Diffuse leptomeningeal glioneuronal tumor: A review of diagnosis and management with an illustrative case

Al-Ghanem, Rajab; Barona, R; Godoy-Hurtado, Alicia; Bulnes, José Manuel Galicia; El-Rubaidi, Osamah Abdullah

doi:10.1016/j.neucie.2022.02.006

Cited by 3 publications

(11 citation statements)

References 8 publications

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“…This finding is consistent with reports in the literature and demonstrates the challenges associated with CSF diversion in this patient population. 8,23 In our report, no further shunt failures were noted after the placement of a valveless shunt system. This may represent a useful clinical observation for neurosurgeons facing a similar clinical dilemma.…”

Section: Management Challengesmentioning

confidence: 43%

“…The optimal place to biopsy is currently unclear, but a consensus appears to exist regarding targeting tissue with leptomeningeal enhancement or intraparenchymal lesions when present in a safe location. 8 , 11 , 12 CSF cytology may be unrevealing 12 ; however, some authors do report obtaining diagnostic samples. 13 In this case, initial intracranial leptomeningeal biopsy and CSF cytology yielded nondiagnostic tissue, but biopsy of the lumbar leptomeninges after further disease progression yielded the diagnosis.…”

Section: Discussionmentioning

confidence: 99%

“…Various treatment regimens have been proposed in the literature, including craniospinal irradiation and the use of chemotherapeutic or biological agents such as carboplatin, bevacizumab, and temozolomide. 8,[24][25][26] Craniospinal irradiation provides more targeted treatment and has been documented to improve clinical outcomes and slow disease progression. 4,5 In addition, multiple groups found that carboplatin and vincristine were able to improve survival and halt disease progression.…”

Section: Management Challengesmentioning

confidence: 99%

“…[3][4][5][6][7] Diagnosis is based on tissue biopsy for histological and molecular pathology analysis. 8 Because of its new identification and low incidence, additional reports and case series are needed to better understand the variations in clinical presentation, diagnosis, and management of DLGNT. 9 Here we present a case of a 47-year-old female with rapidly progressive DLGNT.…”

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confidence: 99%

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Rapidly progressive diffuse leptomeningeal glioneuronal tumor in an adult female: illustrative case

Bao¹,

Sweeney²,

Liu

et al. 2023

Journal of Neurosurgery: Case Lessons

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BACKGROUND Diffuse leptomeningeal glioneuronal tumor (DLGNT) is a rare brain tumor only recently classified by the World Health Organization in 2016 and has few reports on its incidence in adults. OBSERVATIONS The authors describe a case of DLGNT presenting in a 47-year-old female with seizures, cranial neuropathies, and communicating hydrocephalus with rapid clinical progression. Workup demonstrated progressive leptomeningeal enhancement of the skull base, cranial nerves, and spine, and communicating hydrocephalus. Elevated serum rheumatological markers and early response to systemic corticosteroids and immunosuppressant therapy complicated the diagnosis. Multiple biopsy attempts were required to obtain diagnostic tissue. Pathology demonstrated hypercellularity surrounding leptomeningeal vessels with nuclear atypia, staining positive for GFAP, Olig2, S100, and synaptophysin. Molecular pathology demonstrated loss of chromosome 1p, BRAF overexpression but no rearrangement, and H3K27 mutation. Repeat cerebrospinal fluid (CSF) diversion procedures were required for hydrocephalus management due to high CSF protein content. LESSONS This report describes a rare, aggressive, adult presentation of DLGNT. Leptomeningeal enhancement and communicating hydrocephalus should raise suspicion for this disease process. Biopsy at early stages of disease progression is essential for early diagnosis and prompt treatment. Further study into the variable clinical presentation, histological and molecular pathology, and optimal means of diagnosis and management is needed.

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Section: Management Challengesmentioning

confidence: 43%

Section: Discussionmentioning

confidence: 99%

Section: Management Challengesmentioning

confidence: 99%

mentioning

confidence: 99%

See 2 more Smart Citations

Rapidly progressive diffuse leptomeningeal glioneuronal tumor in an adult female: illustrative case

Bao¹,

Sweeney²,

Liu

et al. 2023

Journal of Neurosurgery: Case Lessons

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“…DLGNT can often be difficult to diagnose because patients experience a broad spectrum of symptoms, including hemicrania migraine, other recurrent headaches, hydrocephalus, cranial nerves dysfunction, seizures, gait disorder, aphasia, and urinary dysfunction [ 3 , 5 - 8 , 11 , 12 , 16 ]. The patient, in the current case, sought medical care four times with different symptoms before he was correctly diagnosed.…”

Section: Discussionmentioning

confidence: 99%

Diffuse Leptomeningeal Glioneuronal Tumor: First Description of Metastasis to the Lung and Bone Marrow

Battini,

Gokden,

Palys

et al. 2023

Cureus

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Diffuse leptomeningeal glioneuronal tumor (DLGNT) is a rare neoplasm of the central nervous system (CNS) that primarily affects the leptomeninges. However, it can also involve the brain parenchyma and spinal cord. We report the first case of metastasis of this primary CNS tumor to the lung and bone marrow. An 18-year-old male was diagnosed with DLGNT through meningeal biopsy after multiple events of transient neurologic signs and symptoms that included recurrent episodes of encephalopathy, seizures, cerebral vasospasms, cranial nerve palsy, and urinary dysfunction. Five months after diagnosis, the patient presented with pancytopenia and pulmonary effusion. At that time, he was being treated with temozolomide, after radiation treatment to the brain and spinal cord. Bone marrow biopsy and pleural cytology revealed systemic metastases from the primary CNS tumor. He was then treated with chemotherapy with carboplatin and vincristine which improved his condition for two and a half months. Unfortunately, the patient died of a high systemic metastatic burden. Primary CNS tumors rarely produce systemic metastases, and this is the first report of DLGNT with bone marrow and pulmonary metastases. Chemotherapy with carboplatin and vincristine should be considered as a treatment for patients with DLGNT, as the patient presented a systemic response with clinical and radiological improvement.

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Diffuse Leptomeningeal Glioneuronal Tumors: A Case Series of Five Patients with Parenchymal Forms and an Analysis of the Diagnostic Challenges, Treatment Options and Outcomes

Demir

Yapıcıer

Kilic

et al. 2023

CMIR

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Background and Purpose: Diffuse leptomeningeal glioneuronal tumors (DL-GNT) are rare glioneuronal neoplasms with oligodendroglioma-like cells. These tumors can present as a dominant intracranial mass or as a solitary spinal cord mass without leptomeningeal involvement. In this study, we aimed to determine the magnetic resonance imaging and histopathological features, treatment modalities, and clinical outcomes of the parenchymal forms of DL-GNTs. Methods: This is a retrospective three-center case series study of 5 patients with a confirmed parenchymal form of DLGTs, out of which 4 patients were adults. Brain and spinal cord MR imaging were performed in all patients at either 1.5 or 3T. The patients' age ranged from 5 years to 50 years with a mean age of 27.6 years at presentation. objective: These tumors can present as a dominant intracranial mass or as a solitary spinal cord mass without leptomeningeal involvement. Results: Four of the tumors were located in the frontal lobe, and one in the tectum. They were usually solid-cystic enhancing tumors as the other mixed neuronal-glial tumors. All of the tumors had an extension to the superficial surface of a cerebral hemisphere. One had systemic bone metastases. The clinical signs and symptoms of the parenchymal form varied based on the location of the mass, in contrast to the leptomeningeal form associated with hydrocephalus. In one case, the tumor’s initial grade was defined as intermediate. The initial histopathology of the two cases was low-grade and no upgrade occurred in the follow-up period. In two cases, although the tumors were low grade initially, they progressed to an anaplastic form in the follow-up period. Conclusion: The parenchymal form of DL-GNTs is common in adults. Extension to the superficial surface of a cerebral hemisphere is a distinctive imaging feature. Systemic osseous metastasis may occur. Due to the presence of common histopathological features, including the biphasic composition of glial and neuronal cell elements and oligodendroglioma-like cells, a proposed classification approach might be more beneficial for the histopathological and imaging description, and management of the glioneuronal tumors with oligodendroglioma-like features. other: -

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Diffuse leptomeningeal glioneuronal tumor: A review of diagnosis and management with an illustrative case

Cited by 3 publications

References 8 publications

Rapidly progressive diffuse leptomeningeal glioneuronal tumor in an adult female: illustrative case

Rapidly progressive diffuse leptomeningeal glioneuronal tumor in an adult female: illustrative case

Diffuse Leptomeningeal Glioneuronal Tumor: First Description of Metastasis to the Lung and Bone Marrow

Diffuse Leptomeningeal Glioneuronal Tumors: A Case Series of Five Patients with Parenchymal Forms and an Analysis of the Diagnostic Challenges, Treatment Options and Outcomes

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