Aggressive Pediatric Primitive Round Cell Tumors with MN1::ZNF341 Fusion: A Mimic of Neuroblastoma.

Neuroblastoma is one of the most common tumors in young children, arising from the adrenal medulla or paraspinal sympathetic ganglia. We describe primitive round cell tumors presenting in three patients less than 1.5 years old, with striking clinical and pathologic similarities to neuroblastoma.

Histopathology imaging and clinical data including remission status in pediatric inflammatory bowel disease.

The incidence of inflammatory bowel disease (IBD) is increasing annually. Children with IBD often suffer significant morbidity due to physical and emotional effects of the disease and treatment. Corticosteroids, often a component of therapy, carry undesirable side effects with long term use.

Embolization of a third ventricular choroid plexus papilloma: illustrative case.

Background: Choroid plexus tumors are rare neoplasms that are typically found in the lateral ventricles. They have infrequently been reported in the third ventricle, and treatment strategies have varied.

Observations: The authors described a 6-month-old patient who presented with irritability and hypotonia.

UHRF1 overexpression promotes osteosarcoma metastasis through altered exosome production and AMPK/SEMA3E suppression.

Loss-of-function mutations at the retinoblastoma (RB1) gene are associated with increased mortality, metastasis, and poor therapeutic outcome in several cancers, including osteosarcoma. However, the mechanism(s) through which RB1 loss worsens clinical outcome remains understudied. Ubiquitin-like with PHD and Ring Finger domains 1 (UHRF1) has been identified as a critical downstream effector of the RB/E2F signaling pathway that is overexpressed in various cancers.

Not All that Strictures Is IBD: Gastric Heterotopia Leading to Perforation and Small Bowel Obstruction Mimicking IBD.

A 14-year-old male presented with worsening chronic intermittent abdominal pain, mild anemia, positive fecal occult blood test, and elevated calprotectin. Computerized tomography and magnetic resonance imaging showed ileal dilation with mucosal enhancement and inflammatory changes suspicious for inflammatory bowel disease (IBD). Prominent mucosal folds were suggestive of gastric heterotopia, but Meckel's scan was negative.

A Liver Transplant for Local Control in a Pediatric Patient with Metastatic TFE3-Associated Perivascular Epithelioid Cell Tumor (PEComa) to the Liver.

Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors with widespread distribution throughout the body and unpredictable clinical behavior. Recently, a subset of these tumors has been reported to harbor Transcription Factor E3 (TFE3) gene rearrangement with distinct morphologic and immunophenotypic features. Although limited, these tumors may represent a separate entity from the conventional PEComas and may require different treatment approaches.

A limited panel of INSM1 and LEF1 immunostains accurately distinguishes between pancreatic neuroendocrine tumor and solid pseudopapillary neoplasm.

Solid pseudopapillary neoplasm (SPN) and well differentiated pancreatic neuroendocrine tumor (PNET) can show significant cytomorphological overlap. In this study, we evaluated the role of INSM1 and LEF1 immunohistochemical stains in distinguishing between these two tumors. 22 SPN and 25 PNET surgically resected cases were stained for both INSM1 and LEF1.

Automated leukocyte parameters are useful in the assessment of myelodysplastic syndromes.

Background: Study utility of seven automated VCS parameters (V-volume, C-conductivity and S-scatter) in leukocytes as an objective read-out of dysplasia in Myelodysplastic Syndromes (MDS).

Methods: Peripheral blood was analyzed by Beckman-Coulter DxH800 hematology analyzer in 43 patients with low-grade, high-grade MDS and 21 control individuals. The differences in mean (MN) and standard deviation (SD) of each parameter were examined.

Associations Between Placental Corticotropin-Releasing Hormone, Maternal Cortisol, and Birth Outcomes, Based on Placental Histopathology.

Preterm birth remains the leading cause of neonatal morbidity and mortality, with complex biochemical pathways requiring continued understanding and assessment. The objective of this study is to assess the associations between maternal cortisol and placental corticotropin-releasing hormone (placental CRH) concentrations with birth outcomes when stratified by placental histopathology. We conducted an analysis of 112 singleton pregnancies who received betamethasone between 23 and 34 weeks' gestation.

High-grade B-cell Lymphoma with and Rearrangement Arising from Follicular Lymphoma: Presentation as a Large Peripancreatic Mass.

Follicular lymphoma, the second most common non-Hodgkin lymphoma (NHL), primarily affects adults and shows an indolent clinical course. Rare cases of follicular lymphoma transform to a high-grade B-cell lymphoma with and rearrangements or "double-hit lymphoma". Transformation to a "double-hit lymphoma" portends a worse prognosis and requires aggressive treatment.

Congenital neuroglial choristoma of the foot.

Neuroglial choristomas are rare malformations of heterotopic neural tissue that have been previously reported predominantly in the head and neck. Competing theories of embryogenesis propose their origin as encephaloceles that have undergone resorption of their cranial connection or displaced neuroectodermal cells which have undergone ectopic proliferation. Most cases occur in midline or para-midline structures.

Epstein-Barr virus incidental expression in bone marrow cells: a study of 230 consecutive bone marrow biopsy samples.

Epstein-Barr virus (EBV) is associated with many neoplastic hematologic conditions, but scattered EBV-positive cells can be detected in lymph nodes of healthy individuals and they usually represent latently infected lymphocytes. The incidence of EBV detection in normal bone marrow samples has not been studied and is largely unknown. The lack of knowledge regarding the true incidence of encountering bystander latent EBV-positive cells in the bone marrow may potentially lead to a diagnostic dilemma when assessing a staging bone marrow for a patient with an EBV-positive B or T/NK-cell lymphoma.

Gelatinous bone marrow transformation and emergence of clonal Philadelphia-negative cytogenetic abnormalities with excess blasts in a patient with chronic myeloid leukemia treated with dasatinib.

Gelatinous bone marrow transformation (GBMT) is a rare pathologic entity of unclear etiology characterized by adipose cell atrophy, focal hematopoietic tissue hypoplasia, and a distinct eosinophilic substance that stains with Alcian blue at pH 2.5. It is traditionally described in the context of malnutrition and cachexia from generalized disease and is important to identify because of its potential reversibility.

Epstein - Barr virus association with plasma cell neoplasms.

Aims: Epstein-Barr virus (EBV) expression has been reported in several hematopoietic and non-hematopoietic disorders but its expression in plasma cell neoplasms has been largely limited to immunodeficiency-related cases such as in the setting of post-organ transplantation or human immunodeficiency virus (HIV) infection. The aim of this study is to evaluate the association of EBV with plasma cell neoplasms, mainly in immunocompetent patients.

Methods And Results: We retrospectively studied 147 cases of patients with different plasma cell neoplasms (109 plasma cell myelomas, 22 plasmacytomas, and 16 monoclonal gammopathy cases).