{"title":"Primary Gastrointestinal Iatrogenic Kaposi Sarcoma: A Rare Pediatric Case Report.","authors":"Nil Urganci, Ayse Mine Onenerk Men, Nuray Kepil, Rahsan Ozcan, Nil Comunoglu","doi":"10.1177/10935266251345690","DOIUrl":"10.1177/10935266251345690","url":null,"abstract":"<p><p>Kaposi sarcoma (KS) is a rare, human herpesvirus-8 (HHV-8)-associated vascular tumor that primarily affects the skin but can involve visceral organs, particularly in immunosuppressed patients. While iatrogenic KS is well-documented in adults, isolated gastrointestinal involvement in pediatric cases remains unreported. We describe a 16-year-old HIV-negative male with chronic granulomatous disease (CGD) who initially presented with inflammatory bowel disease-like symptoms and was treated with immunosuppressive therapy. Over 17 months, he developed recurrent infections, hepatosplenomegaly, and ultimately intestinal perforation, necessitating emergency colectomy. Histopathology confirmed iatrogenic KS, highlighting the need for increased awareness of this rare malignancy in immunocompromised pediatric patients and carefully balancing the risks of long-term immunosuppressive therapy.</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":"425-428"},"PeriodicalIF":1.3,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144295650","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}
Peilin Zhang, Octavio Armas, Art Mendoza, Chris Wixom, Omid Bakhtar, Raymond Redline, Jenelle Coon, Lana Kabakibi
{"title":"Umbilical Cord Weight Index Correlated with Fetal Birth Weight and Associated with Placental and Umbilical Cord Abnormalities.","authors":"Peilin Zhang, Octavio Armas, Art Mendoza, Chris Wixom, Omid Bakhtar, Raymond Redline, Jenelle Coon, Lana Kabakibi","doi":"10.1177/10935266251349487","DOIUrl":"10.1177/10935266251349487","url":null,"abstract":"<p><strong>Background: </strong>Umbilical cord consists of 2 arteries and 1 vein embedded in Wharton's jelly. Umbilical cord weight has not been examined for fetal growth and placental pathology.</p><p><strong>Materials and methods: </strong>We have assessed umbilical cord by measuring the weight and the length during placental examination. The cord weight index (CWI) was defined as an average weight of 10 cm cord, and the normal ranges of cord diameter and CWI were established as 10th to 90th percentile.</p><p><strong>Results: </strong>A total of 520 cases of umbilical cord were examined. Normal ranges of 10th to 90th percentile of cord diameter and CWI were established. Light cord was defined as less than 10th percentile and heavy cord was greater than 90th percentile of CWI. Light cord was significantly associated with decreased cord diameter, lower placental and fetal birth weight, female sex, nuchal cord, and IUGR, while heavy cord was significantly associated with increased cord diameter, increased BMI, male sex, diabetes mellitus, and decreased maternal and fetal inflammatory responses.</p><p><strong>Conclusion: </strong>Our data demonstrated that CWI is a novel and superior measure of cord development to diameter, and CWI can add value, providing preliminary evidence for more rigorous study and for incorporating CWI in placental 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":"381-389"},"PeriodicalIF":1.3,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144311207","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}
Conner Thompson, Katsiaryna Khatskevich, Cynthia T Welsh, Tiffany G Baker, Daniel C Butler
{"title":"Neuropathologic Findings in Lennox-Gastaut Syndrome (SCN2A-Related): A Case Report and Review of Literature.","authors":"Conner Thompson, Katsiaryna Khatskevich, Cynthia T Welsh, Tiffany G Baker, Daniel C Butler","doi":"10.1177/10935266251345684","DOIUrl":"10.1177/10935266251345684","url":null,"abstract":"<p><p>Lennox-Gastaut syndrome is a form of severe childhood epilepsy caused by a variety of etiologies including structural abnormalities, inflammatory conditions affecting the brain, malignancy, injury, or unknown causes. Sodium channelopathies have been linked to multiple seizure disorders, including Lennox-Gastaut syndrome, due to their role in action potential propagation in the brain. <i>SCN2A</i> is one such voltage gated sodium channel found primarily in the central nervous system. We present a case of a 6-year-old female with Lennox-Gastaut syndrome and a heterozygous <i>SCN2A</i> variant who became unresponsive during a supervised bath and later died. The neuropathologic exam was remarkable for dentato-olivary dysplasia, among other gross and microscopic abnormalities. We present this case highlighting rarely documented neuropathologic findings in Lennox-Gastaut syndrome associated with a channelopathy (<i>SCN2A</i> variant) as well as a review of literature of previously reported brain abnormalities in patients with <i>SCN2A</i> variants.</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":"420-424"},"PeriodicalIF":1.3,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144328470","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":"SOX2 Expression and Regulation in Pulmonary Aplasia/Agenesis.","authors":"Alexia Apostolou, Madeleine Joubert, Brice Poreau, Christian Piolat, Francine Arbez Gindre, Christophe Nemos, Herve Sartelet","doi":"10.1177/10935266251349491","DOIUrl":"10.1177/10935266251349491","url":null,"abstract":"<p><strong>Background: </strong>Pulmonary agenesis is characterized by the absence of bronchi and lung parenchyma and it differs from pulmonary aplasia by the presence of rudimentary bronchial buds. SOX2 expression is observed during the normal course of lung development. The objective of this study is to investigate the expression of SOX2 and its regulation in the residual airway epithelium of pulmonary agenesis/aplasia.</p><p><strong>Methods: </strong>Six cases of pulmonary agenesis/aplasia aged between 12 and 37 weeks of gestation and 6 age-matched controls were studied. Immunochemistry was performed using primary antibodies against SOX2, BMP4, FGF9, FGF10, TTF1, SHH, and beta-catenin.</p><p><strong>Results: </strong>In sections of bronchi or trachea from lung agenesis or aplasia, the residual epithelium shows a high nuclear expression of SOX2 and an absence of expression of BMP4 as in the esophagus whereas in control cases, the airway epithelium shows an absence of expression of SOX2 and a high expression of BMP4. There were no differences between control and agenesis/aplasia cases concerning the expression of FGF9, FGF10, SHH, TTF1, and beta-catenin.</p><p><strong>Conclusion: </strong>The expression of SOX2 and BMP4 is strongly altered in pulmonary agenesis/aplasia. Thus, these proteins appear to regulate tissue-specific proliferative activity during early lung development.</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":"376-380"},"PeriodicalIF":1.3,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144319202","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":"Incidental Finding of a Herniated Round Ligament of the Liver During Pediatric Epigastric Hernia Repair.","authors":"Javier Arredondo Montero, Samuel Sáez Álvarez","doi":"10.1177/10935266251343934","DOIUrl":"10.1177/10935266251343934","url":null,"abstract":"","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"432-434"},"PeriodicalIF":1.3,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144802416","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}
Elizabeth O Ferreira, Marc R Del Bigio, Jason Morin, Patrick Frosk
{"title":"Infant With a Severe Form of <i>GLRX5</i>-Related Atypical Hyperglycinemia Exhibiting Novel Cardiac and Neurologic Disease Manifestations at Autopsy.","authors":"Elizabeth O Ferreira, Marc R Del Bigio, Jason Morin, Patrick Frosk","doi":"10.1177/10935266251335065","DOIUrl":"10.1177/10935266251335065","url":null,"abstract":"<p><p>Glutaredoxin 5 (GLRX5) is a mitochondrial protein encoded by the GLRX5 gene, which is essential for cellular redox homoeostasis, lipoic acid synthesis, and iron-sulfur cluster transfer. Rare cases of pathogenic GLRX5 mutations have been associated with sideroblastic anemia and non-ketotic hyperglycinemia with progressive spasticity and cavitating leukoencephalopathy. We report an 11-month-old child, who died following aspiration, with severe cardiomyocyte mitochondrial abnormalities and cerebral white matter degeneration in the context of a homozygous GLRX5 variant (c.208A>G, p.S70G).</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":"390-394"},"PeriodicalIF":1.3,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12426336/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144145416","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}
Elizabeth O Ferreira, Stefan Kostadinov, Camelia Stefanovici, Virginia Duncan
{"title":"In Response to Dr. James R. Wright, Jr.'s Letter to the Editor.","authors":"Elizabeth O Ferreira, Stefan Kostadinov, Camelia Stefanovici, Virginia Duncan","doi":"10.1177/10935266251357785","DOIUrl":"10.1177/10935266251357785","url":null,"abstract":"","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"431"},"PeriodicalIF":1.3,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144755662","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}
Tania Marsden, Teck Yee Khong, Jane E Dahlstrom, Fran Boyle, Mu Cheng, Yin Ping Wong, Stacey Prystupa, Gretchen Pomare, Joanna Perry-Keene, Vicki Flenady, Jessica Sexton
{"title":"Placental Pathology Reporting Practices in Australian Stillbirths: A Quality Review.","authors":"Tania Marsden, Teck Yee Khong, Jane E Dahlstrom, Fran Boyle, Mu Cheng, Yin Ping Wong, Stacey Prystupa, Gretchen Pomare, Joanna Perry-Keene, Vicki Flenady, Jessica Sexton","doi":"10.1177/10935266251349492","DOIUrl":"10.1177/10935266251349492","url":null,"abstract":"<p><strong>Background: </strong>Stillbirth continues to pose a significant public health challenge. Autopsy and placental assessments are recognized as the gold standard for stillbirth investigation. The utility of these procedures can vary based on the quality of the examination. The aim of this study is to determine the quality of placenta pathology reporting in Australia in the context of a stillbirth.</p><p><strong>Materials and methods: </strong>Placenta pathology reports from stillbirths were reviewed from 18 maternity hospital from 2013 to 2018. The Khong tool was used to produce a placenta quality score (PQS), by a blinded panel of assessors to the cause of death. Outcome measures were the number of reports achieving the minimal acceptable score (MAS) of 75% or a poor score (PS) of 50% of the PQS.</p><p><strong>Results: </strong>560 placental pathology reports of which 494 were singleton and 66 were twin placentas. 282 (50%) achieved the MAS score. Macroscopic items were recorded well and microscopic items recorded poorly.</p><p><strong>Conclusions: </strong>The standard of placenta pathology reporting can be improved in Australia. The use of templates or checklists for both macroscopic descriptions and histological reporting is recommended to ensure all key components are described.</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":"369-375"},"PeriodicalIF":1.3,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144311206","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}
Anita Nagy, Colleen Collins, Linda J Szymanski, Bruce R Pawel, Portia A Kreiger, Tricia Bhatti, Murillo Olivia, Erin Rudzinski, Jeanette Reyes, Melissa Blessing, Kudakwashe Chikwava, Juan Putra, Chrystalle Katte Carreon
{"title":"A Retrospective Review of Autopsy Data From 6 North American Institutions Indicates Practice Variations That Could Be Leveraged to Address Declining Autopsy Rates.","authors":"Anita Nagy, Colleen Collins, Linda J Szymanski, Bruce R Pawel, Portia A Kreiger, Tricia Bhatti, Murillo Olivia, Erin Rudzinski, Jeanette Reyes, Melissa Blessing, Kudakwashe Chikwava, Juan Putra, Chrystalle Katte Carreon","doi":"10.1177/10935266251346988","DOIUrl":"10.1177/10935266251346988","url":null,"abstract":"","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"435-437"},"PeriodicalIF":1.3,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12426317/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144513073","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}
Josselyn Sofia Vergara, Lianna Jean Marks, Oscar Silva
{"title":"Primary Splenic Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL): First Reported Case in a Young Child.","authors":"Josselyn Sofia Vergara, Lianna Jean Marks, Oscar Silva","doi":"10.1177/10935266251368431","DOIUrl":"https://doi.org/10.1177/10935266251368431","url":null,"abstract":"<p><p>Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a rare subtype of B-cell lymphoma. NLPHL is usually indolent, involves lymph nodes and shows a favorable prognosis with high overall survival. In a minority of cases, patients may present and/or progress to advanced disease with involvement of the spleen, liver, and/or bone marrow. While splenic involvement by NLPHL is usually presumed evidence of advanced disease with poor prognosis, here we report to our knowledge, the first case of primary splenic NLPHL occurring in a child who showed no overt nodal disease and is currently free of disease 3 years post-splenectomy without additional treatment.</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":"10935266251368431"},"PeriodicalIF":1.3,"publicationDate":"2025-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144986837","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}