Focal Nodular Hyperplasia-Like Lesions With Glypican-3 Positivity in Infancy.

Purpose And Context: Glypican-3 is often used to discriminate between neoplastic and nonneoplastic liver. In focal lesions, positivity may be considered suggestive of a malignancy such as hepatoblastoma. However, glypican-3 is also normally expressed in the immature liver.

A Case of an Infantile Lingual Leiomyomatous Hamartoma.

Lingual leiomyomatous hamartomas are rare lesions of the tongue with largely unknown mechanisms of formation. These lesions are often asymptomatic, though they may present with symptoms, particularly relating to swallow function. Workup should include imaging of the head and neck, and diagnosis should be made histologically.

KRIT1-positive hyperkeratotic cutaneous capillary venous malformation.

Cerebral cavernous malformations (CCM) may present in sporadic or familial forms, with different cutaneous manifestations including deep blue nodules, capillary malformations, and hyperkeratotic cutaneous capillary venous malformations (HCCVM). We report the case of an infant with a KRIT1-positive HCCVM associated with familial CCM. Moreover, histopathology showed positive immunohistochemical stain with GLUT1, further expanding the differential diagnosis of GLUT1-positive vascular anomalies.

Prostatic polyp presenting in a young boy with urinary symptoms.

Congenital urethral polyps are a rare anomaly of the urethra seen primarily in male children that present most commonly with features of voiding dysfunction or urinary obstruction. We report a case of a young boy with a posterior urethral polyp presenting with urinary retention, hematuria, and constipation. We also report a new treatment approach to this rare and challenging clinical finding.

Magnetic Resonance Elastography of the Liver in Children and Adolescents: Assessment of Regional Variations in Stiffness.

Rationale And Objectives: We describe our experience in measuring parenchyma stiffness across the liver Couinaud segments in lieu of the conventional practice of using a single slice-wise "global" region-of-interest. We hypothesize that the heterogeneous nature of fibrosis can lead to regional stiffness within the organ, and that it can be reflected by Couinaud segment-based magnetic resonance elastography measurements.

Materials And Methods: This retrospective study involved from 173 patients (116 males, 57 females, 1.

Ultrasound Elastography as a Non-Invasive Method to Monitor Liver Disease in Children with Short Bowel Syndrome: Updated Results.

Purpose: The purpose of the study was to determine the accuracy of acoustic radiation force impulse (ARFI) ultrasound elastography in assessing the degree of liver disease in children with short bowel syndrome (SBS).

Methods: A prospective observational cohort study of patients with SBS who underwent a liver biopsy and ARFI elastography was performed. Mean shear wave speed (SWS) and stage of fibrosis was evaluated using t-tests.

Aneursymal Bone Cyst: An Uncommon Paraspinal Tumor in Children.

Paraspinal tumors with benign histology in the absence of trauma rarely arise in children. Treatment of such benign tumors, in contrast to malignancies, generally consists of surgical resection of the lesion with confirmation of histology via pathologic evaluation. We present a pediatric case of an atraumatic paraspinal mass with a histologic diagnosis of aneurysmal bone cyst, and USP6 gene rearrangement supporting the histologic diagnosis.

A Strategy for Helicobacter Immunohistochemistry Utilization in Pediatric Practice: Insights From Morphologic and Cost-Benefit Analyses.

Objectives: Recent studies in adults have examined the utility of immunohistochemistry (IHC) in detecting Helicobacter in gastric biopsy specimens and reached differing conclusions. Dedicated cost-benefit analysis of Helicobacter IHC in pediatric gastric biopsy specimens has not been performed.

Methods: From 1,955 pediatric gastric biopsies in a 1-year period, we identified 63 Helicobacter -positive and 120 Helicobacter -negative biopsy specimens.

A pilot study of ultrasound elastography as a non-invasive method to monitor liver disease in children with short bowel syndrome.

Background: Our objective was to evaluate the accuracy of acoustic radiation force impulse (ARFI) elastography in determining extent of liver fibrosis in pediatric patients with short bowel syndrome (SBS).

Methods: This prospective cohort study included pediatric patients with SBS who underwent ultrasound with ARFI measurements of shear wave speed (SWS) and liver biopsy within 30days of each other between 12/2014-9/2015. The mean and median SWS were compared to the stage of fibrosis.

The College of American Pathologists guidelines for whole slide imaging validation are feasible for pediatric pathology: a pediatric pathology practice experience.

Whole slide imaging (WSI) is rapidly transforming educational and diagnostic pathology services. Recently, the College of American Pathologists Pathology and Laboratory Quality Center (CAP-PLQC) published recommended guidelines for validating diagnostic WSI. We prospectively evaluated the guidelines to determine their utility in validating pediatric surgical pathology and cytopathology specimens.

Disseminated Rhizomucor pusillus causing early multiorgan failure during hematopoietic stem cell transplantation for severe aplastic anemia.

Matched sibling donor hematopoietic stem cell transplantation is the standard of care for severe aplastic anemia, with an overall survival of 80% to 90%. Only 60% to 70% of patients respond to treatment with immunosuppressive therapy. The main life threatening complications are infections, graft failure, and graft versus host disease.

Chemoresistant hepatoblastoma in a patient with mosaic trisomy 18 treated with orthotopic liver transplantation.

We present a 9-month-old male with mosaic trisomy 18 with a right hepatic lobe mass. The tumor was completely resected and identified as pure fetal histology hepatoblastoma but contained increased mitotic activity. Adjuvant chemotherapy consisted of cisplatin, vincristine, and 5-fluorouracil.

Infantile osteopetrosis and juvenile xanthogranuloma presenting together in a newborn: a case report and literature review.

Osteopetrosis (OP) is a clinically and genetically heterogeneous disorder characterized by increased bone density. Associations between OP and other clinical entities are rare but include muscular degeneration, Dandy-Walker syndrome, craniosynostosis, and poikiloderma. Infantile OP has also been diagnosed in a group of infants with neuronal storage disease.

Primary testicular presentation of ALK-1-negative anaplastic large cell lymphoma in a pediatric patient.

Anaplastic large cell lymphoma is a heterogeneous group of malignant non-Hodgkin lymphomas that occurs in up to 15% of all pediatric non-Hodgkin lymphomas. It is characterized by B-symptoms and involvement of extranodal sites such as skin, bone, and soft tissue. This brief report describes first reported case of pediatric primary testicular anaplastic large cell lymphoma in a 14-year-old boy.

Autoimmune lymphoproliferative syndrome (ALPS): a case with congenital onset.

We describe a case of autoimmune lymphoproliferative syndrome (ALPS), which is very unusual with regard to a clinical onset soon after birth, and a clinical picture dominated by splenomegaly, jaundice, and consumptive peripheral blood cytopenias, with minimal lymphadenopathy. Our documented close follow up demonstrated initial involvement of the spleen, followed by involvement of the bone marrow and the peripheral blood. The patient underwent bone marrow transplant and is alive and well 20 months after diagnosis.