Primary Leptomeningeal Sarcomatosis; A Pathology Proven Case with Challenging MRI and Clinical Findings (original) (raw)
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Primary leptomeningeal gliomatosis: case report and review of the literature
Neurological Sciences, 2005
A 62-year-old woman developed dysphasia, signs of intracranial hypertension and seizures. An MRI scan evidenced extra-axial contrast enhancement in the absence of mass lesions. CSF analysis was negative for malignant cytology and viral or bacterial microbiology. In the absence of other evidence, considering the acute clinical onset and MRI picture, a viral encephalitis was suspected and antiviral therapy was started, however, with no effect. Death occurred three weeks after presentation because of acute brain swelling. Autopsy revealed leptomeningeal gliomatosis and diffuse parenchymal infiltration at the cerebellar and left temporal lobe. Primary leptomeningeal gliomatosis (PLMG) is a rare event. The reported case and the reviewed literature evidence that clinical signs at presentation are non-specific, CSF negative findings are common, the diagnosis is always delayed and the prognosis dismal.
Primary diffuse leptomeningeal gliomatosis: An autopsy case report
2014
limb since 20 days. There was no history of fever, seizures, altered sensorium, and focal weakness and the past history was unremarkable. General and systemic examinations were normal. Neurological examination revealed bilateral papilledema, left sixth nerve paresis with action tremor and signs of cerebellar dysfunction in left upper limb. There were no meningeal signs. Rest of the examination was normal. Magnetic resonance imaging (MRI) brain showed diffuse, thick leptomeningeal enhancement [Figure 1a] with few nodular
Leptomeningeal Tumor: The “Plain Vanilla ” Approach Remains
2016
Findings from the most recent studies suggest that the incidence of leptomeningeal tumor continues to increase. Multiple reasons are generally cited. First, methods of diagnosis have improved with respect to the evaluation of cytologic features. Second, as pa-tients continue to live longer with their systemic tu-mors due to refinements in treatment, ancillary com-plications of the disease, such as leptomeningeal tumor, have more time to develop. Third, improve-ments in imaging increase the number of leptomen-ingeal tumor diagnoses. The major advance in the imaging evaluation of leptomeningeal tumor occurred with the introduction of contrast agents and their use with routine T1-weighted spin-echo sequences. Previous evaluations
Primary diffuse leptomeningeal glioneuronal tumors
Brain Tumor Pathology, 2014
Diffuse leptomeningeal disseminated glioneuronal tumor (DL-GNT) is a rare brain tumor that presents as a plaque-like subarachnoid tumor, commonly involving the basal cisterns and interhemispheric fissure of children but lacking intraparenchymal tumor. Histologically, the tumors are composed of sheets of monotonous rounded cells. Here, we report three cases of DL-GNTs, focusing on clinicopathologic features. Two patients were adult male, but one patient was child. The patients presented with seizures (n = 1) or headaches (n = 2). In all patients, radiography revealed characteristic leptomeningeal thickening and enhancement with minor superficial parenchymal lesions. All three cases were diffusely positive for both GFAP and synaptophysin, and scattered positive for OLIG2 and NeuN, but negative for IDH-1 (H09). Electron microscopic examination showed astrocytic and neuronal differentiation. The patient with the anaplastic tumor died due to aggressive progression of the tumor, but the remaining two patients were stable without tumor recurrence for 23 and 37 months. Thus, these findings suggest that DL-GNT can occur in both children and adult and both supra-and infra-tentorial leptomeninges. It has unique radiological and histopathological features and biological behavior. Further clinicopathological data with molecular genetic study are required for establishing DL-GNT as a unique entity.
Diffuse Leptomeningeal Glioneuronal Tumors: A New Entity?
Brain Pathology, 2010
The peculiar radiological and pathological findings of four pediatric cases admitted to the University Hospital of Padua between 1990 and 2007 are described. In all cases, the contrast-enhanced head and spine magnetic resonance images revealed thickened and abnormally enhancing subarachnoid spaces particularly at the level of basal cisterns and interhemispheric fissure. Furthermore, small cystic lesions scattered throughout the brain and mainly in the cerebellum were also visible. All patients were missing a well-defined intraparenchymal mass, although during the follow-up a small intramedullary lesion appeared within the cervical spine in two and subsequently in the frontal horn of the left lateral ventricle in one of those. All patients presented an indolent long-term follow-up. Histologically, the tumors were composed by a monotonous population of cells arranged in straight lines or in small lobules. The cells were characterized by round to oval nuclei with finely granular dispersed chromatin, inconspicuous nucleoli with oligodendrocyte-like features. The morphological and immunohistochemical findings suggested in all cases a "glioneuronal commitment" of the tumors. Because of the unique similar clinical and neuroradiological characteristics, we propose this small series of tumors as a new possible distinct pathological and clinical entity.
2000
Summary: An autopsy-proved case of cerebral and medullary leptomeningeal gliomatosis and diffuse osteoblastic metastases without evidence of intraaxial tumor is described. MR findings included diffuse thickening of the cerebral and medullary lepto- meninges on T1-weighted, proton density-weighted, and T2- weighted images and abnormal enhancement of the sulci and cisterns of the cerebrum, brain stem, cerebellum, and medulla on postcontrast T1-weighted
Primary Diffuse Leptomeningeal Gliomatosis: Radiological/Pathological Features
Case Reports in Neurological Medicine, 2016
We present the case of a 43-year-old lady who presented with headaches, visual impairment, and seizures, progressing rapidly over the course of a few weeks. Extensive workup excluded an inflammatory or infectious cause. Imaging studies revealed diffuse thickening of the leptomeninges and serial CSF analysis showed raised opening pressures and increased protein levels. A diagnostic biopsy of the lower thoracic dura confirmed the diagnosis of primary diffuse leptomeningeal gliomatosis (PDGL). She was managed supportively for her symptoms and unfortunately she passed away a few weeks later.
Cureus, 2022
Leptomeningeal carcinomatosis (LC) is a rare complication of primary malignancy that spreads to leptomeninges and cerebrospinal fluid (CSF). Due to its rarity, it is often diagnosed as a late complication of an advanced tumor. This report presents a case study of a 72-year-old nonsmoking female with multiple comorbidities with two-week rapidly progressive cognitive decline and extrapyramidal symptoms (EPS). She presented with speech difficulties, tension headaches, and episodes of inattention. On examination, she had a masked face, mild bradykinesia, mild rigidity more apparent in the limbs than axially, and slight hyperreflexia in the lower limbs with a normal plantar reflex (down-going). Magnetic resonance imaging (MRI) of the brain with gadolinium showed diffuse leptomeningeal dissemination. CT of the right lower lobe showed lobe apical segment mass lesion with air bronchogram extension to the hilum, which raised the suspicion that the patient had lung cancer. The microscopic analysis of cerebrospinal fluid (CSF) cytology showed poorly differentiated malignant cells favoring adenocarcinoma. Based on these investigations, leptomeningeal dissemination on the MRI led to a wide differential diagnosis; however, given the findings in the CT scan and CSF, the patient was diagnosed with leptomeningeal carcinomatosis secondary to metastatic lung cancer. Although LC is a rare terminal complication that presents with a wide range of symptoms, typically including headache, altered mental status, diplopia, back pain, cerebral signs, and leg weakness, our patient presented with an uncommon presentation, which was EPS. Therefore, this case report highlights the importance of early detection of LC in any patient presenting with unspecific neurological manifestations.
Diffuse Leptomeningeal Glioneuronal Tumor: A Rare Case Report with Review of Literature
Asian Journal of Neurosurgery
Diffuse leptomeningeal glioneuronal tumor (DLGNT) is a rare tumor that was defined as a separate entity in the 2016 World Health Organization classification of brain tumors. It is most common in the pediatric age group. The diagnosis of this tumor can be made preoperatively by its characteristic imaging findings of diffuse intracranial and intraspinal nodular leptomeningeal thickening and enhancement associated with small cyst-like nonenhancing lesions in the brain and spinal cord. We report a case of DLGNT in a 7-year-old male presenting with hydrocephalus. The patient was treated successfully by cerebrospinal fluid (CSF) diversion followed by biopsy of the lesion from the cerebellum. Histopathology and immunohistochemistry confirmed the diagnosis of DLGNT. Our case would hopefully increase the awareness regarding this rare tumor and facilitate an early diagnosis and management of the affected patients with atypical radiological features.