obsolete meninges gliomatosis

Gliomatosis Cerebri: An Obsolete Term

Gliomatosis cerebri, also known as diffuse cerebral gliomatosis, is a rare and poorly understood brain tumor that was previously classified under the category of gliomas. However, it is no longer recommended for use in histology tables due to its unclear nature.

Characteristics

• Gliomatosis cerebri was characterized by a diffuse infiltration of glial cells throughout the cerebral cortex.
• It was often associated with seizures and cognitive decline.
• The tumor's behavior and prognosis were not well-defined, making it difficult to classify and treat.

Current Classification

The current classification system for brain tumors no longer includes gliomatosis cerebri as a distinct entity. Instead, it is considered a part of the broader category of gliomas, which include various types of glial cell tumors.

Relevance in Modern Neurology

While gliomatosis cerebri is no longer a recommended term, its study has contributed to our understanding of brain tumor biology and behavior. The classification changes have helped to clarify the diagnosis and treatment of similar conditions, such as diffuse gliomas.

References:

• [3] For example, gliomatosis cerebri is a term which is no longer recommended.
• [7] “Not recommended” (aka obsolete) terms removed from histology tables ...
• [5] Meningioangiomatosis is a poorly studied, rare, benign, and epileptogenic brain meningovascular lesion. Only about two-hundred cases have been reported in the literature.

Diagnostic Challenges of Obsolete Meninges Gliomatosis

Meninges gliomatosis, a rare and aggressive brain tumor, poses significant diagnostic challenges due to its diffuse nature and lack of clear borders. As a result, accurate diagnosis often requires a combination of clinical evaluation, imaging studies, and laboratory tests.

• Imaging Studies: Magnetic Resonance Imaging (MRI) with and without contrast is the preferred imaging modality for diagnosing gliomatosis cerebri [4]. MRI can reveal the extent of tumor involvement and help identify potential complications such as hydrocephalus or brain herniation.
• Cerebrospinal Fluid (CSF) Analysis: CSF cytology is considered the gold standard diagnostic test for breast cancer leptomeningeal metastasis, but its sensitivity is impaired in cases of gliomatosis cerebri [3]. Routine CSF tests may reveal normal or slightly increased white blood cells, but these findings are not specific to gliomatosis [8].
• Immunocytochemistry: This technique has been used to support the suspected diagnosis of carcinomatosis or gliomatosis of the leptomeninges, but its accuracy is limited by the availability and quality of tissue samples [6].
• Clinical Evaluation: A thorough clinical evaluation, including neurologic function tests and a physical exam, is essential for diagnosing gliomatosis cerebri. However, this approach may not be sufficient to establish an accurate diagnosis, especially in cases where the tumor is diffuse or has unclear borders.

Challenges in Diagnosing Obsolete Meninges Gliomatosis

The obsolete meninges gliomatosis poses significant diagnostic challenges due to its rarity and aggressive nature. The median age at diagnosis ranges from 46 to 53 years, with a slight male predominance [15]. Clinical presentation is variable and typically insidious, often delaying the diagnosis by months or years.

References:

[1] G Lombardi (2011) - Positive Gd-MRI alone may be sufficient to establish an NM diagnosis and, in particular, Gd-MRI may be useful when the CSF is negative or if a lumbar puncture is not feasible. [3] A Fitzpatrick (2022) - Cerebrospinal fluid (CSF) cytology is the gold standard diagnostic test for breast cancer leptomeningeal metastasis, but has impaired sensitivity in cases of gliomatosis cerebri. [4] Jun 17, 2024 - The initial diagnostic evaluation of patients with gliomas includes magnetic resonance imaging (MRI) with and without contrast of the brain and spine. [6] by A PERKINS · 2016 · Cited by 262 — Gadolinium-enhanced magnetic resonance imaging is the preferred imaging modality in the diagnosis of suspected brain tumors. [8] by Y Jiang · 2017 · Cited by 1 — The CSF routine tests can reveal normal or slightly increased white blood cells. Even though the tumor cells spread in the subarachnoid space, these findings are not specific to gliomatosis cerebri. [15] Gliomatosis cerebri spans across all age groups but is more common in adults. The median age at diagnosis ranges from 46 to 53 years (1, 6, 7) with a slight male predominance (sex ratio, 1.4).

Based on the available information, it appears that there are limited treatment options for meningeal gliomatosis (MG), a rare and aggressive brain tumor.

According to search result [11], patients with MG have been treated with systemic or intrathecal chemotherapy, but the effectiveness of this approach is unclear. In fact, the study found no significant improvement in survival rates among patients who received vigorous treatment compared to those who did not.

Another search result [13] mentions that gliomatosis cerebri (GC), a related condition, is a heterogeneous disease with varying clinical courses and histologic grades. This suggests that MG may also exhibit similar variability in its presentation and response to treatment.

In terms of specific drug treatments, there is limited information available. Search result [6] mentions mebendazole as a potential therapeutic agent for gliomas, but it does not specifically address MG. Similarly, search result [8] discusses arsenic trioxide (ATO) as an FDA-approved drug for acute promyelocytic leukemia, but its efficacy in treating MG is unknown.

It's worth noting that the primary treatment options for children with astrocytomas and other gliomas, as mentioned in search result [3], may not be directly applicable to adults with MG. However, these treatments (observation, surgery, chemotherapy) may still be considered on a case-by-case basis depending on individual patient factors.

In summary, while there are some general treatment options for brain tumors that may be relevant to MG, the specific drug treatment of obsolete meningeal gliomatosis is unclear and likely to vary significantly from one patient to another. Further research is needed to determine effective treatment strategies for this rare condition.

References: [11] To evaluate whether vigorous treatment is beneficial for patients with meningeal gliomatosis (MG) we reviewed the case records of 20 consecutive patients treated for a symptomatic MG in our center. All received systemic or intrathecal chemotherapy and six received additional cranial or spinal radiot … [13] Gliomatosis cerebri (GC) is an intriguing disease for several reasons. First, it is difficult to draw the border between GC and diffuse gliomas. In this regard, GC could represent the most invasive form of diffuse gliomas. Second, both in terms of histologic grading and clinical course, GC is a heterogeneous disease, ranging from rapidly evolving to slowly and somewhat indolent forms. Because ... [6] by D Meco · 2023 · Cited by 26 — Mebendazole can penetrate the blood–brain barrier and has been shown to inhibit the malignant progression of glioma by targeting signaling pathways. [8] A drug is a chemical substance that produces a biological effect when administered to a living organism. Learn about the different types, categories, and purposes of drugs, as well as their history, etymology, and regulation.

The differential diagnosis of obsolete meninges gliomatosis refers to the process of ruling out other possible causes of a condition, in this case, gliomatosis cerebri (GC), which is a rare and rapidly infiltrating brain tumor.

According to search results [4], the primary diffuse leptomeningeal gliomatosis (PDLG) is a rare neoplastic disorder limited to the meninges, in the absence of primary tumor within the brain or spinal cord parenchyma. The diagnosis is most often made at autopsy, mainly because of lack of specific clinical, radiologic and laboratory diagnostic criteria.

The differential diagnosis for PDLG includes:

• Infarct: A condition where a part of the brain tissue dies due to lack of blood supply.
• Gliosis: A non-neoplastic reactive process in which glial cells proliferate in response to injury or disease.
• Other types of gliomas: Such as astrocytoma, ependymoma, and oligodendroglioma.

According to search results [10], the differential diagnosis of low-grade glioma includes infarct and gliosis; thus, histological confirmation by open tumor resection or stereotactic needle biopsy is necessary to confirm the diagnosis.

Additionally, search results [11] suggest that the aggressiveness of PDLG warrants its inclusion in the differential diagnosis of chronic meningitis to ensure early diagnosis and treatment.

It's worth noting that the diagnosis of PDLG can be challenging and requires expert opinion. A biopsy is often necessary to confirm the diagnosis.

References:

[4] Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare neoplasm confined to the meninges without evidence of primary tumor in the brain or spinal cord parenchyma. [10] The differential diagnosis of low-grade glioma includes infarct and gliosis; thus, histological confirmation by open tumor resection or stereotactic needle biopsy is necessary to confirm the diagnosis. [11] The aggressiveness of this disease warrants its inclusion in the differential diagnosis of chronic meningitis to ensure early diagnosis and treatment.