scholarly journals A case of diffuse leptomeningeal glioneuronal tumor in a 10‐year‐old boy: First report from Iran

2021 ◽  
Vol 9 (12) ◽  
Author(s):  
Parvaneh Karimzadeh ◽  
Yalda Nilipour ◽  
Mitra Khalili ◽  
Ali Nikkhah ◽  
Mehdi Taghavijelodar ◽  
...  
Keyword(s):  
Glioneuronal Tumor ◽  
First Report ◽  
Diffuse Leptomeningeal Glioneuronal Tumor

scholarly journals Diffuse leptomeningeal glioneuronal tumor with high-grade features masquerading as tubercular meningitis—a case report

2021 ◽  
Vol 52 (1) ◽  
Author(s):  
Sameer Peer ◽  
Vivek Murumkar ◽  
Karthik Kulanthaivelu ◽  
Chandrajit Prasad ◽  
Shilpa Rao ◽  
...  
Keyword(s):  
Case Report ◽  
Early Diagnosis ◽  
Histopathological Examination ◽  
Glioneuronal Tumor ◽  
Communicating Hydrocephalus ◽  
High Grade ◽  
Endemic Region ◽  
Tubercular Meningitis ◽  
Leptomeningeal Enhancement ◽  
Diffuse Leptomeningeal Glioneuronal Tumor

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.

scholarly journals LGG-26. DIFFUSE LEPTOMENINGEAL GLIONEURONAL TUMOR (DLGNT) IN CHILDREN: DIFFERENT CLINICAL PRESENTATIONS AND OUTCOMES

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii371-iii371
Author(s):  
Andge Valiakhmetova ◽  
Ludmila Papusha ◽  
Ludmila Yasko ◽  
Alexander Druy ◽  
Alexander Karachunsky ◽  
...  
Keyword(s):  
Mapk Pathway ◽  
Braf Inhibitor ◽  
Complete Response ◽  
Mek Inhibitor ◽  
Braf V600e ◽  
Glioneuronal Tumor ◽  
Low Grade ◽  
Conventional Chemotherapy ◽  
Diffuse Leptomeningeal Glioneuronal Tumor ◽  
Braf Fusion

Abstract Diffuse leptomeningeal glioneuronal tumor (DLGNT) is an extremely rare disease, newly recognized in the 2016 WHO classification of tumors of the CNS. Most DLGNTs are low-grade neuroepithelial tumors with variable elements of neuronal/neurocytic and glial differentiation, have diffuse leptomeningeal enhancement on MRI, and typically harbor KIAA1549-BRAF fusions. Other alterations, such as the BRAF V600E substitution, are less common. Here, we present three cases of DLGNT with different presentations and outcomes. The first patient is a 2yr-old male with KIAA1549-BRAF fusion, and was treated with Carbo/VCR chemotherapy after a biopsy, with resultant ongoing stable disease for 3.5 years. The second patient, an 8yr-old male had the BRAF V600E point mutation and was treated with conventional chemotherapy (VCR/carboplatin). On progression, he received the BRAF inhibitor vemurafenib, achieving a complete response which last 14 month. The third patient, a 27 month old male, harbored a KIAA1549-BRAF fusion and was treated at diagnosis with the MEK inhibitor trametinib. The tumor has been radiographically stable in the context of clinical improvement for 21 months since the treatment initiation, ongoing 24 month. In summary, we present further evidence of MAPK pathway alterations in children with DLGNT. We describe a range of molecular presentations and clinical outcomes, including one patient treated with conventional chemotherapy with further stabilization of disease during 3.5 years and two patients who were successfully treated with targeted therapy.

Diffuse leptomeningeal glioneuronal tumor (DLGNT) mimicking Whipple’s disease: a case report and literature review

2017 ◽  
Vol 33 (8) ◽  
pp. 1411-1414 ◽  
Author(s):  
Vega Karlowee ◽  
Manish Kolakshyapati ◽  
Vishwa Jeet Amatya ◽  
Takeshi Takayasu ◽  
Ryo Nosaka ◽  
...  
Keyword(s):  
Case Report ◽  
Literature Review ◽  
Glioneuronal Tumor ◽  
Whipple’S Disease ◽  
Whipple's Disease ◽  
Diffuse Leptomeningeal Glioneuronal Tumor

DIFFUSE LEPTOMENINGEAL GLIONEURONAL TUMOR IN CHILDREN: MR CHARACTERISTICS, CLINICAL FEATURES AND OUTCOME. FOUR CLINICAL CASES

2021 ◽  
Vol 20 (1) ◽  
pp. 42-55
Author(s):  
A. F. Valiakhmetova ◽  
L. I. Papusha ◽  
A. V. Artemov ◽  
G. V. Tereshchenko ◽  
E. A. Sal’nikova ◽  
...  
Keyword(s):  
Fusion Gene ◽  
Molecular Genetic ◽  
Complete Response ◽  
Mek Inhibitor ◽  
Braf V600e ◽  
Glioneuronal Tumor ◽  
Molecular Testing ◽  
Genetic Characteristics ◽  
Diffuse Leptomeningeal Glioneuronal Tumor ◽  
Braf Fusion

Background. Diffuse leptomeningeal glioneuronal tumor (DLGNT) is an extremely rare entity first officially recognized in 2016 WHO classification of tumors of the central nervous system. Magnetic resonance imaging (MRI) of this tumor usually visualizes diffuse meningeal infiltration with contrast enhancement, with the presence of multiple small contrast‑negative cysts, visible mainly in the T2 images. The main molecular markers of DLGNTs include the KIAA1549-BRAF fusion gene, BRAF V600E substitution is less common.The aim of this work is to describe the manifestation of DLGNT, its neuroimaging and molecular genetic characteristics, the experience of using anti‑BRAF and anti‑MEK therapy.Materials and methods. In this article are described four cases of DLGNT. The first patient with the presence of the KIAA1549-BRAF fusion in the tumor tissue received a full course of SIOP‑LGG / 2004 chemotherapy (carbo‑ platin and vincristine), the stabilization of the disease on the MRI remains for 4 years after completion of treatment. Second patient with KIAA1549-BRAF fusion gene in tumour tissue received MEK inhibitor trametinib as first line of treatment with the stabilization of the disease on control MRI which last for 2 years. A third patient with a mutation in the BRAF V600E gene. After disease progression on standard chemotherapy (carboplatin and vincristine) according to the SIOP‑LGG / 2004 protocol, anti‑BRAF therapy with vemurafenib was prescribed. After 10 months on MRI a complete response was recorded, which persists during the drug intake for 2.5 years. In the fourth patient, no molecular genetic aberrations were detected; a refractory / progressive course of the dis‑ ease was noted. To date, the stabilization of the disease is recorded on the fourth line of chemotherapy (everoli‑ mus and temozolomide).Conclusion. Given the rarity of this tumor and the lack of consensus about therapy, despite the limited number of observations, our experience allows us to recommend molecular testing of DLGNT to detect activating events in the BRAF gene, as well as consideration of anti‑BRAF / MEK therapy if either the BRAF V600E mutation is de‑ tected or KIAA1549-BRAF fusion.

Imaging in diffuse leptomeningeal glioneuronal tumor

2019 ◽  
Vol 67 (2) ◽  
pp. 615 ◽  
Author(s):  
Amol Raheja ◽  
Raj Ghoniya
Keyword(s):  
Glioneuronal Tumor ◽  
Diffuse Leptomeningeal Glioneuronal Tumor

scholarly journals Unusual radiological and histological presentation of a diffuse leptomeningeal glioneuronal tumor (DLGNT) in a 13-year-old girl

2019 ◽  
Vol 35 (9) ◽  
pp. 1609-1614 ◽  
Author(s):  
Nishant Tiwari ◽  
Benita Tamrazi ◽  
Nathan Robison ◽  
Mark Krieger ◽  
Jianling Ji ◽  
...  
Keyword(s):  
Glioneuronal Tumor ◽  
Diffuse Leptomeningeal Glioneuronal Tumor

Unusual high-grade features in pediatric diffuse leptomeningeal glioneuronal tumor: comparison with a typical low-grade example

2017 ◽  
Vol 70 ◽  
pp. 105-112 ◽  
Author(s):  
Katherine E. Schwetye ◽  
Akash P. Kansagra ◽  
James McEachern ◽  
Robert E. Schmidt ◽  
Karen Gauvain ◽  
...  
Keyword(s):  
Glioneuronal Tumor ◽  
Low Grade ◽  
High Grade ◽  
Diffuse Leptomeningeal Glioneuronal Tumor

scholarly journals Diffuse Leptomeningeal Glioneuronal Tumor: Case Report and Review of Literature

2020 ◽  
Vol 4 (1) ◽  
Author(s):  
Marianayagam Neelan J ◽  
Ben-Shalom Netanel ◽  
Toledano Helen ◽  
Marglit Nevo ◽  
Michowiz Shalom ◽  
...  
Keyword(s):  
Case Report ◽  
Glioneuronal Tumor ◽  
Review Of Literature ◽  
Diffuse Leptomeningeal Glioneuronal Tumor

Spinal Cord Diffuse Leptomeningeal Glioneuronal Tumor Presenting without Leptomeningeal Dissemination

2021 ◽  
pp. 1-6
Author(s):  
Carlos Perez-Vega ◽  
Oluwaseun O. Akinduro ◽  
Bradley J. Cheek ◽  
Alexandra D. Beier
Keyword(s):  
Spinal Cord ◽  
Spinal Cord Tumor ◽  
Tumor Resection ◽  
World Health ◽  
Glioneuronal Tumor ◽  
Pathology Report ◽  
Leptomeningeal Dissemination ◽  
Unique Type ◽  
Intramedullary Spinal Cord ◽  
Diffuse Leptomeningeal Glioneuronal Tumor

<b><i>Background and Importance:</i></b> Diffuse leptomeningeal glioneuronal tumor (DLGNT) represents a provisional entity in the 2016 World Health Organization classification of tumors; it is characterized by a widespread leptomeningeal growth and oligodendroglial-like cytology. To this day, 4 pediatric patients have been reported to present with an isolated spinal cord tumor in the absence of leptomeningeal dissemination. Gross total resection (GTR) was achieved in only 1 patient. We present the clinical and technical nuances of this unique type of tumor, as well as the second reported case of GTR in a patient with DLGNT. <b><i>Clinical Presentation:</i></b> A 4-year-old boy presented to the emergency department after an episode of flaccid paralysis of bilateral lower extremities. MRI showed an intramedullary spinal cord tumor centered at T8. The patient was taken to the operative room, where a laminectomy and tumor resection were performed; cystic and solid tumor components were identified. Pathology report was consistent with DLGNT. After achieving GTR, patient is free of recurrence after a 15-month follow-up. <b><i>Conclusion:</i></b> No standard treatment for DLGNT has been identified. Current literature report surgery and chemotherapy with variable success rates. DLGNT presenting as an isolated intramedullary tumor is an uncommon condition which progression appears to be halted when treated promptly. Identifying solid and cystic components of this tumor is crucial for achieving GTR.

EPID-16. DIFFUSE LEPTOMENINGEAL GLIONEURONAL TUMOR IN PEDIATRIC PATIENTS: A QUANTITATIVE EXPLORATION

2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi89-vi89
Author(s):  
Victor Lu ◽  
Long Di ◽  
David Daniels ◽  
Ossama Maher ◽  
Toba Niazi
Keyword(s):  
Overall Survival ◽  
Pediatric Patients ◽  
Pediatric Population ◽  
Glioneuronal Tumor ◽  
Surgical Biopsy ◽  
Duration Of Symptoms ◽  
Exact Test ◽  
Entire Cohort ◽  
Prognostic Features ◽  
Diffuse Leptomeningeal Glioneuronal Tumor

Abstract BACKGROUND Diffuse leptomeningeal glioneuronal tumor (DLGNT), also known as oligodendrogliomatosis, is a rare neuro-oncologic condition along the neuraxis that remains poorly understood in children. We sought to describe our institutional experience and quantitively summarize the clinical survival and prognostic features of DLGNT in the pediatric population across the contemporary literature. METHODS We report four institutional cases of pediatric DLGNT diagnosed between 2000-2020 based on retrospective review of our records, and performed a comprehensive literature search for published cases from 2000 onwards to create an integrated cohort for analysis. Kaplan-Meier estimations, Fisher’s exact test, and logistic regression were utilized to interrogate the data. RESULTS Our overall integrated cohort consisted of 54 pediatric DLGNT patients, with 19 (35%) female and 35 (65%) male patients diagnosed at an average age of 6.4 years (range, 1.3-17 years) by means of surgical biopsy. Chemotherapy was used in 45 cases (83%), and mean follow-up time of 54 months (range, 3-204). Across the entire cohort, overall survival 1 month after diagnosis was 96% (95% CI 86-99%), and by 10 years was 69% (95% CI 49-82%). On multivariate analysis of complete data, chemotherapy treatment (HR=0.23, P=0.04) was statistically predictive of longer overall survival. When including limited data, longer duration of symptoms by presentation (HR=1.03, P=0.03) was statistically predictive of shorter overall survival. CONCLUSIONS This is the first quantitative study of pediatric DLGNT clinical course. More than 2-out-of-3 pediatric DLGNT patients survive beyond one decade. Chemotherapy is statistically associated with longer survival in DLGNT pediatric patients and should form the core of any treatment regimen in this setting. Early detection by means of judicious imaging and surgical biopsy for tissue diagnosis can lead to earlier treatment and likely superior outcomes.

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