Advances and Pitfalls in the Diagnosis of Hirschsprung Disease
This article reviews pathologic features of HSCR, considers various specimens the pathologist is required to evaluate, and discusses useful ancillary tests. Potential diagnostic pitfalls are highlighted, and helpful hints are provided to successfully navigate challenging situations. Finally, the article looks forward to new ancillary tests on the horizon and future topics for HSCR research. (Source: Surgical Pathology Clinics)
Source: Surgical Pathology Clinics - October 8, 2020 Category: Pathology Authors: Samuel Hwang, Raj P. Kapur Source Type: research
Pediatric Cystic Lung Lesions
Pediatric cystic lung lesions have long been a source of confusion for clinicians, radiologists, and pathologists. They encompass a wide spectrum of entities with variable prognostic implications, including congenital lung malformations, pulmonary neoplasms, and hereditary conditions. As our understanding of the developmental and genetic origins of these conditions has evolved, revised nomenclature and classifications have emerged in an attempt to bring clarity to the origin of these lesions and guide clinical management. (Source: Surgical Pathology Clinics)
Source: Surgical Pathology Clinics - October 8, 2020 Category: Pathology Authors: Nahir Cortes-Santiago, Gail H. Deutsch Source Type: research
Updates in Pediatric Congenital Enteropathies
Congenital enteropathies comprise a heterogeneous group of disorders typically resulting in severe diarrhea and intestinal failure. Recent advances in and more widespread application of genetic testing have allowed more accurate diagnosis of these entities as well as identification of new disorders, provided a deeper understanding of intestinal pathophysiology through genotype-phenotype correlations, and permitted the exploration of more specific therapies to diseases that have heretofore been resistant to conventional treatments. The therapeutic armamentarium for these disorders now includes intestinal and hematopoietic s...
Source: Surgical Pathology Clinics - October 8, 2020 Category: Pathology Authors: Pierre Russo Source Type: research
New Prognostic Indicators in Pediatric Adrenal Tumors
Pediatric adrenal tumors are unique entities with specific diagnostic, prognostic, and therapeutic challenges. The adrenal medulla gives rise to peripheral neuroblastic tumors (pNTs), pathologically defined by their architecture, stromal content, degree of differentiation, and mitotic-karyorrhectic index. Successful risk stratification of pNTs uses patient age, stage, tumor histology, and molecular/genetic aberrations. The adrenal cortex gives rise to adrenocortical tumors (ACTs), which present diagnostic and prognostic challenges. Histologic features that signify poor prognosis in adults can be meaningless in children, wh...
Source: Surgical Pathology Clinics - October 8, 2020 Category: Pathology Authors: Jason A. Jarzembowski Source Type: research
Pediatric Renal Tumors
Molecular characterization has led to advances in the understanding of pediatric renal tumors, including the association of pediatric cystic nephromas with DICER1 tumor syndrome, the metanephric family of tumors with somatic BRAF mutations, the characterization of ETV6-NTRK3 –negative congenital mesoblastic nephromas, the expanded spectrum of gene fusions in translocation renal cell carcinoma, the relationship of clear cell sarcoma of the kidney with other BCOR-altered tumors, and the pathways affected by SMARCB1 alterations in rhabdoid tumors of the kidney. These adv ances have implications for diagnosis, classification...
Source: Surgical Pathology Clinics - October 8, 2020 Category: Pathology Authors: Amy L. Treece Source Type: research
Round Cell Sarcomas
Undifferentiated sarcomas of soft tissue and bone have been defined as tumors with no identifiable morphologic, immunohistochemical, or molecular features indicating tumor cell origin. In young patients, these tumors frequently have a round or spindle cell morphology. Recently described recurrent translocations within this category have led to the recognition of new molecular subtypes of round cell sarcomas, and several of them have a more aggressive clinical course and less chemosensitivity. Because these “newcomers” are diagnosed based on their molecular characteristics, molecular investigation is key in the diagnosi...
Source: Surgical Pathology Clinics - October 8, 2020 Category: Pathology Authors: Anita Nagy, Gino R. Somers Source Type: research
Histopathologic and Molecular Features of Central Nervous System Embryonal Tumors for Integrated Diagnosis Reporting
Embryonal tumors of the pediatric central nervous system are challenging clinically and diagnostically. These tumors are aggressive, and patients often have poor outcomes even with intense therapy. Proper tumor classification is essential to patient care, and this process has undergone significant changes with the World Health Organization recommending histopathologic and molecular features be integrated in diagnostic reporting. This has especially impacted the workup of embryonal tumors because molecular testing has resulted in the identification of clinically relevant tumor subgroups and new entities. This review summari...
Source: Surgical Pathology Clinics - October 8, 2020 Category: Pathology Authors: Bonnie L. Cole, Christopher R. Pierson Source Type: research
Updates in Pediatric Glioma Pathology
Gliomas are a diverse group of primary central nervous system tumors with astrocytic, oligodendroglial, and/or ependymal features and are an important cause of morbidity/mortality in pediatric patients. Glioma classification relies on integrating tumor histology with key molecular alterations. This approach can help establish a diagnosis, guide treatment, and determine prognosis. New categories of pediatric glioma have been recognized in recent years, due to increasing application of molecular profiling in brain tumors. The aim of this review is to alert pediatric pathologists to emerging diagnostic concepts in pediatric g...
Source: Surgical Pathology Clinics - October 8, 2020 Category: Pathology Authors: Melanie H. Hakar, Matthew D. Wood Source Type: research
Challenges in the Diagnosis of Pediatric Spindle Cell/Sclerosing Rhabdomyosarcoma
Rhabdomyosarcoma (RMS) is the most common pediatric soft tissue sarcoma, representing approximately 40% of all pediatric soft tissue sarcomas. The spindle cell/sclerosing subtype of RMS (SSRMS) accounts for roughly 5% to 10% of all cases of adult and pediatric RMS. Historically, SSRMS were described as paratesticular tumors with an excellent outcome. However, more recent studies have identified unique molecular subgroups of SSRMS, including those with MYOD1 mutations or VGLL2/NCOA2 fusions, which have widely disparate outcomes. The goal of this article is to better describe the biological heterogeneity of SSRMS, which may ...
Source: Surgical Pathology Clinics - October 8, 2020 Category: Pathology Authors: Sonja Chen, Erin R. Rudzinski, Michael A. Arnold Source Type: research
Preface
Pediatric pathology is a distinct and challenging field, encountering a diversity of pathologic conditions, including developmental anomalies, metabolic diseases, malformations, and a set of neoplasms uniquely germane to infants and children. Recent genetic/molecular discoveries have accelerated our knowledge of developmental biology and pediatric pathology. New advancements encompass, but are not limited to, the clinicopathologic and genetic bases of pediatric enteropathies (Updates in Pediatric Enteropathies: Differential Diagnosis, Testing, and Genetics by Pierre Russo), recently described vascular anomalies with recurr...
Source: Surgical Pathology Clinics - October 8, 2020 Category: Pathology Authors: Jessica L. Davis Source Type: research
Grossing of Gastrointestinal Specimens
The proper handling of the gross specimen is imperative, as it is the most important first step in providing excellent patient care. Our diagnoses depend on the correct description and submission of tissue sections for histologic analysis. A logical and problem-solving approach to handling the gross specimen is presented. (Source: Surgical Pathology Clinics)
Source: Surgical Pathology Clinics - August 13, 2020 Category: Pathology Authors: Emma Elizabeth Furth Source Type: research
Diagnosis and Management of Gastrointestinal Neuroendocrine Neoplasms
The latest WHO classification cleanly divides gastrointestinal neuroendocrine neoplasms into neuroendocrine tumor (NET; well-differentiated, any grade) and neuroendocrine carcinoma (NEC; poorly differentiated, high-grade by definition), along with mixed neuroendocrine –non-neuroendocrine neoplasms. NECs are always aggressive, with multiple mutations; they are treated with chemotherapy. NETs have widely different presentations, behavior, and management depending on site of origin. Esophageal examples are vanishingly rare. Most gastric and appendiceal tumors are indolent, as are many colonic and rectal tumors. The duodenum...
Source: Surgical Pathology Clinics - August 13, 2020 Category: Pathology Authors: Raul S. Gonzalez Source Type: research
Impact of Subspecialty Sign-Out on Interobserver Variability and Accuracy in Gastrointestinal Pathology
Subspecialty sign-out is increasingly common in academic medical centers as well as some community practices. Reducing interobserver variability in anatomic pathology is desirable so that clinicians can select the appropriate therapy. Many departments that elect subspecialty sign-out do so with the assumption that it will improve diagnostic accuracy and interobserver variability —but does it? The literature is mixed. (Source: Surgical Pathology Clinics)
Source: Surgical Pathology Clinics - August 13, 2020 Category: Pathology Authors: Christa L. Whitney-Miller Source Type: research
Gastrointestinal Pathology
SURGICAL PATHOLOGY CLINICS (Source: Surgical Pathology Clinics)
Source: Surgical Pathology Clinics - August 13, 2020 Category: Pathology Authors: Raul S. Gonzalez Source Type: research
Copyright
ELSEVIER (Source: Surgical Pathology Clinics)
Source: Surgical Pathology Clinics - August 13, 2020 Category: Pathology Source Type: research
