Primary Diffuse Leptomeningeal Gliomatosis Research Articles

Primary diffuse leptomeningeal gliomatosis with parenhycmal infiltration: A clinico- radio-pathological correlation

Primary Diffuse Leptomeningeal Gliomatosis (PDLG), as first described by Moore (1954), is a very rare form of cancer resulting from the meningeal heterotopic astrocytes that convert into malignancies and possibly causing obstruction on the Virchow spaces leading to cystic parenchymal infiltration. This case, 4th documented case of atypical parenchymal infiltration, is a 45 -year old male with gradually progressive headache, difficulty sleeping and profound anxiety, left-sided numbness and seizures. Cranial CT- Scan showed right thalamic lesion without hydrocephalus. A possible thalamic infarct or cavernoma was considered. Subsequent MRI showed the same thalamic lesion but now with focal leptomeningeal enhancement probably secondary to glioma versus tuberculosis. He was given anti-Koch’s medications and steroids. Leptomeningeal biopsy confirmed this malignancy. Post- mortem autopsy revealed thickened leptomeninges, right thalamic mass involving the centromedial and posterolateral portion and small cystic lesions all over the surface. Histopathology showed Virchow -robin space infiltration proving the parenchymal involvement through the pial vessels. This case highlighted the possibility of parenchymal involvement which challenged the traditional diagnostic criteria and may now serve as a basis for a revised diagnostic criteria . In this case, imaging also showed an underestimation of the extent of atypical brain parenchymal infiltration. Hence, headache coupled by leptomeningeal enhancement should always warrant close imaging surveillance and possibly repeated biopsies to rule out this exceptional condition.

Malignant primary diffuse leptomeningeal gliomatosis with histone H3.3 K27M mutation

IntroductionMalignant primary diffuse leptomeningeal gliomatosis (MPDLG) are rare central nervous system neoplasms associated with a poor outcome. Case reportWe report the case of a 40-year-old woman who presented with unusual worsening of bilateral sciatica, headaches, diplopia and a left proptosis. MRI of the head and spine showed multiple leptomeningeal lesions with no intra parenchymal involvement. The search for a primary tumor was negative. An open surgical biopsy of the prominent intradural lumbar tumor was performed within a week. Histopathology, immunochemistry and molecular analyses revealed a malignant glioma with histone H3.3 K27M mutation. The patient was referred to the neuro-oncologist for chemotherapy and craniospinal radiotherapy. Despite aggressive therapy, she died of disseminated tumoral progression, 18 weeks after the diagnosis. ConclusionMPLG is a rare tumor which should be considered whenever a patient presents with diffuse or multinodular meningeal contrast-enhancing lesions. Some cases of MLPG share histological and immunophenotypical features with diffuse midline gliomas H3-K27M-mutant, a rapidly fatal disease. The diagnosis remains histopathological and, therefore a biopsy is obligatory without delay. Immunohistochemistry and/or molecular analyses are now currently essential for a formal classification and, to provide a better prediction of clinical outcome, particularly in this heterogeneous group of tumors.

Can Medulloblastoma be Presented with Primary Diffuse Leptomeningeal Gliomatosis? Case Report and Literature Review

Primary diffuse leptomeningeal Gliomatosis (PDLG) is a rare neoplastic condition characterized by primary infiltration of leptomeninges by malignant glial cells which is extremely rare to be as a result of medulloblastoma. To the best of our knowledge, there are only 5 reported localized forms of PDLG cases due to medulloblastoma affecting mainly posterior fossa and/or part of cerebrum. In this article, we are reporting for the first time a case of PDLG with extensive diffuse involvement of leptomeninges due to medulloblastoma extending from the cerebrum to sacral area in 4-year old child presented with acute headache, abducent nerve palsy and papilledema with dramatic response to chemotherapy.

Primary Diffuse Leptomeningeal Gliomatosis: Radiological/Pathological Features.

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.

Primary Diffuse Leptomeningeal Gliosarcomatosis

Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare condition with a fatal outcome, characterized by diffuse infiltration of the leptomeninges by neoplastic glial cells without evidence of primary tumor in the brain or spinal cord parenchyma. In particular, PDLG histologically diagnosed as gliosarcoma is extremely rare, with only 2 cases reported to date. We report a case of primary diffuse leptomeningeal gliosarcomatosis. A 68-year-old man presented with fever, chilling, headache, and a brief episode of mental deterioration. Initial T1-weighted post-contrast brain magnetic resonance imaging (MRI) showed diffuse leptomeningeal enhancement without a definite intraparenchymal lesion. Based on clinical and imaging findings, antiviral treatment was initiated. Despite the treatment, the patient's neurologic symptoms and mental status progressively deteriorated and follow-up MRI showed rapid progression of the disease. A meningeal biopsy revealed gliosarcoma and was conclusive for the diagnosis of primary diffuse leptomeningeal gliosarcomatosis. We suggest the inclusion of PDLG in the potential differential diagnosis of patients who present with nonspecific neurologic symptoms in the presence of leptomeningeal involvement on MRI.

NI-47 * GLIOMATOSIS MENINGI AND GLIOMATOSIS CEREBRI: KISSING COUSINS? CASE REPORT AND REVIEW OF THE LITERATURE

Primary Leptomeningeal Gliomatosis is a rare neoplastic disease of the central nervous system. This tumor arises solely in the leptomeninges without a dominant intraparenchymal brain or spinal cord lesion. The diagnosis is very challenging during life, with fewer than 100 reported cases in the medical literature. Initially thought to present as either a nodular or diffuse subtype (Primary Diffuse Leptomeningeal Gliomatosis: PDLG), the nodular forms are currently felt to be more appropriately termed Primary Leptomeningeal Glioma. This tumor is too rare to have been assigned a WHO classification. We propose that the term PDLG be replaced with Gliomatosis Meningi to make the relationship to the other form of Gliomatosis—Gliomatosis Cerebri, clearer. We report the case of a 63 year-old man who first presented with a grand mal seizure followed by progressive neurological decline, hydrocephalus, and spinal radiculopathy. He had an initial tentative diagnosis of neurosarcoidoisis radiographically. He went on to have progressive leptomeningeal enhancement on MRI involving the brain and the spinal cord with a negative initial temporal lobe biopsy followed by a subsequent biopsy of the leptomeninges, which revealed Leptomeningeal Gliomatosis. He continued to decline clinically and died 9 months after symptom onset despite treatment with craniospinal radiation therapy and chemotherapy with oral Temozolamide. We present a comprehensive review of the cases published in the literature to date. We restricted our search to the cases that described diffuse meningeal disease and excluded solitary tumors, non glial tumors as well as secondary leptomeningeal spread. A comprehensive review of the natural history of this disease is provided in the hope that this diagnosis can be made earlier in the future so that treatment can be offered before the tumor is too advanced.

Primary diffuse leptomeningeal gliomatosis diagnosed on CSF cytology: perseverance pays off

Primary diffuse leptomeningeal gliomatosis is a rare neoplasm defined by the infiltration of the subarachnoid spaces by glial neoplastic cells, without there being a detectable intra-axial glioma. Unlike the meningeal...

A case of primary diffuse leptomeningeal gliomatosis

Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare and fatal disease characterized by diffuse infiltration of the leptomeninges by neoplastic glial cells without evidence of tumor in the brain parenchyma or spinal cord. We report a 60-year-old man with PDLG. He suffered transient right hemiparesis and generalized seizures. MRI showed diffuse leptomeningeal thickening and enhancement throughout the brain and spinal cord without any intraaxial involvement. Biopsy resulted in a diagnosis of glioblastoma with methylated MGMT promoter and wild-type IDH1. He underwent craniospinal radiotherapy and temozolomide treatment but despite concomitant adjuvant therapy he died 8 months after initial presentation.

Primary Diffuse Leptomeningeal Gliomatosis Mimicking Tuberculous Meningitis

Primary diffuse leptomeningeal gliomatosis is a disease with an aggressive course that can result in death. To date, 82 cases have been reported. Here, the case of a 3-year-old male patient presenting with strabismus, headache, and restlessness is reported. Physical examination revealed paralysis of the left abducens nerve, neck stiffness, and bilateral papilledema. Tuberculous meningitis was tentatively diagnosed, and antituberculosis treatment was initiated when cranial imaging revealed contrast enhancement around the basal cistern. Craniocervical magnetic resonance imaging (MRI) was performed when there was no response to treatment, and it revealed diffuse leptomeningeal contrast enhancement around the basilar cistern, in the supratentorial and infratentorial compartments, and in the spinal region. Primary diffuse leptomeningeal gliomatosis was diagnosed by a meningeal biopsy.

Teaching Neuro Images: FDG-PET imaging in primary diffuse leptomeningeal gliomatosis

A 50-year-old man presented with progressive tetraparesis and loss of vision. Inflammation and malignancy were considered based on diffuse meningeal enhancement on MRI (figure 1) and CSF content (very high protein, mild pleocytosis, and low to normal glucose). CSF cultures and cytologic examinations, including flow cytometry, were normal. FDG-PET showed increased uptake in the spinal cord and brain (figure 2), matching primary diffuse leptomeningeal gliomatosis (PDLG), leptomeningeal carcinomatosis/lymphoma, or isolated CNS infection.1 A temporal leptomeningeal-cortical biopsy confirmed PDLG.

A FATAL CASE OF ‘PRIMARY’ HEADACHE

We describe a case of a 33 year old caucasian male with a subacute history of headaches presenting to the medical team with pyrexia, confusion and hallucinations. He subsequently became...

Primary spinal leptomeningeal gliomatosis in a 3-year-old boy revealed with MRI and FDG PET/CT mimicking tuberculosis meningitis

A 3-year-old boy was referred to neurologist for inward devition of his left eye, weakness, lack of appetite, headache and ehavioral change. On physical examination, the findings of ilateral abducens nerve paralysis were noted. Cranial magnetic esonance imaging (MRI) revealed diffuse leptomeningeal contrast nhancement at the supra-infratentorial regions predominantly nvolving the basal cisterns and also hydrocephalus and initially uggesting basilar meningitis. Cerebrospinal fluid (CSF) studies howed normal glucose and elevated protein levels but no tumor ells. No growth was detected in any of the bacterial cultures ncluding acid-resistant bacteria. The patient was started on oral nti-tuberculosis medication and corticosteroid treatment with he presumed diagnosis of tuberculosis meningitis and a ventricuoperitoneal (VP) shunt was inserted due to hydrocephalus. After months of treatment, the patient was re-admitted to hospial with vomiting, convulsion and confusion. Cranial MRI showed he similar findings to previous one, and spinal MRI depicted iffuse leptomeningeal contrast enhancement as well as microystic changes along the spinal cord (Fig. 1). Histopathology of the ural biopsy material revealed widespread glioma infiltration of he subarachnoid space suggestive of leptomeningeal gliomatosis. his patient with unknown primary was referred for FDG PET/CT. nder child sedation, brain and whole body non-contrast enhanced ET/CT images were obtained 90 min after the intravenous injecion of 148MBq (4mCi) 18F FDG using a Biograph 6 PET/CT. The can depicted slightly increased FDG uptake (SUVmax 2.1)along the pinal cord without any other FDG-avid primary tumor. The patient as started on chemotherapy including vincristine and carboplatin Figs. 2 and 3). His symptoms were relieved after completion of hemo. Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare isease thought to arise from isolated astrocytic cell nests also nown as heterotropic neuroglial tissue within the leptomeninges HLNT) in the subarachnoid space. The HLNT can transform into two

Primary Diffuse Leptomeningeal Gliomatosis Coincident With a Melanocytic Disorder

Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare neoplasm of the leptomeninges associated with limited survival time. Around 60 cases of PDLG have been reported in the literature and most of them were diagnosed at post mortem examination. We describe the longest survival in a PDLG case, a 24 year old man who survived for 30 months with an aggressive treatment approach that included chemoradiation and repeated surgical excisions of tumor. Our patient also had multiple dysplastic nevi and dysmorphic facial features raising the suspicion of an underlying genetic disorder. doi: http://dx.doi.org/10.4021/jmc1361w

Primary Diffuse Leptomeningeal Gliomatosis in Children

To describe a rare case of primary diffuse leptomeningeal gliomatosis (PDLG) presenting with progressive proptosis and direct involvement of the optic nerve sheath in a child and review of the relevant literature. Retrospective review of a single case and systematic literature review of 26 biopsy-proven cases reported in the MEDLINE-indexed English literature. A 10-year-old girl developed proptosis and progressive visual loss associated with thickening of the optic nerve sheaths and dilation of the subarachnoid spaces with multilobulated appearance of the brain meninges and thickened peripheral nerve root sheaths. Biopsy of the optic nerve sheath was diagnostic. The patient underwent chemotherapy combined with oral temozolomide and conformational radiotherapy to the brain and spine. She died 3 years after the onset of the disease. An extensive review of the published literature using the key words "primary diffuse leptomeningeal gliomatosis" and "optic nerve" confirmed the case herein reported to be the first case of primary diffuse leptomeningeal gliomatosis in which direct optic nerve infiltration was demonstrated during the course of the disease. Immunohistochemistry demonstrated expression of CD56 and glial fibrillary acidic protein, and an elevated level of Ki-67; all the other markers were negative. According to a comprehensive literature review, we report the first case of PDLG that presented with bilateral proptosis and direct involvement of the optic nerve during the course of the disease. These new findings may explain an alternative mechanism of visual loss and proptosis in PDLG. We emphasize the importance of close collaboration between neurologists and ophthalmologists in all cases of visual symptoms associated with a neurologic condition. In case of optic nerve involvement, ophthalmologists could provide an easier route to achieve tissue specimen early in the course of this rare and fatal disease.

Uncommon Leptomeningeal and Dural Based Disorders in a Community Based Hospital (P03.144)

Objective: To describe the clinical, radiological and neuropathological manifestations, response to treatment, and prognosis of non-meningothelial disorders diagnosed at the community hospital. Background Leptomeningeal and dural involvement as detected by neuroimaging is problematic to diagnose because of the broad differential diagnosis. However, it frequently requires an aggressive diagnostic approach as exact diagnosis is crucial for appropriate treatment and prognosis. Design/Methods: We retrospectivelly indentified nine patients with rare leptomeningeal disorders, treated in our facility over the last eight years. All diagnoses were pathologically confirmed. Collected data included demographics, neurological symptoms, radiographic and biopsy/autopsy findings. Results: Two patients were diagnosed with disseminated primary diffuse leptomeningeal gliomatosis, three with isolated CNS lymphoma involving meninges or dura, one with Rosa-Dorfman disease, one inflammatory myofibroblastic tumor, and two with granulomatous disorder. Among the patients with lymphoma, two had diffuse large B cell lymphoma and one had marginal zone lymphoma (MALToma). Patients with leptomeningeal gliomatosis had progressive course with fatal outcome. Neurological manifestations included encephalopathy, cognitive decline and subsequent paraparesis. One patient required placement of ventriculo-peritoneal shunt for acute hydrocephalus. The other patients, including lymphoma cases, responded well to treatment and so far all but one have remained in remission. In this group, clinical manifestations included headaches and seizures that were controlled on anticonvulsants. Conclusions: Based on our experience, uncommon leptomeningeal and dural disorders need to be aggressively investigated as treatment of some disorders can be successful. Neurologists in a community based practice should have a high level of clinical awareness and be familiar with a variety of leptomeningeal disorders othe than infection and leptomeningeal spread from metastatic cancer. Disclosure: Dr. Bronstein has nothing to disclose. Dr. Pinsky has nothing to disclose. Dr. Danesh has nothing to disclose. Dr. Khoiny has nothing to disclose. Dr. Petrovic has nothing to disclose.

Neuro-Ophthalmological Features of Primary Diffuse Leptomeningeal Gliomatosis

We performed an in-depth study of the neuro-ophthalmologic signs and symptoms of a rare but fatal disease known as primary diffuse leptomeningeal gliomatosis (PDLG). Two new cases of PDLG are described, and 22 published cases reviewed. Papilledema and sixth nerve palsy are the most common neuro-ophthalmic findings. Other abnormalities include third and fourth nerve palsies, nystagmus, and vision loss. Involvement of the visual system may be part of the initial presentation of PDLG.

Primary Diffuse Leptomeningeal Gliomatosis Mimicking Meningeal Tuberculosis

Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare condition, with only 45 cases recorded to date, characterized by infiltration of the meninges by glial cells without evidence of primary tumor in the brain or spinal cord parenchyma. Here, we describe a patient with PDLG who was managed with tuberculostatic drugs owing to multiple findings that were suggestive of tuberculous meningitis. A 19-year-old woman presented with headaches and behavioral changes. A sudden decrease in visual acuity with papilledema, bilateral sixth nerve palsies, and neck stiffness developed. Lumbar puncture showed elevated opening pressure (50 cm H2O). Cerebrospinal fluid (CSF) analysis showed glucose 30 mg/dL, protein 26.5 mg/dL, white blood cell count 150 (60% lymphocytes, 40% neutrophils). The second sample of CSF provided adenosine deaminase activity 21.9 U/L. Polymerase chain reaction for Koch's bacillus was positive in the third CSF sample. Magnetic resonance imaging revealed meningeal thickening of the quadrigeminal cistern, tentorium cerebelli, cerebral convexity, and spinal cord, with gadolinium enhancement in nodular lesions. The patient died 22 weeks after symptom onset owing to brainstem infarction. Postmortem pathologic studies revealed PDLG. This entity should be included in the differential diagnosis of tuberculous meningitis that does not respond to treatment with antituberculous drugs. Surgical biopsy should be considered in contrast-enhanced areas in magnetic resonance imaging.

Primary diffuse leptomeningeal gliosarcomatosis with a sphenoid/sellar mass: Confirmation of the ectopic glial tissue theory?

Gliosarcoma is a rare glioblastoma variant, classically arising in the cerebral hemispheres. We report a patient with primary diffuse leptomeningeal gliomatosis (PDLG) with a sphenoid sinus and sellar mass. An 84-year-old woman presented with progressive headache and right-sided visual failure, associated with ipsilateral oculomotor nerve palsy and left temporal field loss. Neuraxial MRI showed a large lesion within the sphenoid sinus and sella resulting in chiasmal compression, and diffuse cranial and spinal leptomeningeal enhancement. Endoscopic transphenoidal biopsy and debulking of the sphenosellar lesion was performed, and gliosarcoma was diagnosed on histopathological examination. The patient was palliated due to poor performance status. To our knowledge, this is the only report of gliosarcoma within the paranasal sinuses and the second report of PDLG where the histological analysis has confirmed gliosarcoma. We believe this adds significant weight to the theory that heterotopic nests of glial tissue, in this instance within the sphenoid or sella, are the putative origin of PDLG.

Long survival of primary diffuse leptomeningeal gliomatosis following radiotherapy and temozolomide: case report and literature review

ObjectivePrimary diffuse leptomeningeal gliomatosis (PDLG) is a rare neoplasm with a short survival time of a few months. there is currently no standardized therapeutic approach for PDLG.Materials and methodsWe report on a 53-year-old male patient who presented with epileptic seizures, gait disturbance, paraparesis and sensory deficits in the dermatomes T8-10.ResultsMagnetic resonance imaging (MRI) revealing numerous spinal and cranial gadolinium-enhancing nodules in the meninges and histopathology led us to diagnose primary diffuse leptomeningeal gliomatosis with WHO grade III astrocytic cells. Consecutively, the patient underwent craniospinal radiotherapy (30 Gy) and 11 sequential cycles of temozolomide. This regimen led to partial tumor regression. Thirteen months later, spinal MRI revealed tumor progression. Second-line chemotherapy with 5 cycles of irinotecan and bevacizumab did not prevent further clinical deterioration. The patient died twenty-two months after diagnosis, being the longest survival time described thus far with respect to PDLG consisting of astrocytic tumor cells.ConclusionsRadiochemotherapy including temozolomide, as established standard therapy for brain malignant astrocytomas, might be valid as a basic therapeutic strategy for this PDLG subtype.

A case of primary diffuse leptomeningeal gliomatosis predominantly involving the cervical spinal cord and mimicking chronic meningitis

Gliomas may rarely arise in the leptomeninges without any evidence of intraaxial involvement. A case of primary diffuse leptomeningeal gliomatosis (PDLG) histologically diagnosed as oligoastrocytoma is presented. A 50-year-old woman presented with nausea, vomiting and headache. Magnetic resonance imaging (MRI) of cranium and cervical region revealed dural thickening starting from the craniocervical junction to the level of C4 without any parenchymal lesions. CSF examination showed an increase in protein and decrease in glucose levels. There were neither any kind of atypical cells nor any kind of growth in bacterial cultures. The patient underwent biopsy at the level of C1 for diagnosis. The specimen was diagnosed as primary diffuse leptomeningeal gliomatosis, with phenotypic features of astrocytoma and oligodendroglioma.