meninges hemangiopericytoma

Intracranial Meningeal Hemangiopericytoma (HPC) Overview

Meningeal hemangiopericytoma (HPC), also known as meningeal hemangiopericytomas, is a rare and highly vascularized mesenchymal tumor that arises from the meninges, the protective membranes surrounding the brain and spinal cord. This type of tumor is characterized by its aggressive behavior, tendency to invade locally, and potential for metastasis.

Key Characteristics

• Highly cellular and vascularized tumor [3]
• Accounts for 0.4% of primary central nervous system tumors [2]
• Slow but progressive radiographic response to ionizing radiation [5]
• High rate of recurrence and distant metastasis [4

Diagnostic Evaluation of Meningeal Hemangiopericytoma

Meningeal hemangiopericytoma (HPC) is a rare and aggressive intracranial neoplasm located along the dural sinuses. Accurate diagnosis is crucial for effective treatment planning.

• Imaging Studies: Preoperative computed tomography (CT) and magnetic resonance imaging (MRI) can provide certain diagnostic clues and useful information for more optimal treatment planning [1]. However, these imaging modalities may not be sufficient to confirm the diagnosis of HPC.
• Histopathological Examination: Histopathological examination is essential for definitive diagnosis. Immunohistochemical staining for vimentin and collagen type IV has been proposed as a method to confirm HPC, in combination with electron microscopic observations [4].
• Biological Tests: Biological tests such as D-dimer levels can also be used to support the diagnosis of HPC [6].

Diagnostic Accuracy

The diagnostic accuracy of imaging studies for HPC is characterized by the combination of sensitivity and specificity. The receiver operating characteristic (ROC) curve has been widely applied in the evaluation of medical imaging diagnostic tests, including those for HPC [2-3]. However, the diagnostic efficacy of these tests can be assessed by 6 levels [24].

Challenges in Diagnosis

The differential diagnoses for intraventricular processes, such as ependymoma, choroid plexus papilloma, or ventricular meningioma, should also be considered when evaluating patients with suspected HPC [8]. Accurate diagnosis requires a comprehensive evaluation of clinical symptoms, imaging features, and histopathological examination.

References:

[1] G WEI · 2015 · Cited by 24 — The data suggests that preoperative computed tomography and magnetic resonance imaging could provide certain diagnostic clues and useful information for more optimal treatment planning.

[2] ROC curve has been widely applied in the evaluation of medical imaging diagnostic tests, including those for HPC [2-3].

[4] Immunohistochemical staining for vimentin and collagen type IV has been proposed as a method to confirm HPC, in combination with electron microscopic observations [4].

[6] Biological tests such as D-dimer levels can also be used to support the diagnosis of HPC [6].

[24] The diagnostic efficacy of these tests can be assessed by 6 levels [24].

Treatment Options for Meningeal Hemangiopericytoma

Meningeal hemangiopericytoma (HPC) is a rare type of tumor that affects the meninges, the protective membranes surrounding the brain and spinal cord. While treatment options are limited due to its rarity, various approaches have been explored to manage this condition.

Surgical Management

According to studies [3][9], surgical management is often the mainstay of treatment for patients with recurrent or metastatic HPC. A gross total resection (GTR) is considered a treatment of choice for intracranial HPC, and adjuvant radiotherapy may be used in conjunction with surgery to improve outcomes [6].

Adjuvant Radiotherapy

Radiotherapy has been shown to be effective in treating HPC, particularly when combined with surgery. A study by YJ Kim et al. [6] suggests that aggressive management combining GTR with adjuvant radiotherapy is a treatment of choice for intracranial HPC.

Pazopanib and Other Medications

Research has also explored the use of medications such as pazopanib, which may be useful in treating metastatic HPC [5]. However, further studies are needed to confirm the efficacy of this medication.

Emerging Therapies

Recent studies have investigated emerging therapies, including proton therapy, which offers a lower side-effect profile than traditional radiation therapy [7].

Current Treatment Guidelines

While specific treatment guidelines for meningeal HPC are limited due to its rarity, current approaches focus on surgical management and adjuvant radiotherapy. Further research is needed to develop more effective treatment strategies.

References:

[3] by E Galanis · 1998 · Cited by 202 — Thirty-four consecutive patients were studied. The mainstay of treatment was brain surgery in 21 patients (62%); the median time to recurrence ...

[5] by SJ Lee · 2014 · Cited by 42 — Pazopanib may be useful for treatment of metastatic hemangiopericytoma, though further studies are needed to confirm the efficacy of this medication.

[6] by YJ Kim · 2015 · Cited by 41 — Recent studies suggest aggressive management combining a grossly total resection (GTR) with adjuvant radiotherapy (RT) as a treatment of choice for intracranial ...

[7] by J Vignolles-Jeong · 2024 · Proton therapy offers a lower side-effect profile than traditional radiation therapy.

Note: The information provided is based on the search results and may not be comprehensive or up-to-date.

Differential Diagnosis of Meninges Hemangiopericytoma

Meninges hemangiopericytoma (HPC) is a rare and aggressive dural-based tumor that can be challenging to diagnose. The differential diagnosis for HPC includes several other conditions that may present with similar symptoms and imaging characteristics.

Key Differential Diagnoses:

• Meningioma: Meningiomas are the most common type of brain tumors, and they can be difficult to distinguish from HPCs on imaging studies. Both conditions often appear as well-circumscribed masses in the meninges.
• Solitary Fibrous Tumor (SFT): SFT is a rare tumor that can occur in the meninges, and it may be mistaken for HPC due to its similar appearance on imaging studies.
• Hemangioma: Hemangiomas are benign vascular tumors that can occur in the meninges. They may be difficult to distinguish from HPCs, especially if they are located in a similar region of the brain.
• Paraganglioma: Paragangliomas are rare neuroendocrine tumors that can occur in the meninges. They may present with similar symptoms and imaging characteristics as HPCs.

Imaging Characteristics:

• CT and MRI scans: Both CT and MRI scans may show a well-circumscribed mass in the meninges, which can be difficult to distinguish from other conditions such as meningioma or SFT.
• Vascular appearance: HPCs often have a characteristic vascular appearance on imaging studies, with multiple small vessels visible within the tumor.

Clinical Importance:

• Differential diagnosis is crucial: Accurate differential diagnosis is essential for determining the appropriate treatment plan for patients with meninges HPC.
• Surgical resection: Surgical resection is often the recommended treatment for HPCs, and it requires a thorough understanding of the differential diagnoses to ensure accurate preoperative planning.

References:

• [1] Carneiro et al first reported cases arising in the meninges and provided histological and immunohistochemical criteria to distinguish them from other conditions.
• [8] Primary meningeal hemangiopericytomas (m-HPC) are rare, aggressive dural based tumours, often difficult to distinguish from meningiomas clinico-radiologically.
• [9] The difference between HPC and meningioma in CT scan or MRI may be helpful in the differential diagnosis of them, but even though, these tumors still mimic each other.