Fatma Cavide Sönmez, Marcus Pauly, Annette M Müller
{"title":"Multicystic Adenomatoid Pancreatic Hamartoma in a Child: Case Report.","authors":"Fatma Cavide Sönmez, Marcus Pauly, Annette M Müller","doi":"10.1177/10935266251381436","DOIUrl":"https://doi.org/10.1177/10935266251381436","url":null,"abstract":"<p><p>Multicystic adenomatoid pancreatic hamartoma (MAPH) is a rare lesion of the pancreas, particularly in the pediatric age group with only a few cases reported in the literature. We report an unusual cystic lesion diagnosed MAPH in a 26-month-old boy who presented with abdominal swelling on the right side for a period of 1 week, accompanied by icterus and itching. Magnetic resonance imaging (MRI) revealed a giant multicystic intra-abdominal mass with pressure-induced cholestasis. The large retroperitoneal lesion was completely resected. Histologically, the lesion consisted of multilocular micro- and macrocysts lined by flat, cuboidal, and columnar epithelium without atypia. The cyst walls contained notably well-formed pancreatic acini, and partially dilated ducts, and inflammatory cells, hemorrhage, edema, fibrosis with reactive fibroblasts.</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":"10935266251381436"},"PeriodicalIF":1.3,"publicationDate":"2025-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145234890","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}
Yan Zhou, Stephen P Sanders, Chrystalle Katte Carreon
{"title":"Left Pulmonary Artery Sling: Anatomy, Associations, and Outcome of Surgical Repair as Demonstrated by Autopsies.","authors":"Yan Zhou, Stephen P Sanders, Chrystalle Katte Carreon","doi":"10.1177/10935266251366015","DOIUrl":"https://doi.org/10.1177/10935266251366015","url":null,"abstract":"<p><p>Left pulmonary artery (LPA) sling is a rare congenital anomaly in which the LPA abnormally originates from the right pulmonary artery (RPA) and courses between the trachea and esophagus to reach the left pulmonary hilum. This anomaly is frequently associated with tracheobronchial and other cardiovascular anomalies and patients may manifest with varying airway and cardiovascular symptoms. Surgical repair is often required for symptomatic patients. Clinical outcomes largely depend on the extent and severity of coexisting anomalies, particularly tracheobronchial abnormalities. We report 2 autopsy cases of LPA sling, 1 pre- and 1 post-surgical repair. Comprehensive autopsy examination was crucial for confirmation of the clinical diagnoses and identification of a rare surgical complication.</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":"10935266251366015"},"PeriodicalIF":1.3,"publicationDate":"2025-10-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145215200","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":"Letter to the Editor.","authors":"Linda M Ernst","doi":"10.1177/10935266251357777","DOIUrl":"https://doi.org/10.1177/10935266251357777","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":"10935266251357777"},"PeriodicalIF":1.3,"publicationDate":"2025-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145188302","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}
Geoffrey C Halling, Jonathan B McHugh, Amer Heider
{"title":"PRRX1-Rearranged Mesenchymal Tumor: A Case Report and Review of the Literature.","authors":"Geoffrey C Halling, Jonathan B McHugh, Amer Heider","doi":"10.1177/10935266251374258","DOIUrl":"https://doi.org/10.1177/10935266251374258","url":null,"abstract":"<p><p>PRRX1-rearranged mesenchymal/fibroblastic tumor is a recently proposed, molecularly defined soft tissue tumor, which has not yet been included in the WHO's most recent edition of soft tissue tumors. This entity has previously been reported in a wide age range (20-76 years). However, we report the first pediatric case in an 11-year-old patient presenting with 5.5 cm mass in the head and neck region. Our case is also the second reported case of a PRRX1 (exon 1)::NCOA1 (exon 15) fusion transcript to date.</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":"10935266251374258"},"PeriodicalIF":1.3,"publicationDate":"2025-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145077189","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}
Shengmei Zhou, Ryan Schmidt, Paul Zamiara, Nick Shillingford
{"title":"High-Grade Malignant Pleuropulmonary Neoplasm With <i>YAP1::MAML2</i> Gene Fusion.","authors":"Shengmei Zhou, Ryan Schmidt, Paul Zamiara, Nick Shillingford","doi":"10.1177/10935266251374255","DOIUrl":"https://doi.org/10.1177/10935266251374255","url":null,"abstract":"<p><p>A 17-month-old male presented with a 4-week history of cough, and a CT scan revealed a large right intrathoracic mass with metastases to the brain and femur. Tumor biopsy demonstrated a high-grade malignant neoplasm with histological and immunostaining features most suggestive of, but not entirely typical for type III pleuropulmonary blastoma (PPB). Molecular analysis identified a <i>YAP1::MAML2</i> gene fusion and a complex copy number profile, with no mutations in <i>DICER1</i> or <i>TP53</i>. The patient initially improved with chemotherapy, but progressive brain metastases led to palliative care, and he passed away 10 months later. This is the first reported case of a <i>YAP1::MAML2</i> fusion in a pleuropulmonary neoplasm.</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":"10935266251374255"},"PeriodicalIF":1.3,"publicationDate":"2025-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145071593","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}
Ansa Mehreen, Erica Price, Vivien Wang, Sunitha Suresh, Kathy Mangold, Linda Sabatini, John Lee, Linda M Ernst
{"title":"Pregnancy Loss Associated with COVID-19 Infection: Associations with Detection of Viral RNA in Placental and Fetal Tissue.","authors":"Ansa Mehreen, Erica Price, Vivien Wang, Sunitha Suresh, Kathy Mangold, Linda Sabatini, John Lee, Linda M Ernst","doi":"10.1177/10935266251368434","DOIUrl":"https://doi.org/10.1177/10935266251368434","url":null,"abstract":"<p><strong>Background: </strong>The purpose of this study was to investigate placental pathology and cause of death (COD) in pregnancy losses associated with maternal COVID-19 infection and examine associations with detection of SARS-CoV-2 in placenta and fetal organs.</p><p><strong>Methods: </strong>Demographics, placental pathology, and COD information was collected from fetal autopsies done at Evanston hospital, IL between 03/1/2020 and 12/31/2022 where maternal history indicated positive SARS-CoV-2 test during pregnancy or SARS-CoV-2 infection was suspected after fetal autopsy. Polymerase chain reaction (PCR) testing for SARS-CoV-2 RNA was performed on placenta, fetal liver, and lung tissue.</p><p><strong>Results: </strong>We identified 13 autopsies and detected SARS-CoV-2 in placentas of 5 cases, all showed chronic histiocytic intervillositis (CHI) and massive perivillous fibrin deposition (MPVFD). CHI and MPVFD were absent in all the PCR-negative cases (<i>P</i> < .001). Fetal liver was positive for SARS-CoV-2 in 1 case, which was in the group with negative placental SARS-CoV-2 PCR. The lung tissue was negative in all cases.</p><p><strong>Conclusion: </strong>We confirm the association between detectable placental SARS-CoV-2 with CHI and MPVFD in placentas from pregnancy losses in mother who had COVID-19 during their pregnancy. Detection of SARS-CoV-2 in fetal liver, despite negative placental tissue, was unexpected and may be important for understanding the impact of maternal COVID infection on neonates.</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":"10935266251368434"},"PeriodicalIF":1.3,"publicationDate":"2025-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145035104","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}
Muhammad Usman Tariq, Nasir Ud Din, Taha Nafees, Naila Kayani
{"title":"Phyllodes Tumor of Breast in Pediatric and Adolescent Population: A Detailed Clinicopathological Study of 22 Cases.","authors":"Muhammad Usman Tariq, Nasir Ud Din, Taha Nafees, Naila Kayani","doi":"10.1177/10935266251369273","DOIUrl":"https://doi.org/10.1177/10935266251369273","url":null,"abstract":"<p><strong>Introduction: </strong>Phyllodes tumor (PT) are rarely seen in young population. Some authors believe that PT behave less aggressively in young patients and the need for aggressive management is questioned.</p><p><strong>Objective: </strong>We aimed to describe the clinicopathological features of PT in pediatric and adolescent population.</p><p><strong>Methodology: </strong>We reviewed the reports and microscopy glass slides of PT from 22 pediatric and adolescent patients (≤20 years old), diagnosed between 2010 and 2024.</p><p><strong>Results: </strong>Eighteen cases were malignant PT (MPT), 3 benign PT, and 1 borderline PT. Median patient's age was 17 years (range: 14-20). Median tumor size was 11.25 cm (range: 1.3-22). Tumor margins were either positive or <1 mm away in 10/17 (58.9%) tumor excisions. Follow up information was available for 12 MPT patients with median follow up duration of 26.5 months. Recurrence was observed in 2/10 (20%) primary MPT cases at 2- and 3-months interval. Distant metastasis was observed in 4/12 (33.3%) cases at median interval of 12 months. One of the metastatic cases and another patient without definite treatment, died of disease.</p><p><strong>Conclusion: </strong>We observed higher frequency of MPT patients, larger tumor size, and higher rate of mastectomy. MPT behave aggressively as evidenced by recurrence, metastasis, and tumor-related death.</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":"10935266251369273"},"PeriodicalIF":1.3,"publicationDate":"2025-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145025186","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}
Aida Glembocki, Robert Siddaway, Anthony Arnoldo, Gino R Somers
{"title":"Fusion-Negative Rhabdomyosarcoma: Clinical Application of Targeted RNA Sequencing.","authors":"Aida Glembocki, Robert Siddaway, Anthony Arnoldo, Gino R Somers","doi":"10.1177/10935266251370493","DOIUrl":"https://doi.org/10.1177/10935266251370493","url":null,"abstract":"<p><strong>Background: </strong>Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood. For stratification purposes, rhabdomyosarcoma is classified into fusion-positive RMS (alveolar rhabdomyosarcoma) and fusion-negative RMS (embryonal or spindle cell/sclerosing, FN-RMS) subtypes according to its <i>PAX::FOXO1</i> fusion status. This study aims to highlight the pathologic and molecular characteristics of a cohort of FN-RMS using a targeted NGS RNA-Seq assay.</p><p><strong>Methods: </strong>Twelve tumors were analyzed through targeted RNA-Seq using the Trusight Pancancer panel from Illumina. Molecular alterations were then correlated with the clinicopathological features.</p><p><strong>Results: </strong>Of the 12 tumors analyzed, we identified 6 embryonal rhabdomyosarcomas (ERMSs) harboring mutations in key signaling molecules (<i>KRAS, HRAS, NRAS</i>, and <i>FGFR4</i>), oncogenic <i>DICER1</i> mutations in 2 ERMS, pathogenic <i>TP53</i> and <i>NF1</i> mutations in an ERMS with features of anaplasia, a <i>TEAD1::NCOA2</i> gene fusion in a congenital spindle cell and sclerosing rhabdomyosarcoma (SSRMS), and a <i>FUS::TFCP2</i> gene fusion in a skull base SSRMS. Only 1 ERMS in the bladder showed no reportable molecular alterations.</p><p><strong>Conclusion: </strong>We illustrate case examples demonstrating how a combined morphological and molecular approach with targeted RNA-Seq can aid in diagnosis and identify clinically actionable alterations in pediatric FN-RMS.</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":"10935266251370493"},"PeriodicalIF":1.3,"publicationDate":"2025-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145025179","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":"Evaluating Intestinal Metaplasia in Pediatric Gallbladder Specimens: Clinicopathologic Significance and Sampling Thresholds.","authors":"Adeyinka Akinsanya, Iván A González","doi":"10.1177/10935266251341508","DOIUrl":"10.1177/10935266251341508","url":null,"abstract":"<p><strong>Background: </strong>Gallbladders are a commonly encounter specimen in pediatric pathology practice. In the adult population, intestinal metaplasia (IM) in the gallbladder is associated with the development of dysplasia and adenocarcinoma; however, in children its significance is unknown, and the appropriate sampling has not been described which is the goal of this study.</p><p><strong>Methods: </strong>Twenty-five routine pediatric cholecystectomy cases with IM were identified, and their clinical and histologic findings were reviewed.</p><p><strong>Results: </strong>Of these 25 cases, 23 were female (92%). The most common indication for surgery was cholelithiasis (84%). Stones were present in 21 cases (84%). Twenty-three cases (92%) had additional sections submitted with an average of 3.9 slides (range = 3-6), and 52% had IM in additional blocks. However, no dysplasia or carcinoma was identified in any case.</p><p><strong>Conclusion: </strong>IM in the gallbladder is frequently seen in the setting of gallstones and chronic inflammation. Based on our experience, no additional sampling is required when incidentally identified.</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":"362-368"},"PeriodicalIF":1.3,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144103502","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":"Lethal Neonatal Pulmonary Hypertension in Trisomy 21 (T21) Likely Due to Congenital Portosystemic Shunts.","authors":"Rachel Guest, Dilshad Dhaliwal, Darryl Kinnear, Debra Kearney, Nahir Cortes-Santiago, Nidhy Varghese, Kalyani R Patel","doi":"10.1177/10935266251343287","DOIUrl":"10.1177/10935266251343287","url":null,"abstract":"<p><p>Children with Trisomy 21 (T21) have an increased incidence of pulmonary hypertension (PHTN); most commonly due to congenital heart/lung diseases but also in conjunction with other T21-associated gastrointestinal and metabolic abnormalities. This report describes a 2 days old, full-term male neonate with T21, without significant cardiac anomalies presenting with severe hypoxic respiratory failure eventually leading to death. Autopsy confirmed T21-associated dysmorphic features and revealed both extra and intrahepatic congenital portosystemic shunts (CPSS). Additionally, the liver demonstrated dilated hepatic and portal veins and mildly altered lobular architecture without any focal lesions. Bilateral lungs showed mild alveolar maldevelopment, double capillary loops, and normal lung to body weight ratio without vascular hypertensive changes. This case underscores the importance of careful examination of the liver hilum and considering CPSS as a potential etiology of PHTN in neonates with T21 in the absence of cardiac defects or significant lung disease.</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":"416-419"},"PeriodicalIF":1.3,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144136627","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}