.3,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13332187/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148379579","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}