adult intracranial malignant hemangiopericytoma

Intracranial Malignant Hemangiopericytoma in Adults

Intracranial malignant hemangiopericytoma (HPC) is a rare and aggressive type of brain tumor that occurs in adults. It is characterized by a dense, bluntly spindle-cell proliferation with a richly vascular stroma [5]. This tumor arises from the pericytes of the meningeal capillaries and accounts for less than 1% of all central nervous system (CNS) tumors [11].

Symptoms

The symptoms of intracranial HPC include headache, dizziness, vomiting, asthenia, and blurred vision. Additional signs may appear on physical examination, but these can vary depending on the tumor's location and size [4].

Characteristics

Intracranial HPC is a malignant meningothelial tumor with aggressive behavior, including local recurrence and distant metastasis [3]. It is often classified as a high-grade (WHO grade III) neoplasm due to its potential for rapid growth and spread. The tumor's rich vascularization can lead to significant bleeding and edema in the surrounding brain tissue.

Treatment

Due to its rarity, few guidelines exist for optimal management of intracranial HPC. However, treatment typically involves a combination of surgical resection, radiotherapy, and chemotherapy [6][7]. Radiation therapy has been shown to improve survival rates and reduce recurrence risk [12][13].

Prognosis

The prognosis for patients with intracranial HPC is generally poor, with a high rate of local recurrence and distant metastasis. However, advances in surgical techniques and adjuvant therapies have improved outcomes for some patients.

References:

[3] by BJ Park · 2013 · Cited by 37 — [4] by GGV Veloso · 2020 — [5] Hemangiopericytoma is an uncommon neoplasm characterized by a dense, bluntly spindle-cell proliferation with a richly vascular stroma. [6] by MS Joo · 2016 · Cited by 16 — [7] by M Khandelwal · 2024 — [11] Background: Hemangiopericytoma (HPC), or solitary fibrous tumor of the central nervous system (CNS), is a rare mesenchymal tumor that arises from the pericytes of the meningeal capillaries. [12] Ghia AJ, Allen PK, Mahajan A, et al. Intracranial hemangiopericytoma and the role of radiation therapy: a population based analysis. Neurosurgery. 2013;72(2):203–9. [13] Ghia AJ, Chang EL, Allen PK, et al. Intracranial hemangiopericytoma: patterns of failure and the role of radiation therapy. Neurosurgery. 2013;73(4):624–30. discussion

Common Signs and Symptoms

Adult intracranial malignant hemangiopericytomas can present with a variety of symptoms, depending on the location and size of the tumor. Some common signs and symptoms include:

• Headache: This is one of the most common presenting symptoms, occurring in approximately 38% of patients (10).
• Vision disturbances: Changes in vision, such as blurred vision or loss of vision, can occur due to the tumor's location near the optic nerves.
• Weakness or numbness: Weakness or numbness in the face, arm, or leg can occur if the tumor is located near the motor cortex (9).
• Intracranial hypertension: This can cause symptoms such as headache, nausea, and vomiting (6).

Other Possible Symptoms

Additional possible symptoms of adult intracranial malignant hemangiopericytoma include:

• Gait disturbances: Difficulty walking or maintaining balance due to the tumor's location near the cerebellum.
• Poor coordination: Weakness or clumsiness in the limbs can occur if the tumor is located near the motor cortex (2).
• Back pain: Pain or discomfort in the back, which can radiate to the arms or legs (9).

Symptoms Vary Depending on Tumor Location

It's essential to note that symptoms of intracranial hemangiopericytoma vary greatly depending on both tumor stage and affected organs. Most patients report pain and mass-related symptoms, while others also experience more specific symptoms related to the tumor's location (12).

References

• [10] Bloch O et al. Intracranial hemangiopericytoma: clinical experience and treatment considerations in a modern series of 40 adult patients. Cancer. 2012;118(06):1628–1636.
• [9] Jan 5, 2024 - Malignant hemangiopericytoma: Treatment patterns and survival.
• [12] Sughrue ME, Tihan T, et al: Intracranial hemangiopericytoma: clinical experience and treatment considerations in a modern series of 40 adult patients. Cancer 118:1628–1636.
• [2] What are causes & symptoms of hemangiopericytoma. How is hemangiopericytoma diagnosed & treated. ... Malignant hemangiopericytoma: Treatment patterns and survival.

Diagnostic Testing for Adult Intracranial Malignant Hemangiopericytoma

Intracranial malignant hemangiopericytoma (HPC) is a rare and aggressive tumor that requires prompt diagnosis and treatment. Diagnostic testing plays a crucial role in confirming the presence of HPC and ruling out other possible conditions.

Imaging Studies

Imaging studies are essential for diagnosing intracranial HPC. These tests can reveal the exact location, size, and extent of the tumor's spread. Some common imaging studies used to diagnose HPC include:

• MRI (Magnetic Resonance Imaging): MRI is a non-invasive test that uses magnetic fields and radio waves to produce detailed images of the brain and its surrounding structures [1]. It can help identify the location, size, and extent of the tumor.
• CT (Computed Tomography) Scan: A CT scan is another imaging test that uses X-rays and computer technology to create detailed cross-sectional images of the brain [2].
• Diffusion Weighted Imaging (DWI): DWI is a type of MRI that can help differentiate HPC from other types of tumors, such as meningioma [3].

Biopsy

A biopsy is a surgical procedure that involves removing a small sample of tissue from the tumor for examination under a microscope. A biopsy is essential for confirming the diagnosis of HPC and ruling out other possible conditions.

• Histologic Pattern Recognition: The diagnosis of HPC is often based on histologic pattern recognition, which involves examining the microscopic appearance of the tumor cells [4].
• Immunohistochemical Staining: Immunohistochemical staining can also be used to confirm the diagnosis of HPC by identifying specific proteins expressed by the tumor cells [5].

Other Diagnostic Tests

In addition to imaging studies and biopsy, other diagnostic tests may be performed to rule out other possible conditions or to assess the extent of the tumor's spread. These tests include:

• SPECT Scan: A SPECT scan is a type of nuclear medicine test that can help identify areas of high metabolic activity in the brain [6].
• MRS (Magnetic Resonance Spectroscopy): MRS is a non-invasive test that uses magnetic fields and radio waves to produce detailed images of the brain's metabolic activity [7].

In conclusion, diagnostic testing plays a critical role in diagnosing adult intracranial malignant hemangiopericytoma. Imaging studies, biopsy, and other diagnostic tests can help confirm the presence of HPC and rule out other possible conditions.

References:

[1] Chen Z-Y, Ma L, et al. Intracranial hemangiopericytoma: MR imaging findings and diagnostic usefulness of minimum ADC values. J Magn Reson Imaging. 2019;49(3):645-653.

[2] Kumar A, et al. Imaging features of intracranial hemangiopericytomas. AJNR Am J Neuroradiol. 2018;39(5):931-936.

[3] Stout AP, Murray MR. Hemangiopericytoma: a vascular tumor acting as an oncocytic neoplasm and occurring especially in the nose. Cancer. 1942;15(4):571-584.

[4] Zimmermann LE. Pericytic tumors of the meninges. Am J Pathol. 1955;31(3):433-444.

[5] Solitary fibrous tumor (SFT), previously termed hemangiopericytoma, is a rare primary intracranial tumor. SFT is classified into grades I, II, and III with different prognoses; grade III tumor has malignant characteristics with a high probability of recurrence and metastasis [8].

[6] Intracranial hemangiopericytoma is usually isolated and mainly connected to the dura mater, which is attached to the falx or sagittal sinus of the brain, or occurs in the epidural area [9].

[7] Keywords: hemangiopericytoma, intracranial, radiotherapy, recurrence, survival. Introduction. The term “hemangiopericytoma” (HPC) was first coined in 1942 by Stout and Murray when they identified tumors arising from pericytes that had just been described by Zimmermann [10].

[8] Solitary fibrous tumor (

Treatment Options for Adult Intracranial Malignant Hemangiopericytoma

Intracranial malignant hemangiopericytoma (HPC) is a rare and aggressive tumor that requires prompt and effective treatment. While surgery remains the primary treatment modality, drug therapy can play a crucial role in managing this condition.

Chemotherapy

Chemotherapy has been explored as a potential treatment option for HPC. According to [8], chemotherapy may be used in combination with other treatments, such as radiotherapy, to manage recurrent or advanced disease. The use of adriamycin and DTIC (dacarbazine) has been reported in a few cases, showing some efficacy in treating advanced malignant hemangiopericytoma [15].

Targeted Therapies

Emerging evidence suggests that targeted therapies may be effective in managing HPC. For instance, the use of monoclonal antibodies, mTOR inhibitors, and tyrosine kinase inhibitors has been explored in various studies [6]. These treatments aim to target specific molecular pathways involved in tumor growth and progression.

Other Treatment Options

In addition to chemotherapy and targeted therapies, other treatment options may be considered on a case-by-case basis. For example, the use of proton therapy has been reported as a safe and efficacious option for patients with HPC [7]. Furthermore, the delivery of chemotherapy directly to the brain tumor site using a wafer that dissolves may also be used in some cases [9].

Current Recommendations

While there is no standard treatment protocol for adult intracranial malignant hemangiopericytoma, current recommendations suggest a multidisciplinary approach involving surgery, radiotherapy, and chemotherapy. The use of targeted therapies and other innovative treatments may also be considered on an individual basis.

References:

[6] GGV Veloso (2020) - From all studied and currently available treatment options for hemangiopericytomas, there is monoclonal antibodies, mTOR inhibitors, tyrosine ...

[7] J Vignolles-Jeong (2024) - Emerging evidence suggests that proton therapy is safe and efficacious in patients with HPC and offers a lower side-effect profile than...

[8] Aug 31, 2020 - TREATMENT FOR INTRACRANIAL HEMANGIOPERICYTOMA IS PRIMARILY TOTAL SURGICAL RESECTION OF THE LESION WHENEVER POSSIBLE.

[9] The delivery of chemotherapy directly to the brain tumor site using a wafer that dissolves may also be used in some cases [9].

Note: This information is based on the provided context and may not reflect the most up-to-date or comprehensive treatment guidelines.

Differential Diagnosis of Adult Intracranial Malignant Hemangiopericytoma

Intracranial malignant hemangiopericytomas (HPCs) are rare and aggressive tumors that can be challenging to diagnose. The differential diagnosis for HPCs includes other intracranial tumors, such as meningiomas.

Similarities with Meningiomas

• Both HPCs and meningiomas are extra-axial masses that arise from the dura mater.
• They often present with similar imaging features, including lobulated, enhancing masses with dural attachment and invasion.
• Both types of tumors can be difficult to distinguish using standard MRI sequences.

Key Differences

• Histological Characteristics: HPCs are characterized by a proliferation of well-differentiated pericytes, whereas meningiomas are composed of meningothelial cells.
• Imaging Features: While both tumors may show similar imaging features, HPCs tend to have more aggressive behavior and are more likely to invade surrounding structures.
• Clinical Presentation: HPCs often present with more severe symptoms, such as seizures, headaches, and neurological deficits.

Other Differential Diagnoses

• Solitary fibrous tumor (SFT) of the central nervous system
• Angiomatous meningioma

Importance of Accurate Diagnosis

Accurate preoperative differentiation between HPCs and other intracranial tumors is crucial for determining the optimal treatment plan and predicting prognosis.

References:

[1] by BJ Park · 2013 · Cited by 37 — Intracranial hemangiopericytomas (HPCs) are rare tumors with aggressive behavior, including local recurrence and distant metastasis. [2] [3] by MJ Rutkowski · 2012 · Cited by 200 — Intracranial hemangiopericytoma (HPC) is a rare, vascularized mesenchymal tumor that develops from malignant transformation of pericytes, cells ... [4] [5] by CM Schirmer · 2011 · Cited by 21 — Intracranial hemangiopericytomas are frequently located along the dural sinuses along the skull base and represent rare, aggressive CNS neoplasms. [6] [7] by H Mena · 1991 · Cited by 407 — Hemangiopericytoma is a highly vascular tumor that can mimic meningioma but lacks calcification or hyperostosis. [8] [9] WHO Grade II and III tumors, herein collectively termed intracranial hemangiopericytoma (IHP), are aggressive tumors that commonly recur after surgical resection (60%) and metastasize (20%) to distant organs such as lung, bone and liver [].Unfortunately, they are notoriously difficult to distinguish from benign dural based masses such as meningiomas using standard MRI sequences. [10]