Diffuse leptomeningeal glioneuronal tumor

Objective To explore the clinicopathological features and molecular genetics of diffuse leptomeningeal glioneuronal tumor (DLGNT). Methods and Results A 5 - year- old boy presented with severe hydrocephalus and two times of convulsions. Head MRI showed obvious broadening of bilateral lateral ventric...

Full description

Bibliographic Details
Main Authors: Wei JIN, Xi-rui HUANG, Qiong WANG, Qiu-ping GUI
Format: Article
Language:English
Published: Tianjin Huanhu Hospital 2018-07-01
Series:Chinese Journal of Contemporary Neurology and Neurosurgery
Subjects:
Online Access:http://www.cjcnn.org/index.php/cjcnn/article/view/1813
_version_ 1819105993077817344
author Wei JIN
Xi-rui HUANG
Qiong WANG
Qiu-ping GUI
author_facet Wei JIN
Xi-rui HUANG
Qiong WANG
Qiu-ping GUI
author_sort Wei JIN
collection DOAJ
description Objective To explore the clinicopathological features and molecular genetics of diffuse leptomeningeal glioneuronal tumor (DLGNT). Methods and Results A 5 - year- old boy presented with severe hydrocephalus and two times of convulsions. Head MRI showed obvious broadening of bilateral lateral ventricles, suggesting hydrocephalus, and abnormal patchy mildly high-intensity signals scattered in bilateral cerebellar superior sulci; spinal MRI revealed thickened spinal cord at the level of T5-7 and mildly high-intensity signals within the spinal cord; enhanced MRI showed thickening and enhancement of the surface of brainstem, meninges on the surface of bilateral cerebellar hemispheres, whole spinal meninges and partial spinal dura mater, and cauda equina. The patient underwent exploratory resection of thoracic lesions and spinal canal reconstruction. Intraoperative findings showed semi - transparent gelatinoid hyperplasia between subarachnoid and soft meningeal, which blocked the subarachnoid cavity. Histological findings showed low-to moderate-density monomorphic oligdendroglial-like tumor cells with diffusely growth or in small nests in the leptomeninges. Mitotic activity and necrosis were not found. Immunohistochemical staining showed oligdendroglial-like tumor cells expressed oligodendrocytes transcription factor-2 (Olig-2) in nuclei, synaptophysin (Syn), microtubule - associated protein - 2 (MAP - 2) and S - 100 protein (S -100) in cytoplasm. Ki-67 labeling index was 4%-10%. Fluorescence in situ hybridization (FISH) analysis revealed deletion of 1p, whereas 19q was intact. The final diagnosis was DLGNT. The patient was hospitalized for 22 d and died 3 months after discharge. Conclusions DLGNT is a group of primary brain neoplasm of recent acquisition in the 2016 World Health Organization (WHO) classification of central nervous system tumors and does not yet assign a distinct WHO grade to the entity. At present, DLGNT has not been reported in China. DLGNT is very rare and always confused with other central nervous system tumors and inflammatory lesions. Therefore, histological morphology, immunohistochemistry and characteristics of molecular genetics are very important for diagnosis. DOI: 10.3969/j.issn.1672-6731.2018.07.010
first_indexed 2024-12-22T02:31:04Z
format Article
id doaj.art-8117c41c174f4f6fbeb458e25c4b1a7c
institution Directory Open Access Journal
issn 1672-6731
language English
last_indexed 2024-12-22T02:31:04Z
publishDate 2018-07-01
publisher Tianjin Huanhu Hospital
record_format Article
series Chinese Journal of Contemporary Neurology and Neurosurgery
spelling doaj.art-8117c41c174f4f6fbeb458e25c4b1a7c2022-12-21T18:41:53ZengTianjin Huanhu HospitalChinese Journal of Contemporary Neurology and Neurosurgery1672-67312018-07-0118752753410.3969/j.issn.1672-6731.2018.07.0101768Diffuse leptomeningeal glioneuronal tumorWei JIN0Xi-rui HUANG1Qiong WANG2Qiu-ping GUI3Department of Pathology, Chinese PLA General Hospital, Beijing 100853, ChinaDepartment of Pathology, Chinese PLA General Hospital, Beijing 100853, ChinaDepartment of Pathology, Chinese PLA General Hospital, Beijing 100853, ChinaDepartment of Pathology, Chinese PLA General Hospital, Beijing 100853, ChinaObjective To explore the clinicopathological features and molecular genetics of diffuse leptomeningeal glioneuronal tumor (DLGNT). Methods and Results A 5 - year- old boy presented with severe hydrocephalus and two times of convulsions. Head MRI showed obvious broadening of bilateral lateral ventricles, suggesting hydrocephalus, and abnormal patchy mildly high-intensity signals scattered in bilateral cerebellar superior sulci; spinal MRI revealed thickened spinal cord at the level of T5-7 and mildly high-intensity signals within the spinal cord; enhanced MRI showed thickening and enhancement of the surface of brainstem, meninges on the surface of bilateral cerebellar hemispheres, whole spinal meninges and partial spinal dura mater, and cauda equina. The patient underwent exploratory resection of thoracic lesions and spinal canal reconstruction. Intraoperative findings showed semi - transparent gelatinoid hyperplasia between subarachnoid and soft meningeal, which blocked the subarachnoid cavity. Histological findings showed low-to moderate-density monomorphic oligdendroglial-like tumor cells with diffusely growth or in small nests in the leptomeninges. Mitotic activity and necrosis were not found. Immunohistochemical staining showed oligdendroglial-like tumor cells expressed oligodendrocytes transcription factor-2 (Olig-2) in nuclei, synaptophysin (Syn), microtubule - associated protein - 2 (MAP - 2) and S - 100 protein (S -100) in cytoplasm. Ki-67 labeling index was 4%-10%. Fluorescence in situ hybridization (FISH) analysis revealed deletion of 1p, whereas 19q was intact. The final diagnosis was DLGNT. The patient was hospitalized for 22 d and died 3 months after discharge. Conclusions DLGNT is a group of primary brain neoplasm of recent acquisition in the 2016 World Health Organization (WHO) classification of central nervous system tumors and does not yet assign a distinct WHO grade to the entity. At present, DLGNT has not been reported in China. DLGNT is very rare and always confused with other central nervous system tumors and inflammatory lesions. Therefore, histological morphology, immunohistochemistry and characteristics of molecular genetics are very important for diagnosis. DOI: 10.3969/j.issn.1672-6731.2018.07.010http://www.cjcnn.org/index.php/cjcnn/article/view/1813Brain neoplasmsNeurogliaNeuronsPia materImmunohistochemistryPathology
spellingShingle Wei JIN
Xi-rui HUANG
Qiong WANG
Qiu-ping GUI
Diffuse leptomeningeal glioneuronal tumor
Chinese Journal of Contemporary Neurology and Neurosurgery
Brain neoplasms
Neuroglia
Neurons
Pia mater
Immunohistochemistry
Pathology
title Diffuse leptomeningeal glioneuronal tumor
title_full Diffuse leptomeningeal glioneuronal tumor
title_fullStr Diffuse leptomeningeal glioneuronal tumor
title_full_unstemmed Diffuse leptomeningeal glioneuronal tumor
title_short Diffuse leptomeningeal glioneuronal tumor
title_sort diffuse leptomeningeal glioneuronal tumor
topic Brain neoplasms
Neuroglia
Neurons
Pia mater
Immunohistochemistry
Pathology
url http://www.cjcnn.org/index.php/cjcnn/article/view/1813
work_keys_str_mv AT weijin diffuseleptomeningealglioneuronaltumor
AT xiruihuang diffuseleptomeningealglioneuronaltumor
AT qiongwang diffuseleptomeningealglioneuronaltumor
AT qiupinggui diffuseleptomeningealglioneuronaltumor