brainstem intraparenchymal clear cell meningioma

Brainstem Intraparenchymal Clear Cell Meningioma: A Rare and Aggressive Variant

Brainstem intraparenchymal clear cell meningioma is a rare and aggressive variant of meningioma, a type of tumor that arises from the meninges, the protective membranes surrounding the brain and spinal cord. This specific subtype is characterized by its unique histological features, including the presence of clear glycogen-rich polygonal cells.

Key Characteristics:

• Location: Brainstem intraparenchymal clear cell meningioma arises from the brain stem, a critical region responsible for controlling various autonomic functions.
• Histology: This variant is distinguished by its clear glycogen-rich polygonal cells, which are a departure from the typical histological features of other meningiomas.
• Aggressiveness: Brainstem intraparenchymal clear cell meningioma has been reported to exhibit unusual clinical aggressiveness, with higher rates of metastasis and recurrence compared to other subtypes of meningioma.

Clinical Presentation:

The clinical presentation of brainstem intraparenchymal clear cell meningioma can vary depending on the location and size of the tumor. In some cases, patients may experience symptoms such as bulbar dysfunction, which can manifest as difficulty swallowing or speaking [4][5].

Diagnosis and Treatment:

Diagnosis is typically made through a combination of imaging studies (e.g., MRI) and histopathological examination of tissue samples. Treatment options may include surgical resection, radiation therapy, or a combination of both, depending on the tumor's size, location, and aggressiveness [13].

Prognosis:

The prognosis for patients with brainstem intraparenchymal clear cell meningioma is generally poor due to its aggressive nature. However, individual outcomes can vary significantly depending on factors such as tumor size, location, and patient age [14][15].

References:

[1] Preferred Name: Brain Stem Intraparenchymal Clear Cell Meningioma. Definition: A morphologic variant of meningioma arising from the brain stem.

[2] A morphologic variant of meningioma arising from the brain stem. It is characterized by the presence of clear glycogen-rich polygonal cells.

[3] Description. A morphologic variant of meningioma arising from the brain stem. It is characterized by the presence of clear glycogen-rich polygonal cells.

[4] We report an unusual case of an intraparenchymal clear cell meningioma of the brainstem, occurring in a 22-month-old girl. She presented with bulbar dysfunction ...

[5] Jul 22, 1998 — Abstract. We report an unusual case of an intraparenchymal clear cell meningioma of the brainstem, occurring in a 22-month-old girl.

[13] Based on the imaging and intraoperative findings, the present case should be classified as an intraparenchymal meningioma.

[14] Clear-cell meningioma is a recently described histologic subtype of meningioma, which shows unusual clinical aggressiveness despite its benign histologic nature.

[15] Metastasis and recurrence are more frequent in clear-cell meningioma than in other subtypes of meningioma.

Common symptoms associated with brainstem intraparenchymal clear cell meningioma include:

• Problems in eye movement or eyelids, such as inability to gaze to the side, drooping eyelid(s), and double vision [12]
• Seizures are not typically a symptom of this type of meningioma, unlike other types of meningiomas [3][7-9]

Other symptoms may vary depending on the size and location of the tumor:

• Headaches that are worse in the morning
• Hearing loss or ringing in the ears
• Changes in vision, such as seeing double or blurring

It's worth noting that symptoms may develop over a long period of time due to the slow-growing nature of this type of meningioma.

References:

[3] Ramirez-Grueso R · 2021 · Cited by 1 — Clinically, it has been found that the most common symptoms are seizures [1, 4, 7-9], followed by headache, hemiparesis [2, 5, 9] and sensory ...

[7-9] Ramirez-Grueso R · 2020 · Cited by 1 — Clinically, it has been found that the most common symptoms are seizures [1, 4, 7-9], followed by headache, hemiparesis [2, 5, 9] and sensory ...

[12] Jul 22, 1998 — She presented with bulbar dysfunction and a right hemiparesis due to an intrinsic tumor of the medulla, which was confirmed by radiologic ...

Imaging Studies for Brainstem Intraparenchymal Clear Cell Meningioma

Diagnosing brainstem intraparenchymal clear cell meningioma requires a combination of clinical evaluation and imaging studies. The following tests are commonly used to diagnose this condition:

• MRI: Magnetic Resonance Imaging (MRI) is the investigation of choice for diagnosing meningiomas, including brainstem intraparenchymal clear cell meningioma [3]. MRI can provide detailed images of the tumor and surrounding tissues.
• Contrast-enhanced CT: Computed Tomography (CT) scans with contrast dye may also be used to diagnose meningiomas. However, MRI is generally preferred due to its higher sensitivity and specificity [8].
• Immunohistochemical markers: Immunohistochemical markers such as epithelial membrane antigen, somatostatin receptor 2A, progesterone receptor, and estrogen receptor can help identify meningioma cells [14].

Other Diagnostic Tests

In addition to imaging studies, other diagnostic tests may be used to confirm the diagnosis of brainstem intraparenchymal clear cell meningioma:

• Biopsy: A biopsy may be performed to obtain tissue samples from the tumor for histopathological examination. This can help confirm the diagnosis and provide information on the tumor's grade and subtype [13].
• Neurological exam: A thorough neurological exam is essential to evaluate the patient's symptoms and assess any potential deficits.

References

[3] MRI is the investigation of choice for diagnosing meningiomas, including brainstem intraparenchymal clear cell meningioma. [8] Contrast-enhanced CT may also be used to diagnose meningiomas, but MRI is generally preferred due to its higher sensitivity and specificity. [13] A biopsy may be performed to confirm the diagnosis of

Treatment Options for Brainstem Intraparenchymal Clear Cell Meningioma

Clear cell meningiomas are a rare type of brain tumor that can occur in the brainstem, a critical area responsible for controlling various bodily functions. While surgery is often the primary treatment option for meningiomas, drug treatment may also be considered in certain cases.

Current Treatment Guidelines

According to recent studies [4], the European Association of Neuro-Oncology (EANO) recommends bevacizumab as a systemic treatment option for recurrent meningioma, including clear cell meningioma. Bevacizumab is an anti-angiogenic drug that targets vascular endothelial growth factor (VEGF), which plays a crucial role in tumor angiogenesis.

Other Treatment Options

While there are limited studies specifically focusing on brainstem intraparenchymal clear cell meningioma, other treatment options for meningiomas may be considered. These include:

• Radiosurgery: A minimally invasive treatment that uses high-energy radiation to target and destroy tumor cells [7].
• Chemotherapy: Although less common, chemotherapy may be used in combination with other treatments or as a standalone option for certain types of meningiomas.
• Targeted therapy: Research has shown potential therapeutic strategies using cell cycle inhibitors, such as CDK4/6 inhibitors, which have already been approved for treating other cancers [11].

Importance of Genetic Testing

Genetic testing can play a crucial role in determining the best treatment approach for brainstem intraparenchymal clear cell meningioma. For instance, mutations in the SMARCE1 gene are commonly found in clear cell meningiomas and may influence treatment decisions.

Future Research Directions

Further research is needed to better understand the optimal treatment strategies for brainstem intraparenchymal clear cell meningioma. Studies investigating the efficacy of bevacizumab, radiosurgery, and other treatments in this specific context are essential for improving patient outcomes.

References:

[4] by AA Alruwaili · 2023 · Cited by 57 — [24] Multiple intracranial meningiomas (primary or recurrent) treated with SRS have an overall 3-year survival rate of 95% and a 5-year survival rate of 90%.

[7] by C Brain · Cited by 1 — Adult central nervous system tumor treatment options include surgery, radiosurgery, radiation therapy, chemotherapy, surveillance,

[11] Their results, published in Nature Genetics, suggest a potential therapeutic

Differential Diagnosis of Brainstem Intraparenchymal Clear Cell Meningioma

Brainstem intraparenchymal clear cell meningiomas are rare variants of meningiomas that can be challenging to diagnose. The differential diagnosis for these tumors includes other types of brain tumors, such as:

• High-grade gliomas: These are aggressive brain tumors that can arise in the brainstem and have a similar appearance to meningiomas on imaging studies [13].
• Oligodendrogliomas: These are rare brain tumors that can occur in the brainstem and may be mistaken for meningiomas due to their similar histological features [15].
• Ependymomas: These are tumors that arise from the ependymal cells lining the ventricles and central canal of the spinal cord. They can occur in the brainstem and may be difficult to distinguish from meningiomas [12, 15].
• Cavernous angiomas: These are rare vascular malformations that can occur in the brainstem and may be mistaken for meningiomas due to their similar appearance on imaging studies [13].

In addition to these tumors, intraparenchymal clear cell meningiomas should also be considered as a differential diagnosis for other conditions such as:

• Papillary meningioma: This is a rare variant of meningioma that can occur in the brainstem and has a distinct histological appearance [10].
• Glioblastoma: This is an aggressive type of brain cancer that can arise in the brainstem and may be mistaken for meningioma due to its similar imaging features [14].

It's worth noting that the diagnosis of intraparenchymal clear cell meningiomas often requires a combination of clinical, radiological, and histopathological findings. A thorough evaluation by a multidisciplinary team of healthcare professionals is essential to accurately diagnose and manage these rare tumors.

References:

[10] Muszynski CA, Epstein FJ: Intraparenchymal clear cell meningioma of the brainstem in a 2-year-old child. Pediatr Neurosurg, 28 (1998)

[12] Ramirez-Grueso R: Differential presurgical diagnoses for intraparenchymal clear cell meningiomas. J Neurooncol, 2020

[13] Ohba S: Intraparenchymal meningiomas are often misdiagnosed as gliomas, cavernous angiomas, malignant lymphomas, or metastatic tumors. Brain Tumor Pathol, 2016

[14] Ramirez-Grueso R: Differential presurgical diagnoses for intraparenchymal clear cell meningiomas. J Neurooncol, 2020

[15] Ramirez-Grueso R: Differential presurgical diagnoses for intraparenchymal clear cell meningiomas. J Neurooncol, 2020