143 research outputs found
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor and Related Tumors
No full textEwing sarcoma/peripheral primitive neuroectodermal tumor (EWS/pPNET) and other tumors with EWS gene rearrangements encompass a malignant and intermediate neoplasm with a broad anatomic distribution and a wide age range but a predilection for soft tissue in children, adolescents, and young adults. The overlapping histologic, immunohistochemical and cytogenetic and molecular genetic features create diagnostic challenges despite significant clinical and prognostic differences. Ewing sarcoma is the 3rd most common sarcoma in children and adolescents, and desmoplastic small round cell tumor is a rare neoplasm that occurs more often in older children, adolescents, and young adults. Pathologic examination is complemented by immunohistochemistry, cytogenetics, and molecular genetics. This article reviews the clinicopathologic features of EWS/pPNET and desmoplastic small round cell tumor in the spectrum of tumors with EWS gene rearrangements. Other tumors with different histopathologic features and an EWS gene rearrangement are discussed elsewhere in this volume
Vascular and Perivascular Lesions of Skin and Soft Tissues in Children and Adolescents
No full textVascular anomalies in children and adolescents are the most common soft tissue lesions and include reactive, malformative, and neoplastic tumefactions, with a full spectrum of benign, intermediate, and malignant neoplasms. These lesions are diagnostically challenging because of morphologic complexity and recent changes in classification systems, some of which are based on clinical features and others on pathologic findings. In recent decades, there have been significant advances in clinical diagnosis, development of new therapies, and a better understanding of the genetic aspects of vascular biology and syndromes that include unusual vascular proliferations. Most vascular lesions in children and adolescents are benign, although the intermediate locally aggressive and intermediate rarely metastasizing neoplasms are important to distinguish from benign and malignant mimics. Morphologic recognition of a vasoproliferative lesion is straightforward in most instances, and conventional morphology remains the cornerstone for a specific diagnosis. However, pathologic examination is enhanced by adjunctive techniques, especially immunohistochemistry to characterize the type of vessels involved. Multifocality may cause some uncertainty regarding the assignment of "benign" or "malignant." However, increased interest in vascular anomalies, clinical expertise, and imaging technology have contributed greatly to our understanding of these disorders to the extent that in most vascular malformations and in many tumors, a diagnosis is made clinically and biopsy is not required for diagnosis. The importance of close collaboration between the clinical team and the pathologist cannot be overemphasized. For some lesions, a diagnosis is not possible from evaluation of histopathology alone, and in a subset of these, a specific diagnosis may not be possible even after all assembled data have been reviewed. In such instances, a consensus diagnosis in conjunction with clinical colleagues guides therapy. The purpose of this review is to delineate the clinicopathologic features of vascular lesions in children and adolescents with an emphasis on their unique aspects, use of diagnostic adjuncts, and differential diagnosis
Some general considerations about the clinicopathologic aspects of soft tissue tumors in children and adolescents.
No full textSoft tissue tumors in children and adolescents are an important group of neoplasms, pseudoneoplasms, and tumefactive malformations with some distinctive clinicopathologic, genetic, syndromic, and therapeutic implications. In addition to the basic pathologic examination, there is the availability of diagnostic adjuncts in various settings based upon the histopathologic features that facilitate and/or corroborate a diagnosis. Immunohistochemistry, cytogenetics, molecular genetics, and an ever-increasing array of new technologies are available to address specific diagnostic questions and even potential therapeutic strategies. This review focuses upon some of the unique aspects of soft tissue tumors in children, including the classification, approach to the diagnosis, grading, clinical and pathologic staging, therapy-related changes, pathogenesis, and risk factors
sj-xlsx-1-pdp-10.1177_10935266221132602 – Supplemental material for Calretinin Staining in Anorectal Line Biopsies Accurately Distinguished Hirschsprung Disease in a Retrospective Study
No full textSupplemental material, sj-xlsx-1-pdp-10.1177_10935266221132602 for Calretinin Staining in Anorectal Line Biopsies Accurately Distinguished Hirschsprung Disease in a Retrospective Study by Suzanna J. Logan, Hong Yin, Beverly Rogers, Nicoleta Arva, Miriam R. Conces, Sandy Cope-Yokoyama, Louis P. Dehner, Carlos Galliani, Shipra Garg, Mai He, Aliya N. Husain, Matthew Keisling, Chandra Krishnan, Elena Puscasiu, Christopher Rossi, Faiza Siddiqui, Lisa Sutton, Jefferson Terry, Ameet I. Thaker, Yuan Huang, Jie Zhang, Courtney McCracken and Heather Rytting in Pediatric and Developmental Pathology</p
Prune belly syndrome: clinicopathologic study of 29 cases.
No full textThe clinical course and the pathologic features of 29 patients with the prune belly syndrome (PBS) are reviewed. There were 26 males and 3 females. In addition to the classical triad of deficient abdominal musculature, urinary tract abnormalities, and cryptorchidism, a broader spectrum of other defects was found including musculoskeletal (58\%) and gastrointestinal (31\%) abnormalities. Genital anomalies were present in all three female patients. Many of these defects may be inapparent at birth, but are the cause of morbidity and mortality later in life. Severe urinary tract maldevelopment and pulmonary hypoplasia as part of the oligohydramnios syndrome was the most common cause of perinatal deaths. In these patients, major portions of the renal parenchyma were dysplastic, but in survivors, renal dysplasia, when present, was minor by comparison, and affected less than 1/3 of the parenchyma. Although several questions remain unanswered, we believe that the PBS results from the effect of one or more teratogenic agents on the somatic mesoderm, producing inappropriate mesenchymal development and inadequate mesenchymal-epithelial interactions that lead to abnormal development and dilatation of some of its derivatives (abdominal muscles, ureter, bladder, prostate, urethra, and gubernaculum). Although abnormalities in derivatives of the intermediate mesoderm (kidney) may also be produced by the injurious agent(s), they are more likely a result of urinary obstruction. Abnormalities in other organs and systems are the consequence of oligohydramnios
Primitive Neuroectodermal Tumors of the Central Nervous System in Childhood. Retrospective and Overview
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