1,743 results match your criteria Pediatric and Developmental Pathology [Journal]


Kurt Benirschke, MD (1924-2018): Pioneer in Perinatal and Placental Pathology.

Pediatr Dev Pathol 2019 Apr 19:1093526619843723. Epub 2019 Apr 19.

1 Surgical Pathology, Department of Pathology and Laboratory Medicine, New York Presbyterian Hospital-Weill Cornell Medical College, Weill Cornell Medicine, New York, New York.

Kurt Benirschke, noted pathologist and animal conservationist, passed away on September 10, 2018 at the age of 94. Kurt Benirschke is a legendary figure in perinatal pathology and was likely the first pathologist to have a genuine interest in the placenta. With Shirley Driscoll, he wrote the first textbook on placental pathology-The Pathology of the Human Placenta published in 1967. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526619843723DOI Listing
April 2019
1 Read

INSM1 Expression in Peripheral Neuroblastic Tumors and Other Embryonal Neoplasms.

Pediatr Dev Pathol 2019 Apr 11:1093526619843725. Epub 2019 Apr 11.

1 Department of Pathology, Stanford University School of Medicine, Stanford, California.

Insulinoma-associated protein 1 (INSM1) is a transcription factor that functions in neuroepithelial tissue development and shows expression in neuroendocrine neoplasms. Given the role of INSM1 in controlling differentiation of the sympatho-adrenal lineage, we hypothesized that INSM1 expression would define a subset of neuroblastic tumors. This study aimed to characterize the immunohistochemical profile of INSM1 in a cohort of peripheral neuroblastic tumors and compare INSM1 expression in these tumors to that seen in other embryonal neoplasms, using both tissue microarrays and whole-slide histologic sections. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526619843725DOI Listing
April 2019
1 Read

Neuropathological Findings in a Case of IFIH1-Related Aicardi-Goutières Syndrome.

Pediatr Dev Pathol 2019 Apr 5:1093526619837797. Epub 2019 Apr 5.

4 Departments of Pathology, Neurology, Neurosurgery, University of Colorado, Aurora, Colorado.

Aicardi-Goutières syndrome (AGS) is a rare syndrome characterized by calcification, diffuse demyelination, and variable degree of brain atrophy. The syndrome is genetically heterogeneous with mutations in 7 genes, including TREX1, RNASEH2A, RNASEH2B, RNASEH2C, SAMHD1, ADAR1, and IFIH1 (interferon-induced helicase c domain-containing protein 1) associated with the syndrome, so far. These mutations lead to the overproduction of α-interferon within the central nervous system. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526619837797DOI Listing
April 2019
4 Reads

Fatal Exsanguination Following Rupture of an Iliac Artery Aneurysm in an Infant With Menkes Disease.

Pediatr Dev Pathol 2019 Apr 1:1093526619841152. Epub 2019 Apr 1.

1 Department of Paediatric Surgery, John Hunter Children's Hospital, Newcastle, Australia.

Menkes disease (MD) usually presents in infancy with respiratory and neurological complications. Severe isolated vasculo-connective tissue involvement in infancy is rare, and hence the precise and timely diagnosis is difficult. We report a case of an 8-week-old male infant who succumbed to acute, severe exsanguination, and hemorrhagic shock secondary to a large retroperitoneal hematoma due to rupture of a right iliac artery aneurysm. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526619841152DOI Listing
April 2019
1 Read

Absence of Distinct Immunohistochemical Distribution of Annexin A5, C3b, C4d, and C5b-9 in Placentas From Patients With Antiphospholipid Antibodies, Preeclampsia, and Systemic Lupus Erythematosus.

Pediatr Dev Pathol 2019 Mar 28:1093526619836025. Epub 2019 Mar 28.

1 Department of Pathology and Laboratory Medicine, New York Presbyterian Hospital-Weill Cornell Medical College, New York, New York.

Introduction: In pregnancy, the presence of preeclampsia (PEC), systemic lupus erythematosus (SLE), and/or antiphospholipid antibody syndrome (APLS) is characterized by poor obstetric outcomes, with potential adverse effects for both mother and fetus. Although the histopathologic changes observed in these entities have been well established, the pathogenic mediators associated with tissue injury are poorly understood.

Methods: Forty placentas were evaluated, including 10 patients with preeclampsia, 9 with SLE, 11 with APLS, and 10 disease-free controls. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526619836025DOI Listing

Authors' Response: Prenatal Ultrasound Presentations in Late Pregnancies Affected With Alpha Thalassemia Major.

Pediatr Dev Pathol 2019 Mar 28:1093526619837086. Epub 2019 Mar 28.

5 Department of Obstetrics and Gynecology, University of Calgary, Calgary, Alberta, Canada.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526619837086DOI Listing

Prenatal Ultrasound Presentations in Late Pregnancies Affected With Alpha Thalassemia Major.

Pediatr Dev Pathol 2019 Mar 28:1093526619837782. Epub 2019 Mar 28.

2 Department of Ultrasound, The Sixth Affiliated Hospital of SunYat-sen University, Guangzhou, Guangdong, China.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526619837782DOI Listing

Infra-anastomotic Innervation of Residual Aganglionic Distal Rectum After Pull-through Surgery for Hirschsprung Disease.

Pediatr Dev Pathol 2019 Mar 27:1093526619837788. Epub 2019 Mar 27.

2 Department of Pathology and Laboratory Medicine, Nationwide Children's Hospital, Columbus, Ohio.

Background: Descending neurons are important for intestinal reflex activities, including the recto-anal inhibitory reflex involved in normal defecation. Pull-through surgery for Hirschsprung disease results in the anastomosis of ganglionic bowel to native aganglionic rectum just superior to the internal anal sphincter, which potentially could allow for physiologically significant infra-anastomotic innervation.

Methods: The density and distribution of intramuscular neuronal nitric oxide synthase (nNOS)- and mucosal calretinin-immunoreactive nerves were evaluated proximal and distal to the anastomosis in redo resection specimens after pull-through surgery for Hirschsprung disease. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526619837788DOI Listing

Pulmonary Infantile Hemangioma Mimicking a Congenital Cystic Adenomatoid Malformation.

Pediatr Dev Pathol 2019 Mar 26:1093526619838743. Epub 2019 Mar 26.

2 Department of Pathology, Hôpital Femme Mère Enfant, CHU de Lyon, Bron, France.

Infantile hemangioma (IH) is the most common benign vascular tumor of infancy, occurring predominantly in the head and neck. It is characterized by specific endothelial expression of glucose transporter-1 (GLUT-1) and involution with time, spontaneous or on beta-blockers treatment. Although some predisposing factors are known, the exact pathogenesis remains unclear. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526619838743DOI Listing
March 2019
1 Read

Do Doppler Changes Reflect Pathology of Placental Vascular Lesions in IUGR Pregnancies?

Pediatr Dev Pathol 2019 Mar 20:1093526619837790. Epub 2019 Mar 20.

1 Department of Pathology, St. John's Medical College, Bangalore, Karnataka, India.

Objectives: Doppler assessment of uteroplacental (UP) and fetoplacental (FP) circulation detects abnormal waveforms in intrauterine growth-restricted (IUGR) pregnancies. Similarly, histopathology also reveals lesions of vascular compromise in IUGR placenta. We evaluated an association between Doppler and histopathological (HP) assessment of the maternal and fetal circulation in IUGR. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526619837790DOI Listing
March 2019
1 Read

Clinical Diagnosis of Classical Cornelia de Lange Syndrome Made From Postmortem Examination of Second Trimester Fetus With Novel NIPBL Pathogenic Variant.

Pediatr Dev Pathol 2019 Mar 19:1093526619834429. Epub 2019 Mar 19.

1 Department of Clinical Genetics, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.

Classical Cornelia de Lange syndrome (CdLS) is a rare genetic disorder which is associated with distinctive facial features, growth retardation, significant intellectual disability and global developmental delay, hirsutism, and upper-limb reduction defects. Classical CdLS is associated with pathogenic variants in NIPBL. We present a clinical diagnosis of classical CdLS made in a second trimester male fetus with advanced maceration who had undergone intrauterine death at 15 + 6 weeks gestation. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526619834429DOI Listing
March 2019
2 Reads

Genomic and Immunologic Drivers of Very Early-Onset Inflammatory Bowel Disease.

Pediatr Dev Pathol 2019 Mar 6:1093526619834807. Epub 2019 Mar 6.

1 Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Philadelphia, Pennsylvania.

Purpose Of Review: Inflammatory bowel disease (IBD) is a multifactorial disease caused by dysregulated immune responses to commensal or pathogenic intestinal microbes, resulting in chronic intestinal inflammation. However, a subset of patients with IBD diagnosed <6 years of age, known as very early-onset (VEO)-IBD, can be phenotypically and genetically distinct from older onset IBD. We aim to review the clinical presentation of children with VEO-IBD and recent discoveries that point to the underlying genomic and immunologic drivers of disease, and the significant impact on our therapeutic decisions. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526619834807DOI Listing
March 2019
4 Reads

Primary Adrenal Angiomatoid Fibrous Histiocytoma With Novel EWSR1-ATF1 Gene Fusion Exon-Exon Breakpoint.

Pediatr Dev Pathol 2019 Mar 1:1093526619830287. Epub 2019 Mar 1.

2 Department of Pathology and Laboratory Medicine, KK Women's and Children's Hospital, Singapore, Republic of Singapore.

We describe the clinical, pathological, and molecular features of a primary adrenal angiomatoid fibrous histiocytoma (AFH) in an 11-year-old girl presenting with pyrexia of unknown origin. We performed next-generation sequencing-based anchored multiplex polymerase chain reaction (Archer® FusionPlex® sarcoma assay), which revealed an EWSR1-ATF1 gene fusion with novel breakpoints in exon 11 of EWSR1 and exon 3 of ATF1. The pyrexia resolved fully after surgical resection, and the patient was disease-free on follow-up at 1 year and 6 months. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526619830287DOI Listing
March 2019
4 Reads

Recurrent Non-immune Fetal Hydrops Associated With IPEX Syndrome.

Pediatr Dev Pathol 2019 Feb 27:1093526619834809. Epub 2019 Feb 27.

1 Department of Pathology and Laboratory Medicine, NorthShore University HealthSystem, Evanston, Illinois.

Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) is a clinical syndrome associated with mutations in FOXP3 and consequent abnormalities of T regulatory cells. Affected males typically die in infancy or early childhood from a variety of autoimmune conditions. Reports of recurrent pregnancy loss of male fetuses in these families have been accompanied by descriptions of nonimmune fetal hydrops, with or without additional fetal anomalies. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526619834809DOI Listing
February 2019
1 Read

A Case of Fulminant Bacillus cereus Lung Necrosis in a Preterm Neonate.

Pediatr Dev Pathol 2019 Feb 13:1093526619825895. Epub 2019 Feb 13.

5 Department of Pathology and Laboratory Medicine, The Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada.

Bacillus cereus is a ubiquitous Gram-positive rod seldom considered pathogenic in clinical isolates. However, it possesses multiple virulence factors explaining why it has been linked to fulminant and pyogenic infections in vulnerable hosts. Its recovery from sterile samples in immunocompromised patients cannot be disregarded. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526619825895DOI Listing
February 2019
1 Read

Focal Chorangiosis: Does It Have Clinical and Pathologic Significance?

Pediatr Dev Pathol 2019 Feb 13:1093526619830866. Epub 2019 Feb 13.

1 Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, New York.

Introduction: Chorangiosis is a proliferation of capillaries in terminal chorionic villi and is considered to be a marker for hypoxia and poor clinical outcome. Not all cases with hypervascular villi meet the generally accepted diagnostic criteria as reported by Altshuler. Our aim was to evaluate cases with villous hypervascularity that do not meet the diagnosis of chorangiosis, in which increased vascularity was present in a significant portion of the villous tissue but was not a diffuse process, which we call focal chorangiosis, to ascertain whether there were clinical or pathologic associations. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526619830866DOI Listing
February 2019

Lingual Alveolar Soft Part Sarcoma in a 1-Year-Old Infant: Youngest Reported Case With Characteristic ASPSCR1-TFE3 Fusion.

Pediatr Dev Pathol 2019 Feb 11:1093526619830290. Epub 2019 Feb 11.

1 Department of Paediatric Haematology and Oncology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.

Alveolar soft part sarcoma (ASPS) is an exceptionally rare non-rhabdomyosarcomatous soft tissue sarcoma (NRSTS), characterized by the translocation t(X;17) p(11.2;q25). This translocation results in the chimeric ASPSCR1-TFE3 transcription factor which drives tumorigenesis. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526619830290DOI Listing
February 2019

Extrahepatic Nonreticuloendothelial Siderosis Is Not Specific to Gestational Alloimmune Liver Disease.

Pediatr Dev Pathol 2019 Feb 5:1093526619826429. Epub 2019 Feb 5.

1 Department of Pathology, Texas Children's Hospital, Houston, Texas.

Autopsy reports of 78 stillbirths and early infant deaths (up to age 8 weeks) were reviewed to investigate the prevalence of extrahepatic nonreticuloendothelial siderosis (EHNRS) in the context of neonatal liver failure. Of these, 10 liveborns (12.8%), M:F 3:2, with mean gestational age 37. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526619826429DOI Listing
February 2019
4 Reads

Fractal Dimension Does Not Correlate As Well With Models of Neonatal Chronic Lung Injury as It Does With Placental Distal Villous Hypoplasia.

Pediatr Dev Pathol 2019 Mar-Apr;22(2):171-174. Epub 2019 Feb 2.

3 Department of Pediatrics, Children's Hospital of Eastern Ontario, University of Ottawa, Ottawa, Ontario, Canada.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526618782499DOI Listing
March 2019
1 Read

Rapid Alterations in Cerebral White Matter Lipid Profiles After Ischemic-Reperfusion Brain Injury in Fetal Sheep as Demonstrated by MALDI-Mass Spectrometry.

Pediatr Dev Pathol 2019 Jan 25:1093526619826721. Epub 2019 Jan 25.

1 Department of Medicine, Rhode Island Hospital, Providence, Rhode Island.

Background: Perinatal ischemia-reperfusion (I/R) injury of cerebral white matter causes long-term cognitive and motor disabilities in children. I/R damages or kills highly metabolic immature oligodendroglia via oxidative stress, excitotoxicity, inflammation, and mitochondrial dysfunction, impairing their capacity to generate and maintain mature myelin. However, the consequences of I/R on myelin lipid composition have not been characterized. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526619826721DOI Listing
January 2019
3 Reads

Pathophysiological Mechanism of Extravasation via Umbilical Venous Catheters.

Pediatr Dev Pathol 2019 Jan 25:1093526619826714. Epub 2019 Jan 25.

2 Neonatal Unit, Birmingham Women's Hospital, Birmingham Women's and Children's NHS Foundation Trust, Birmingham, UK.

A rare complication of umbilical venous catheter (UVC) insertion is the extravasation of the infusate into the peritoneal cavity. We report 3 cases of abdominal extravasation of parenteral nutrition (PN) fluid via UVCs. Two of these cases presented as "acute abdomen" which were assumed to be necrotizing enterocolitis clinically; however, during postmortem, PN ascites and liver necrosis were found. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526619826714DOI Listing
January 2019
13 Reads

Noonan Syndrome Case Report: PTPN11 and Other Potential Genetic Factors Contributing to Lethal Hypertrophic Right Ventricular Cardiomyopathy.

Pediatr Dev Pathol 2019 Jan 21:1093526618825411. Epub 2019 Jan 21.

1 Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas, USA.

Noonan syndrome is a genetic condition with a heterogeneous phenotype and multisystem involvement. The pathogenesis of this disorder has been attributed to the mutations in the RAS/MAPK signaling pathway involved in cell proliferation and differentiation. The most common clinical presentations are related to cardiovascular abnormalities with congestive heart failure as the most common mechanism of death. Read More

View Article

Download full-text PDF

Source
http://journals.sagepub.com/doi/10.1177/1093526618825411
Publisher Site
http://dx.doi.org/10.1177/1093526618825411DOI Listing
January 2019
10 Reads

Basal Chronic Villitis and Disorders of the Placental Basal Plate: A Possible Immunological Link Between Hypertensive Disorders of Pregnancy and Morbidly Adherent Placenta.

Pediatr Dev Pathol 2019 Jan 21:1093526619825708. Epub 2019 Jan 21.

1 Division of Surgical Pathology, Department of Pathology and Laboratory Medicine, University of Rochester Medical Center, Rochester, New York.

Background: Hypertensive disorders of pregnancy (HDP) are a common cause for preterm delivery. Prior studies showed that chronic villitis (CV) is associated with intrauterine growth restriction, preeclampsia, intrauterine fetal death, and morbidly adherent placenta (MAP). The authors hypothesize that disorders of the placental basal plate, especially basal chronic villitis (BCV), are associated with HDP. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526619825708DOI Listing
January 2019
2 Reads

Early Bilateral Gonadoblastoma Associated With 45,X/46,XY Mosaicism: The Spectrum of Undifferentiated Gonadal Tissue and Gonadoblastoma in the First Months of Life.

Pediatr Dev Pathol 2019 Jan 15:1093526618824469. Epub 2019 Jan 15.

2 Children's Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.

45,X/46,XY mosaicism is one of a heterogenous group of congenital conditions known as differences (disorders) of sex development (DSD) that results in abnormal development of internal and external genitalia. Patients with DSD, particularly those with segments of the Y chromosome, are at increased risk for germ cell tumors including gonadoblastoma. Gonadoblastoma is a neoplasm comprised of a mixture of germ cells and elements resembling immature granulosa or Sertoli cells with or without Leydig cells or lutein-type cells in an ovarian type stroma. Read More

View Article

Download full-text PDF

Source
http://journals.sagepub.com/doi/10.1177/1093526618824469
Publisher Site
http://dx.doi.org/10.1177/1093526618824469DOI Listing
January 2019
15 Reads
0.857 Impact Factor

Neuroblastoma of the Bone and Bone Marrow Without an Apparent Primary Site: Report of 4 Cases With Long-term Follow-up.

Pediatr Dev Pathol 2019 Jan 2:1093526618822597. Epub 2019 Jan 2.

1 Division of Hematology-Oncology, Department of Pediatrics, Texas Children's Cancer & Hematology Centers, Baylor College of Medicine, Houston, Texas.

Children with neuroblastoma rarely present with metastatic disease without identifiable primary tumors. We describe the clinical and histopathologic characteristics of 4 patients aged 1, 7, 7, and 11 years with neuroblastoma involving bone or bone marrow without an apparent primary site. One patient presented with a periorbital bone lesion, 1 presented with a distal femoral lesion, and 2 presented with diffuse bone marrow involvement. Read More

View Article

Download full-text PDF

Source
http://journals.sagepub.com/doi/10.1177/1093526618822597
Publisher Site
http://dx.doi.org/10.1177/1093526618822597DOI Listing
January 2019
3 Reads

Bone Marrow Morphology Associated With Germline RUNX1 Mutations in Patients With Familial Platelet Disorder With Associated Myeloid Malignancy.

Pediatr Dev Pathol 2019 Jan 2:1093526618822108. Epub 2019 Jan 2.

9 Division of Hematology Oncology, Boston Children's Hospital, Boston, Massachusetts.

Germline mutations in RUNX1 result in autosomal dominant familial platelet disorder with associated myeloid malignancy (FPDMM). To characterize the hematopathologic features associated with a germline RUNX1 mutation, we reviewed a total of 42 bone marrow aspirates from 14 FPDMM patients, including 24 cases with no cytogenetic clonal abnormalities, and 18 with clonal karyotypes or leukemia. We found that all aspirate smears had ≥10% atypical megakaryocytes, predominantly characterized by small forms with hypolobated and eccentric nuclei, and forms with high nuclear-to-cytoplasmic ratios. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526618822108DOI Listing
January 2019
14 Reads

A 10-Month-Old Infant Presenting With Signs of Precocious Puberty Secondary to a Sclerosing Stromal Tumor of the Ovary in the Absence of Hormonal Elevation.

Pediatr Dev Pathol 2018 Dec 21:1093526618819605. Epub 2018 Dec 21.

1 Division of Pediatric Surgery, Children's Hospital Los Angeles, Los Angeles, California.

Precocious puberty in an infant is an alarming and infrequent finding, making the differential diagnosis difficult for practitioners. Precocious puberty secondary to a sclerosing stromal tumor (SST) of the ovary is rare. We present a case of a child that began precocious puberty at 3 months of age including development of breast buds, pubic hair, growth spurt, and menarche 5 days prior to presenting to pediatric endocrinology at 10 months. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526618819605DOI Listing
December 2018

Kidney Biopsy Findings in a Patient With Valproic Acid-Associated Fanconi Syndrome.

Pediatr Dev Pathol 2018 Dec 19:1093526618819334. Epub 2018 Dec 19.

2 Department of Pathology, Zucker School of Medicine, Hofstra University, Hempstead, New York.

A 7-year-old boy with a history of febrile illness-related epilepsy syndrome presented with proteinuria and elevated creatinine. His severe epileptic disorder has been treated since age 2 with multiple antiepileptic medications, including valproic acid. More recently, he was noted to have features of Fanconi syndrome with acidosis, hypophosphatemia, hypokalemia, glucosuria, and nephrotic-range proteinuria. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526618819334DOI Listing
December 2018
2 Reads

Atypical Prenatal Ultrasound Presentation and Neuropathological Findings in a Neonate With Alpha Thalassemia Major: A Case Report.

Pediatr Dev Pathol 2019 Mar-Apr;22(2):166-170. Epub 2018 Dec 14.

5 Department of Obstetrics and Gynecology, University of Calgary, Calgary, Canada.

Alpha thalassemia major is a hemoglobinopathy caused by the inactivation or deletion of all 4 α-globin alleles. We describe a case of α-thalassemia major with atypical ultrasound and neuropathological findings. The mother had her first prenatal visit at 27 4/7 gestational weeks. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526618817655DOI Listing
April 2019
1 Read

Remodeling of Rectal Innervation After Pullthrough Surgery for Hirschsprung Disease: Relevance to Criteria for the Determination of Retained Transition Zone.

Pediatr Dev Pathol 2018 Dec 12:1093526618817658. Epub 2018 Dec 12.

7 Department of Pathology, Microbiology, and Immunology, Vanderbilt University Medical Center, Nashville, Tennessee.

Background: After pullthrough surgery for Hirschsprung disease (HSCR), Glut1-positive submucosal nerve hypertrophy is used to diagnose retained transition zone in the neorectum. We hypothesized that pelvic nerves, severed during pullthrough surgery, sprout into the neorectum to mimic transition zone.

Methods: The density (nerves/100x field) and maximum diameter of Glut1-positive submucosal nerves were measured in biopsies and redo resections from 20 patients with post-pullthrough obstructive symptoms. Read More

View Article

Download full-text PDF

Source
http://journals.sagepub.com/doi/10.1177/1093526618817658
Publisher Site
http://dx.doi.org/10.1177/1093526618817658DOI Listing
December 2018
4 Reads

Changes in the Prevalence of Embryologic Remnants in Umbilical Cord With Gestational Age.

Pediatr Dev Pathol 2018 Dec 12:1093526618813663. Epub 2018 Dec 12.

2 Department of Clinical Genetics, Lund University, Lund, Sweden.

The aim of this study was to examine the prevalence of embryologic remnants in umbilical cords of different gestational ages. Sections from 392 umbilical cords were examined using light microscopy. Of these, 52% contained at least 1 remnant, most commonly of the allantoic duct type. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526618813663DOI Listing
December 2018

Prevalence, Morphology, Embryogenesis, and Diagnostic Utility of Umbilical Cord Vitelline Vascular Remnants.

Authors:
James R Wright

Pediatr Dev Pathol 2018 Dec 12:1093526618811734. Epub 2018 Dec 12.

1 Department of Pathology & Laboratory Medicine, University of Calgary/Calgary Laboratory Services, Alberta Children's Hospital, Calgary, Alberta, Canada.

The objective of this study is to better understand embryonic vitelline vascular remnants in the umbilical cord, to assess their prevalence, to categorize their morphology, and then finally to describe and assess inflammation arising from these structures. During routine placental sign out, the author noted the presence or absence of vitelline vessel remnants for 1 year; when present, he assessed their histologic patterns and noted whether there were neutrophils marginating from the remnants and into the adjacent Wharton's jelly and whether there was any other evidence of amniotic fluid infection in sections of placental disc, membranes, or cord. All cord sections with vitelline vessel remnants were immunostained for CD15 to document any infiltrates, to highlight patterns of infiltration, and to evaluate whether mild cases of umbilical phlebitis were associated with these lesions and were at risk of being missed. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526618811734DOI Listing
December 2018
1 Read

Atypical Hepatic Mesenchymal Hamartoma: Histologic Appearance, Immunophenotype, and Molecular Findings.

Pediatr Dev Pathol 2018 Nov 29:1093526618806750. Epub 2018 Nov 29.

9 Department of Pediatric Surgery, Children's Hospital of Eastern Ontario and University of Ottawa Faculty of Medicine, Ottawa, Ontario, Canada.

Hepatic mesenchymal hamartoma is a rare benign neoplasm principally encountered in young children. Its origin is unknown. We report an unusual hepatic mesenchymal hamartoma in a 7-month-old girl, including histopathologic findings, immunophenotype, and karyotype. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526618806750DOI Listing
November 2018
2 Reads
0.857 Impact Factor

2018 SPP Fall Abstracts.

Authors:

Pediatr Dev Pathol 2018 Nov 27:1093526618806423. Epub 2018 Nov 27.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526618806423DOI Listing
November 2018

Spontaneous Regression of Atypical Teratoid Rhabdoid Tumor Without Therapy in a Patient With Uncommon Regional Inactivation of SMARCB1 ( hSNF5/INI1).

Pediatr Dev Pathol 2019 Mar-Apr;22(2):161-165. Epub 2018 Nov 23.

3 Department of Pathology, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas.

Atypical teratoid/rhabdoid tumor (ATRT) is a high-grade central nervous system tumor, with poor prognosis despite intensive multimodal therapy. Loss of nuclear immunostaining for INI1 due to inactivation of the hSNF5/INI1 tumor suppressor gene is pathognomonic of ATRT. We present a patient with congenital ATRT, who had spontaneous tumor regression without therapy, and is disease-free 4 years later. Read More

View Article

Download full-text PDF

Source
http://journals.sagepub.com/doi/10.1177/1093526618814696
Publisher Site
http://dx.doi.org/10.1177/1093526618814696DOI Listing
April 2019
4 Reads

Fetal Anthropometric Features: A Postmortem Study of Fetuses After the Termination of Pregnancy for Psychosocial Reasons Between 12 and 20 Gestational Weeks.

Pediatr Dev Pathol 2018 Nov 19:1093526618812528. Epub 2018 Nov 19.

3 Division of Clinical Pathology, Geneva University Hospitals, Geneva, Switzerland.

Introduction: Reference ranges in fetal postmortem anthropometric data derive from heterogeneous studies and rely on data obtained after intrauterine fetal death and abortion, which may introduce bias in the reported fetal growth parameters. We report anthropometric findings in fetuses with the least variation due to cause of death or developmental anomalies.

Methods: We analyzed fetuses after the termination of pregnancy for psychosocial reasons. Read More

View Article

Download full-text PDF

Source
http://journals.sagepub.com/doi/10.1177/1093526618812528
Publisher Site
http://dx.doi.org/10.1177/1093526618812528DOI Listing
November 2018
10 Reads

Adequacy and Accuracy of Core Biopsy in Children: A Radiologic/Pathologic Correlation Study.

Pediatr Dev Pathol 2019 Mar-Apr;22(2):137-141. Epub 2018 Nov 14.

1 Department of Pathology, Children's Hospital of Wisconsin and Medical College of Wisconsin, Milwaukee, Wisconsin.

Core biopsy (CB) is increasingly popular for assessing solid lesions in children. To date, pediatric literature is limited regarding factors contributing to diagnostically inadequate or inaccurate CB. Therefore, we retrospectively examined radiologic/pathologic factors associated with adequacy/accuracy of CB in pediatric patients. Read More

View Article

Download full-text PDF

Source
http://journals.sagepub.com/doi/10.1177/1093526618809862
Publisher Site
http://dx.doi.org/10.1177/1093526618809862DOI Listing
April 2019
15 Reads

Genetic Analysis of Copy Number Variation in Large Chorangiomas.

Pediatr Dev Pathol 2018 Nov 14:1093526618811744. Epub 2018 Nov 14.

1 Section of Perinatal Pathology, Department of Pathology, Karolinska University Hospital Huddinge, Stockholm, Sweden.

Introduction: Chorangioma (CA) is the most common nontrophoblastic, vascular tumor-like lesion of the placenta with a reported incidence of 0.5% to 1% in all examined placentas. The underlying molecular mechanisms of CAs are still poorly elucidated, and a systematic investigation of the genetic background of CAs has not previously been done. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526618811744DOI Listing
November 2018
26 Reads

Pediatric Anastomosing Hemangioma: Case Report and Review of Renal Vascular Tumors in Children.

Pediatr Dev Pathol 2018 Oct 28:1093526618809230. Epub 2018 Oct 28.

5 Department of Pathology and Laboratory Medicine, National Kidney and Transplant Institute, Philippines.

The case involves a 10-year-old child who underwent a left radical nephrectomy for what was believed to be a Wilms' tumor. Histopath examination indicated a benign vascular lesion, subsequently determined to be an anastomosing hemangioma of the kidney. A comparison with the previously cited pediatric patients with renal vascular tumors is provided, and the inconsistent diagnostic terminologies for these conditions are highlighted. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526618809230DOI Listing
October 2018
16 Reads

Spectrum of Changes Seen With Placental Intravascular Organisms.

Pediatr Dev Pathol 2018 Oct 18:1093526618801616. Epub 2018 Oct 18.

4 Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.

Fetal bacterial infections are a common cause of fetal/neonatal morbidity and mortality. The pathologic correlates of congenital bacterial infection include acute chorioamnionitis, acute villitis, and acute intervillositis. The strength of the association of congenital bacterial infection differs among these pathologies. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526618801616DOI Listing
October 2018
18 Reads

Primary Nonanaplastic Peripheral Natural Killer/T-Cell Lymphoma in Pediatric Patients-An Unusual Distribution Pattern of Subtypes.

Pediatr Dev Pathol 2019 Mar-Apr;22(2):128-136. Epub 2018 Oct 18.

1 Department of Pathology, University of Colorado School of Medicine, Aurora, Colorado.

Peripheral NK/T-cell lymphoma (PNKTCL) represents a group of uncommon diagnoses for children in Western countries, and studies have often necessitated multiple institutions to assemble enough cases. We retrospectively analyzed 11 cases of nonanaplastic PNKTCL in children over 19 years at our institution with comparison to several published large multi-institutional studies. Patients included 9 males and 2 females of white (5), Native American (3), and Hispanic (3) background with 6 cases of extranodal NK/T-cell lymphoma, nasal type (EN-NKTL, 54. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526618807110DOI Listing
April 2019
1 Read

Paraspinal Leiomyoma.

Pediatr Dev Pathol 2018 Oct 17:1093526618808723. Epub 2018 Oct 17.

1 Department of Pathology, School of Medicine, Uniformed Services University of the Health Sciences, Bethesda, Maryland.

View Article

Download full-text PDF

Source
http://journals.sagepub.com/doi/10.1177/1093526618808723
Publisher Site
http://dx.doi.org/10.1177/1093526618808723DOI Listing
October 2018
3 Reads

Myxoid Lipoblastoma and Mimickers on Fine-Needle Biopsy in a Child.

Pediatr Dev Pathol 2019 Mar-Apr;22(2):157-160. Epub 2018 Oct 15.

1 Service d'Anatomie et de Cytologie Pathologiques, Hôpital d'Enfants Armand Trousseau, Université Pierre et Marie Curie, Paris, France.

Image-guided percutaneous core needle biopsy is a standard and safe procedure for the diagnosis of both solid and hematological malignancies in children. Despite recent improvements, nondiagnosis biopsies persist. Lipoblastoma is a benign adipocytic tumor composed of embryonal fat admixed with mature adipocytes and occurring before the age of 1 year in one-third of cases. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526618805894DOI Listing
April 2019
19 Reads

Variation in Platelet Delta Granules Over Time in Young Women Undergoing Evaluation for Heavy Menstrual Bleeding.

Pediatr Dev Pathol 2019 Mar-Apr;22(2):123-127. Epub 2018 Oct 9.

3 Division of Pediatric Hematology/Oncology, Nationwide Children's Hospital, Columbus, Ohio.

Delta-granule platelet storage pool deficiency (δ-PSPD) is a qualitative platelet function defect associated with variable bleeding phenotypes. Platelet electron microscopy (EM) is commonly utilized to evaluate for δ-PSPD, but intrapatient variability in platelet δ-granule numbers by EM is currently unknown. Fifteen young women aged 11 to 17 years presenting to a young women's hematology clinic for the evaluation of heavy menstrual bleeding underwent platelet EM testing at their initial hematology clinic visit and at 1 and 3 months later. Read More

View Article

Download full-text PDF

Source
http://journals.sagepub.com/doi/10.1177/1093526618803838
Publisher Site
http://dx.doi.org/10.1177/1093526618803838DOI Listing
April 2019
21 Reads

Correlation of Endoscopic and Histologic Severity Scores in Pediatric Ulcerative Colitis at First Presentation.

Pediatr Dev Pathol 2019 Mar-Apr;22(2):106-111. Epub 2018 Oct 9.

1 Division of Pediatric Pathology, Department of Pathology, Microbiology & Immunology, Vanderbilt University Medical Center, Nashville, Tennessee.

Detailed histologic scoring systems have been developed for the assessment of disease activity in ulcerative colitis. Literature from adult patients has shown some correlation between endoscopy and histology, and reproducibility of histologic scoring systems has also been supported. The effectiveness of endoscopic appearance at predicting histologic scores in pediatric patients has not been well studied, and none of the histologic scoring systems used in adults have had interobserver reproducibility assessed in pediatric patients. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526618803736DOI Listing
April 2019
12 Reads

Histological Features of Shallow Placental Implantation Unify Early-Onset and Late-Onset Preeclampsia.

Authors:
Jerzy Stanek

Pediatr Dev Pathol 2019 Mar-Apr;22(2):112-122. Epub 2018 Oct 9.

1 Division of Pathology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

Preeclampsia is distinguishable from other hypertensive conditions of pregnancy by its high rates of decidual arteriopathy, the uterine type of chronic hypoxic placental injury, the occurrence of villous infarctions, and clusters of multinucleate trophoblasts in the maternal floor. To retrospectively study the clinical and placental phenotypes of 230 women with early-onset preeclampsia, 261 women with late-onset preeclampsia, and 5059 women without hypertension in pregnancy (comparative group), 24 clinical and 46 placental phenotypes were statistically compared (analysis of variance, χ with Bonferroni correction). The frequency of decidual arteriopathy (both hypertrophic and atherosis), patterns of chronic hypoxic placental injury, villous infarction, membrane laminar necrosis, membrane microscopic chorionic pseudocysts, clusters of maternal floor multinucleated trophoblasts, excessive number of extravillous trophoblasts, and intervillous thrombi was strikingly higher in both late-onset preeclampsia and early-onset preeclampsia than in the comparative group without hypertension in pregnancy. Read More

View Article

Download full-text PDF

Source
http://journals.sagepub.com/doi/10.1177/1093526618803759
Publisher Site
http://dx.doi.org/10.1177/1093526618803759DOI Listing
April 2019
3 Reads

Succinate Dehydrogenase-Deficient Gastrointestinal Stromal Tumor With SDHC Germline Mutation and Bilateral Renal and Neck Cysts.

Pediatr Dev Pathol 2018 Oct 9:1093526618805354. Epub 2018 Oct 9.

2 Department of Pathology, UPMC Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania.

Gastrointestinal stromal tumors (GISTs) are rare in children. Succinate dehydrogenase (SDH)-deficient GISTs are wild type and lack KIT proto-oncogene receptor tyrosine kinase and platelet-derived growth factor receptor A ( KIT or PDGFRA) mutations. These tumors result from germline SDH mutations, somatic SDH mutations, or SDH epimutants. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526618805354DOI Listing
October 2018
22 Reads

Early Onset Allergic Proctitis in a Preterm Neonate-A Case Report and Review of the Literature.

Pediatr Dev Pathol 2019 Mar-Apr;22(2):152-156. Epub 2018 Oct 4.

3 Department of Pediatrics, Division of Gastroenterology, Children's Hospital of Eastern Ontario Research Institute, Ottawa, Canada.

Cow's milk protein allergy/intolerance (CMPA/CMPI) is a common entity in the pediatric population with a nonspecific presentation ranging from gastrointestinal symptoms to systemic manifestations. Most infants with CMPI are term, and symptoms often appear in the week following the introduction of cow's milk-based formula. There is typically a significant delay in the onset of milk allergy in premature infants compared to full term. Read More

View Article

Download full-text PDF

Source
http://journals.sagepub.com/doi/10.1177/1093526618803770
Publisher Site
http://dx.doi.org/10.1177/1093526618803770DOI Listing
April 2019
3 Reads
0.860 Impact Factor

Placental Histology After Minor Trauma in Pregnancy: A Pilot Study.

Pediatr Dev Pathol 2018 Sep 12:1093526618799292. Epub 2018 Sep 12.

1 Department of Obstetrics and Gynaecology, VU University Medical Center, Amsterdam, the Netherlands.

Objective Trauma in pregnancy may cause placental abruption. Consequences of moderate placental injury on neurodevelopment are unknown. The aim was to evaluate placental histology after maternal trauma. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1093526618799292DOI Listing
September 2018
14 Reads

Postmortem Diagnosis of Heart-hand Syndrome Associated With a 7p22.1p22.3 Deletion in a 16-week-old Fetus.

Pediatr Dev Pathol 2019 Mar-Apr;22(2):146-151. Epub 2018 Sep 7.

2 Service d'anatomopathologie, CHU Charles Nicolle, Rouen, France.

We report a male fetus with a 6.8 Mb deletion on chromosome 7p22.1p22. Read More

View Article

Download full-text PDF

Source
http://journals.sagepub.com/doi/10.1177/1093526618799293
Publisher Site
http://dx.doi.org/10.1177/1093526618799293DOI Listing
April 2019
7 Reads