scholarly journals Case Report: Unusual High-Grade Diffuse Leptomeningeal Glioneuronal Tumor Mimicking Tuberculous Meningitis in a Child From an Endemic Region

2021 ◽  
Vol 9 ◽  
Author(s):  
Yong Guang Teh ◽  
Nornazirah Azizan ◽  
Nur Atifah Mohd Naim ◽  
Chiak Yot Ng ◽  
Ke Juin Wong ◽  
...  

Background: Diffuse leptomeningeal glioneuronal tumor (DL-GNT) is a new entity described in the 2016 World Health Organization (WHO) classification of brain tumors. While DL-GNT is predominantly an indolent tumor that affects young boys, high-grade DL-GNT is unusual and seldom reported in children.Case Presentation: In this report, we describe the challenges and pitfalls associated with diagnosing this high-grade variant in a tuberculosis-endemic region. We highlight the importance of identifying non-typical imaging findings, i.e., non-enhancing cystic lesions with high T2 signal along the leptomeningeal surface, that may expedite the diagnosis of this condition. Histopathologic correlations with MR spectroscopy findings are also discussed.Conclusion: We provide the first clinical imaging report of utilizing MR spectroscopy to distinguish DL-GNT from tuberculosis with histopathologic correlation.

2002 ◽  
Vol 10 (3) ◽  
pp. 175-177 ◽  
Author(s):  
Slobodan Dozic ◽  
Dubravka Cvetkovic-Dozic ◽  
Milica Skender-Gazibara ◽  
Branko Dozic

(Conclusion) Classifications of the nervous system tumors should be neither static nor definitive. The most recent, third, current WHO classification of nervous system tumors was published in 2000. Many substantial changes were introduced. New entities include the chordoid glioma of the third ventricle, the atypical teratoid/rhabdoid tumor, cerebellar liponeurocytoma (the former lipomatous medulloblstoma of the cerebellum), solitary fibrous tumor and perineurioma. The new tumor variants include the large cell medulloblastoma, tanacytic ependymoma and rhabdoid meningioma. Several essential changes were introduced in the meningiomas regarding histological subtypes, grading and proliferation index. In addition to new entities described in the 2000 WHO classification there are newly brain tumor entities and tumor variants, which are not included in the current classification due to the insufficient number of reporeted cases, for example papillary glioneuronal tumor, rosetted glioneuronal tumor, lipoastrocytoma and lipomatous meningioma. They will be probably accepted in the next WHO classificaton. In the current WHO classification the importance of cytogenetic and molecular biologic investigation in the understanding and further classifications of these tumors has been emphasized.


Author(s):  
Anna Gabryś ◽  
Julia Kuzaj ◽  
Dominika Pawełczak ◽  
Katarzyna Seliga ◽  
Agnieszka Jelińska ◽  
...  

AbstractDiffuse leptomeningeal glioneuronal tumor (DLGNT) is an entity introduced in 2016 World Health Organization classification of tumors of the central nervous system. The tumor occurs very rarely. Due to the lack of specific clinical and radiological features, biopsy is necessary to be performed and histological and immunohistochemical testing is essential to reach the diagnosis. A 4.5-year-old girl presented with a history of headache, vomiting, and right eye convergent squint. Imaging revealed multiple enhancing lesions located supra- and infratentorially and intramedullary. Histopathological examination demonstrated diffused growth of neoplastic cells. Molecular testing revealed KIAA1549-BRAF fusion and the diagnosis of DLGNT was stated.


Author(s):  
Sameer Peer ◽  
Vivek Murumkar ◽  
Karthik Kulanthaivelu ◽  
Chandrajit Prasad ◽  
Shilpa Rao ◽  
...  

Abstract Background Diffuse leptomeningeal glioneuronal tumor (DLGNT) has been recently described in the literature. The complete neuroimaging spectrum and histopathological characteristics of this entity are yet to be elucidated. In an endemic region, diffuse leptomeningeal enhancement on neuroimaging with associated communicating hydrocephalus is usually suggestive of infective meningitis and the patients are started on empirical anti-microbial therapy. However, it is important to consider other differential diagnosis of leptomeningeal enhancement in such cases, particularly if the clinical condition does not improve on anti-microbial therapy. An early diagnosis of a neoplastic etiology may be of particular importance as the treatment regimens vary considerably depending on the underlying disease condition. Case presentation In this case report, we describe a case of DLGNT with high-grade histopathological features which was initially managed as tubercular meningitis based on the initial neuroimaging findings. Due to worsening of the clinical course and subsequent imaging findings at follow-up, a diagnosis of DLGNT was considered and subsequently proven to be DLGNT with features of anaplasia on histopathological examination of leptomeningeal biopsy specimen. Conclusion This case highlights the importance of recognizing certain subtle finding on MRI which may help in an early diagnosis of DLGNT which is crucial for appropriate treatment.


Author(s):  
Hans Michael Kvasnicka ◽  
Jürgen Thiele

The classification of the World Health Organization (WHO) continues to advocate the diagnostic importance of bone marrow (BM) morphology in the diagnostic workup of myeloproliferative neoplasms (MPN). In this regard, distinctive histological BM patterns characterize specific subtypes of MPN and are the key to a meaningful clinical and molecular-defined risk stratification of patients. In this regard, the morphological denominator includes a characteristic megakaryocytic proliferation along with variable changes in the granulopoiesis and erythropoiesis. Importantly, diagnosis of MPN requires absence of relevant dysgranulopoiesis or dyserythropoiesis. In terms of clinical practice, the concept of precursor stages provides the possibility of an early intervention by appropriate therapeutic regimens that might prevent fatal complications like thrombosis and haemorrhage, especially in early stages of polycythaemia vera or in primary myelofibrosis. However, the WHO classification is not aimed to capture all biological true cases of MPN or guarantee a complete diagnostic specificity and thus might be in need of continuous improvement following clinical experience.


2010 ◽  
Vol 21 (3) ◽  
pp. 259-262 ◽  
Author(s):  
Ramón Manuel Alemán Navas ◽  
María Guadalupe Martínez Mendoza ◽  
Mário Roberto Leonardo ◽  
Raquel Assed Bezerra da Silva ◽  
Henry W. Herrera ◽  
...  

Congenital pathologies are those existing at or dating from birth. Occurrence of congenital cystic lesions in the oral cavity is uncommon in neonates. Eruption cyst (EC) is listed among these unusual lesions. It occurs within the mucosa overlying teeth that are about to erupt and, according to the current World Health Organization (WHO) classification of epithelial cysts of the jaws, EC is a separate entity. This paper presents a case of congenital EC successfully managed by close monitoring of the lesion, without any surgical procedure or tooth extraction. Eruption of the teeth involved, primary central incisors, occurred at the fourth month of age. During this time neither the child nor mother had any complication such as pain on sucking, refusal to feed, airway obstruction, or aspiration of fluids or teeth.


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 1446-1446
Author(s):  
Guntram Buesche ◽  
Arnold Ganser ◽  
Ludwig Wilkens ◽  
Brigitte Schlegelberger ◽  
Hartmut Hecker ◽  
...  

Abstract Marrow fibrosis (MF) is rarely considered in myelodysplastic syndromes (MDS) although the frequency of this complication ranges from 10 to 50 % in the few reports on this issue, and there are no data on occurrence and significance of this complication in the context of the International Prognostic Scoring System (IPSS) and the World Health Organization (WHO) classification of disease. In a retrospective study, diagnostic bone marrow biopsies from a total of 936 patients with MDS were examined for MF and its relevance to the course of disease. Frequency of MF varied markedly between different types of MDS ranging from 3 % (RARS) to 37 % (MDS, therapy-related; WHO classification, P < 0.000005). Risk of MF furthermore correlated with multilineage dysplasia (P < 0.000005). However, there was no obvious correlation to the IPSS or to karyotype abnormalities. The survival time of patients was significantly reduced by about 50 % from 11 (RAEB-1/-2) - 55 (RARS, RCMD-RS) down to 6 (RAEB-1/-2) - 33 months (RARS, RCMD-RS) in median when MF was detected independently of the IPSS and the classification of disease (FAB, WHO; P = 0.0001). We conclude that MF is an unfavorable complication of MDS significantly shortening the survival time of patients independently of the IPSS and the classification of disease.


Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 3317-3317
Author(s):  
Matthew J. Matasar ◽  
Weiji Shi ◽  
Jonathan Silberstien ◽  
Julie T. Feldstein ◽  
Daniel Filippa ◽  
...  

Abstract Background: The effective management of lymphoma depends upon an accurate and precise pathologic diagnosis. However, the classification of lymphoma continues to evolve. Reports addressing the role of second opinion expert pathology review have found varying impact, and little is known regarding the predictors of a change in diagnosis. Furthermore, the impact of the World Health Organization (WHO) classification of lymphomas over the 5 years following their formal publication has not been formally assessed. Methods: All outside pathology is reviewed at Memorial Sloan-Kettering Cancer Center (MSKCC) before a clinical opinion is finalized. We performed a chart review of all externally referred lymphoma cases from 1/1/01 to 6/30/01 and from 1/1/06 to 6/30/06 with second opinions from MSKCC hematopathology. Statistical analysis was performed using Chi-square or Fisher’s exact test for univariate analysis and logistic regression for multivariate analysis. Results: 719 patients (365 in 2001, 354 in 2006) met inclusion criteria. Diagnostic revisions were classified as major or minor; major changes were those that would lead to management changes as per National Comprehensive Cancer Network guidelines. 122 patients (18% in 2001, 16% in 2006) had a major diagnostic revision and an additional 22 (4% in 2001, 2% in 2006) had confirmation of major revisions rendered previously at second opinion from another National Cancer Institute Comprehensive Cancer Center (CCC). This did not change significantly by era, with 79 major revisions (22%) in 2001 and 65 (18%) in 2006 (P=NS). An additional 55 patients [24 (7%) in 2001, 31 (9%) in 2006] received minor revisions. Common categories of major revision included changing from nondiagnostic/ambiguous to definitive [6 in 2001, 8 in 2006], definitive to nondiagnostic [9 in 2001, 9 in 2006], malignant to benign [1 in 2001, 6 in 2006], indolent B-cell lymphoma (BCL) to aggressive BCL [15 in 2001, 8 in 2006], and aggressive BCL to indolent BCL [4 in 2001, 1 in 2006]. Major diagnostic revision was significantly associated with additional immunohistochemistry (IHC) testing in 2001 (OR=2.3; 95%CI 1.3, 4). In 2006, additional IHC (OR=1.8; 95%CI 1, 3.4), repeat biopsy (OR=3.1; 95%CI 1.2, 8.0), and skin biopsy (versus lymph node biopsy; OR 3.3; 95%CI 1.6, 7.0) were significantly associated with major revision. Two of the 7 patients reclassified as benign received revisions based on additional IHC, whereas 7 of the 14 patients reclassified as malignant were revised due to either additional IHC (4) or repeat biopsy (3). No effect was seen by biopsy type, nor were patient gender, age, race or ethnicity associated with odds of major revision. Of cases seen first at another CCC, 12% in 2001 and 16% in 2006 received major revisions, compared to 19% (2001) and 16% (2006) of other cases; these differences were not statistically significant. Conclusion: The rate of clinically meaningful diagnostic revisions at second opinion expert pathology review was high for patients seen at MSKCC, and remained so despite five years of increased familiarity with the WHO classification schema. These data confirm the fact that an appropriate evaluation, including detailed IHC and an adequate biopsy specimen, plays a central role in the accurate diagnosis of lymphoma. The high rates of diagnostic revision reported here lend support to the routine application of expert second opinion hematopathology review.


2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
Patricia Gargano ◽  
Graciela Zuccaro ◽  
Fabiana Lubieniecki

Gliofibroma is a rare tumor with biphasic morphology, commonly occurring in the first two decades of life. Currently, the tumor is not listed as a distinct entity in the current World Health Organization (WHO) classification of central nervous system tumors. As its biological behavior, histogenesis, and prognostic factors are still debated, the aim of this paper was to describe a case of a gliofibroma and to update the data about these lesions. Hence, we present here clinical symptoms, pathological findings, and evolution observed in a child with gliofibroma. A 10-year-old girl with seizures was referred for study. Neuroimaging showed a hemispheric hyperdense tumor with little peritumoral edema and no mass effect. The tumor was totally removed. Histologically, the tumor consisted of a mixture of glial cells and collagen-rich stroma. Immunohistochemical examination revealed positive staining for GFAP, CD 99, S100, and vimentin. EMA staining showed a paranuclear dot pattern in only few cells in isolated areas. These findings of a glial component with collagenous stroma were consistent with a desmoplastic glioma. Because of the rarity of this entity, we believe it is important to report every case in order to adequately analyze and categorize the tumor in the next WHO classification.


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