scholarly journals Radiohistogenomics of pediatric low-grade neuroepithelial tumors

2021 ◽  
Author(s):  
Asim K. Bag ◽  
Jason Chiang ◽  
Zoltan Patay
Keyword(s):  
Holistic Approach ◽  
Genetic Alterations ◽  
Genetic Alteration ◽  
World Health ◽  
Low Grade ◽  
Neuroepithelial Tumors ◽  
Molecular Features ◽  
Standard Clinical Practice ◽  
Imaging Characteristics ◽  
Health Organization

Abstract Purpose In addition to histology, genetic alteration is now required to classify many central nervous system (CNS) tumors according to the most recent World Health Organization CNS tumor classification scheme. Although that is still not the case for classifying pediatric low-grade neuroepithelial tumors (PLGNTs), genetic and molecular features are increasingly being used for making treatment decisions. This approach has become a standard clinical practice in many specialized pediatric cancer centers and will likely be more widely practiced in the near future. This paradigm shift in the management of PLGNTs necessitates better understanding of how genetic alterations influence histology and imaging characteristics of individual PLGNT phenotypes. Methods The complex association of genetic alterations with histology, clinical, and imaging of each phenotype of the extremely heterogeneous PLGNT family has been addressed in a holistic approach in this up-to-date review article. A new imaging stratification scheme has been proposed based on tumor morphology, location, histology, and genetics. Imaging characteristics of each PLGNT entity are also depicted in light of histology and genetics. Conclusion This article reviews the association of specific genetic alteration with location, histology, imaging, and prognosis of a specific tumor of the PLGNT family and how that information can be used for better imaging of these tumors.

scholarly journals Genetic Abnormalities, Clonal Evolution, and Cancer Stem Cells of Brain Tumors

2018 ◽  
Vol 6 (4) ◽  
pp. 85 ◽  
Author(s):  
Ugo Testa ◽  
Germana Castelli ◽  
Elvira Pelosi
Keyword(s):  
Brain Tumors ◽  
Clonal Evolution ◽  
Pediatric Brain Tumors ◽  
Genetic Alterations ◽  
World Health ◽  
Driver Mutations ◽  
Low Grade ◽  
High Grade ◽  
Genetic Profiling ◽  
Health Organization

Brain tumors are highly heterogeneous and have been classified by the World Health Organization in various histological and molecular subtypes. Gliomas have been classified as ranging from low-grade astrocytomas and oligodendrogliomas to high-grade astrocytomas or glioblastomas. These tumors are characterized by a peculiar pattern of genetic alterations. Pediatric high-grade gliomas are histologically indistinguishable from adult glioblastomas, but they are considered distinct from adult glioblastomas because they possess a different spectrum of driver mutations (genes encoding histones H3.3 and H3.1). Medulloblastomas, the most frequent pediatric brain tumors, are considered to be of embryonic derivation and are currently subdivided into distinct subgroups depending on histological features and genetic profiling. There is emerging evidence that brain tumors are maintained by a special neural or glial stem cell-like population that self-renews and gives rise to differentiated progeny. In many instances, the prognosis of the majority of brain tumors remains negative and there is hope that the new acquisition of information on the molecular and cellular bases of these tumors will be translated in the development of new, more active treatments.

Treatment of Adult Lower-Grade Glioma in the Era of Genomic Medicine

2016 ◽  
pp. 75-81 ◽  
Author(s):  
Susan M. Chang ◽  
Daniel P. Cahill ◽  
Kenneth D. Aldape ◽  
Minesh P. Mehta
Keyword(s):  
Molecular Genetic ◽  
Genomic Medicine ◽  
Genetic Alterations ◽  
World Health ◽  
Low Grade ◽  
Lower Grade ◽  
Cell Of Origin ◽  
Low Grade Gliomas ◽  
Molecular Features ◽  
Anaplastic Gliomas

By convention, gliomas are histopathologically classified into four grades by the World Health Organization (WHO) legacy criteria, in which increasing grade is associated with worse prognosis and grades also are subtyped by presumed cell of origin. This classification has prognostic value but is limited by wide variability of outcome within each grade, so the classification is rapidly undergoing dramatic re-evaluation in the context of a superior understanding of the biologic heterogeneity and molecular make-up of these tumors, such that we now recognize that some low-grade gliomas behave almost like malignant glioblastoma, whereas other anaplastic gliomas have outcomes comparable to favorable low-grade gliomas. This clinical spectrum is partly accounted for by the dispersion of several molecular genetic alterations inherent to clinical tumor behavior. These molecular biomarkers have become important not only as prognostic factors but also, more critically, as predictive markers to drive therapeutic decision making. Some of these, in the near future, will likely also serve as potential therapeutic targets. In this article, we summarize the key molecular features of clinical significance for WHO grades II and III gliomas and underscore how the therapeutic landscape is changing.

scholarly journals Solid Pseudopapillary Neoplasm of the Pancreas: Unfolding an Intriguing Condition

2021 ◽  
pp. 1-12
Author(s):  
Manuel António Alves Cruz ◽  
Pedro Moutinho-Ribeiro ◽  
Pedro Costa-Moreira ◽  
Guilherme Macedo
Keyword(s):  
Pancreatic Cancer ◽  
Survival Rate ◽  
Malignant Neoplasm ◽  
World Health ◽  
Ductal Adenocarcinoma ◽  
Malignant Neoplasms ◽  
Low Grade ◽  
Solid Pseudopapillary Neoplasm ◽  
Imaging Characteristics ◽  
Health Organization

Pancreatic cancer is one of the most lethal malignant neoplasms, with a 1-year survival rate after diagnosis of 24%, and a 5-year survival rate of only 9%. While this illustrates the behavior of its main histologic type – ductal adenocarcinoma, there are other histologic subtypes of pancreatic cancer that can harbor excellent prognosis. Solid pseudopapillary neoplasm, described as a rare low-grade malignant neoplasm by the World Health Organization, is the best example of that, having an overall 5-year survival rate of about 97%. Not only the prognosis, but everything about this entity is unique: its histogenesis, epidemiology, presentation, imaging characteristics, cytology features, immunohistochemical profile, and treatment. This explains the urge to improve our understanding about this entity and thus our ability to accurately recognize and manage it. Having this in mind, this article aims to summarize the most relevant topics regarding this entity.

Chondroid Tumors as Incidental Findings and Differential Diagnosis between Enchondromas and Low-grade Chondrosarcomas

2019 ◽  
Vol 23 (01) ◽  
pp. 003-018 ◽  
Author(s):  
Amanda Isaac ◽  
José Villagrán ◽  
P. Afonso
Keyword(s):  
Bone Tumor ◽  
World Health ◽  
Bone Lesions ◽  
Low Grade ◽  
Diagnostic Features ◽  
Primary Bone Tumor ◽  
Imaging Characteristics ◽  
Primary Bone ◽  
Health Organization ◽  
Cartilage Tumor

Chondroid tumors are a heterogeneous group of neoplasms that all share the production of chondroid matrix. This ranges from a fetal type to mature hyaline cartilage and mirrors its imaging characteristics.The benign chondroid tumors represent some of the most encountered incidental bone lesions, with osteochondroma the most frequent benign bone tumor. Enchondroma is mostly asymptomatic, and yet it is probably the second most common primary bone tumor. Similarly, its malignant counterpart, chondrosarcoma, is the second most common malignant primary bone tumor.The 2013 World Health Organization (WHO) updated this group of tumors, and grade 1 chondrosarcoma was renamed “atypical cartilage tumor” and classified as an intermediate type of tumor, not a malignancy, which better describes its clinical behavior.In this article we summarize changes made in the updated 2013 WHO classification and highlight the diagnostic features differentiating an enchondroma from a low-grade chondrosarcoma. We also describe practical imaging aspects of the remaining chondroid tumors.

Lung Tumors With Neuroendocrine Morphology: Essential Radiologic and Pathologic Features

2008 ◽  
Vol 132 (7) ◽  
pp. 1055-1061 ◽  
Author(s):  
Teri J. Franks ◽  
Jeffrey R. Galvin
Keyword(s):  
Neuroendocrine Tumors ◽  
Classification Systems ◽  
World Health ◽  
National Library ◽  
Molecular Features ◽  
Distinct Subset ◽  
Pathologic Features ◽  
The World ◽  
Health Organization

Abstract Context.—Tumors with neuroendocrine morphology are a distinct subset of lung neoplasms sharing characteristic histologic, immunohistochemical, ultrastructural, and molecular features. Objective.—To review the current histologic classification and the diagnostic criteria for the major categories of neuroendocrine tumors of the lung. Data Sources.—Published classification systems from the World Health Organization and pertinent peer-reviewed articles indexed in PubMed (National Library of Medicine) form the basis of this review. Conclusions.—Accurate classification of the neuroendocrine tumors of the lung requires knowledge of specific criteria separating the major categories, which is essential for determining prognosis and treatment.

scholarly journals Spindle cell/sclerosing rhabdomyosarcoma with intracranial invasion without destroying the bone of the skull base: a case report and literature review

2017 ◽  
Vol 6 (8) ◽  
pp. 205846011772731
Author(s):  
Daichi Momosaka ◽  
Osamu Togao ◽  
Akio Hiwatashi ◽  
Koji Yamashita ◽  
Koji Yoshimoto ◽  
...  
Keyword(s):  
Spindle Cell ◽  
Bone Destruction ◽  
Japanese Woman ◽  
World Health ◽  
Imaging Characteristics ◽  
The World ◽  
Sclerosing Rhabdomyosarcoma ◽  
Elderly Japanese ◽  
Health Organization ◽  
Worse Prognosis

Spindle cell/sclerosing rhabdomyosarcoma (ssRMS) is a new subtype of rhabdomyosarcoma included in the World Health Organization soft tissue and bone tumor classification in 2013. Despite the increasing number of reported cases of ssRMS, the imaging characteristics of ssRMS are not established. Herein, we present the case of an elderly Japanese woman with ssRMS of the masticator space with intracranial invasion without destruction of the adjacent bone. Attention should be paid to the presence of intracranial infiltration that may indicate a worse prognosis. Tumor growth without bone destruction could be a key finding to differentiate ssRMSs from conventional subtypes of rhabdomyosarcoma.

scholarly journals Well-Differentiated Liposarcoma of The Larynx: A Case Report and Review of Literature

2016 ◽  
Vol 9 (1) ◽  
pp. 85-89
Author(s):  
Svetlana A. Mateva ◽  
Margarita R. Nikolova ◽  
Alexandar V. Valkov ◽  
Margarita R. Nikolova
Keyword(s):  
Case Report ◽  
Soft Tissue ◽  
Soft Tissue Sarcomas ◽  
World Health ◽  
Low Grade ◽  
Review Of Literature ◽  
Grade Malignancy ◽  
Well Differentiated ◽  
Health Organization ◽  
Histologic Types

Summary Liposarcoma is one of the most common soft tissue sarcomas in adults with a relative incidence amongst other sarcomas ranging from 9.8% to 16%. It usually locates in the limbs and retroperitoneum. Primary liposarcomas of the larynx and hypopharynx are rare, comprising less than 20% of all head and neck liposarcomas. According to World Health Organization, these tumors are divided into four histologic types, and well-differentiated liposarcoma is the most common one. It is a tumor of low-grade malignancy that may recur locally, but does not metastasize. We present a case of laryngopharyngeal well- differentiated liposarcoma in an old patient with two previous removals. We also discuss recently published cases with this unusual location of liposarcoma.

miR-9-1 Is a New Circulating Biomarker for Higher-grade Meningioma Regulated via the EGFR-FOS Network

2020 ◽  
Author(s):  
Daniele Baiz ◽  
Caterina Negroni ◽  
Sara Ferluga ◽  
Emanuela Ercolano ◽  
Claire L Adams ◽  
...  
Keyword(s):  
Tumor Grade ◽  
World Health ◽  
Tumor Growth Rate ◽  
Low Grade ◽  
Serum Samples ◽  
Circulating Biomarker ◽  
Mirna Array ◽  
Malignant Grade ◽  
Health Organization

Abstract Background: Meningiomas are the most common primary CNS tumors. According to the World Health Organization Classification (WHO), they are classified as benign (grade I), atypical (grade II), and anaplastic/malignant (grade III). Chemotherapy has proven ineffective in treating these tumors, which are primarily managed by surgery, radiotherapy, or a combination of them. Morbidity and mortality correlate with meningioma grade. Currently, risk assessment for treatment is based on the radiological assessment of tumor size, tumor growth rate, and/or clinical progression of symptoms. Methods: We performed a cancer miRNA array in an in vitro model of meningioma in order to identify circulating biomarkers in meningioma patients. We validated the miRNA biomarker candidate in cells and tissues and analyzed its regulation. We then investigated expression in tissues and blood. Results: We identified miR-9-1 as significantly overexpressed in atypical and anaplastic cells compared to benign. We further demonstrated that miR-9-1 overexpression is due to increased levels of FOS via upregulation of the EGFR receptor, and showed that miR-9-1 and FOS are upregulated in a cohort of higher-grade meningioma biopsies. Next, we isolated circulating exosomes from meningioma patients’ serum samples, and found higher levels of miR-9-1 in higher-grade compared to low-grade meningiomas patients. Conclusions: Overall, our study shows overexpression and the mechanism of miR-9-1 regulation and suggests miR-9-1 as a novel circulating biomarker candidate to identify tumor grade in meningioma.

Management for Different Glioma Subtypes: Are All Low-Grade Gliomas Created Equal?

2019 ◽  
pp. 133-145 ◽  
Author(s):  
Martin C. Tom ◽  
Daniel P. Cahill ◽  
Jan C. Buckner ◽  
Jörg Dietrich ◽  
Michael W. Parsons ◽  
...  
Keyword(s):  
Molecular Genetic ◽  
World Health ◽  
Low Grade ◽  
Lower Grade ◽  
Future Directions ◽  
Molecular Alterations ◽  
Lower Grade Glioma ◽  
Grade 3 ◽  
Health Organization

Following the identification of key molecular alterations that provided superior prognostication and led to the updated 2016 World Health Organization (WHO) Central Nervous System (CNS) Tumor Classification, the understanding of glioma behavior has rapidly evolved. Mutations in isocitrate dehydrogenase (IDH) 1 and 2 are present in the majority of adult grade 2 and 3 gliomas, and when used in conjunction with 1p/19q codeletion for classification, the prognostic distinction between grade 2 versus grade 3 is diminished. As such, the previously often used term of “low-grade glioma,” which referred to grade 2 gliomas, has now been replaced by the phrase “lower-grade glioma” to encompass both grade 2 and 3 tumors. Additional molecular characterization is ongoing to even further classify this heterogeneous group of tumors. With such a colossal shift in the understanding of lower-grade gliomas, management of disease is being redefined in the setting of emerging molecular-genetic biomarkers. In this article, we review recent progress and future directions regarding the surgical, radiotherapeutic, chemotherapeutic, and long-term management of adult lower-grade gliomas.

scholarly journals Succinate Dehydrogenase–Deficient Renal Cell Carcinoma

2018 ◽  
Vol 143 (5) ◽  
pp. 643-647 ◽  
Author(s):  
Tsung-Heng Tsai ◽  
Wen-Ying Lee
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Succinate Dehydrogenase ◽  
Renal Cell ◽  
World Health ◽  
Low Grade ◽  
Long Term Follow Up ◽  
Health Organization

Succinate dehydrogenase (SDH)–deficient renal cell carcinoma is a recently recognized distinct subtype of renal cell carcinoma in the 2016 World Health Organization classification. It is associated with SDH gene germline mutations, which also cause paraganglioma/pheochromocytoma, gastrointestinal stromal tumor, and pituitary adenoma. The tumor most commonly presents in young adulthood. The tumors are arranged in solid nests or in tubules and frequently show cystic change. The tumors are composed of cuboidal to oval cells with round nuclei, dispersed chromatin, and inconspicuous nucleoli. The cytoplasm is eosinophilic or flocculent but not truly oncocytic. The most distinctive histologic feature is the presence of cytoplasmic vacuoles or inclusions. Loss of SDH subunit B immunostaining is needed for a definite diagnosis. The prognosis is good for low-grade tumors but worse for tumors with high-grade nuclei, sarcomatoid change, or coagulative necrosis. Long-term follow-up is indicated.

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