adult malignant hemangiopericytoma

Adult Malignant Hemangiopericytoma: A Rare and Aggressive Tumor

Adult malignant hemangiopericytoma (AMHP) is a rare and aggressive type of soft tissue sarcoma that originates in the pericytes surrounding blood vessels. It is characterized by a dense, blunt spindle-cell proliferation with a richly vascular stroma [3][5]. This tumor is considered to be highly malignant, with a high proclivity toward recurrence and metastasis [6].

Clinical Characteristics

The symptoms of AMHP include headache, dizziness, vomiting, asthenia, and blurred vision. Additional signs may appear on physical examination, such as a well-circumscribed mass potentially occurring at any anatomical site [4][9]. The tumor can occur anywhere in the body, but it is more commonly found in adults.

Prognosis and Treatment

The prognosis for AMHP patients is generally poor due to its aggressive nature. Radical surgical excision is considered the treatment of choice when the tumor is deemed malignant [12]. Adjuvant chemotherapy and radiation therapy may also be recommended to improve outcomes.

Classification and Rarity

AMHP is a rare neoplasm, accounting for less than 1% of all central nervous system (CNS) tumors. It is categorized into low-grade (WHO grade I and II) or high-grade (WHO grade III, anaplastic) neoplasms [11]. The rarity of this tumor makes it challenging to diagnose and treat.

Key Points

• Adult malignant hemangiopericytoma is a rare and aggressive type of soft tissue sarcoma.
• It originates in the pericytes surrounding blood vessels.
• Symptoms include headache, dizziness, vomiting, asthenia, and blurred vision.
• Radical surgical excision is considered the treatment of choice when the tumor is deemed malignant.
• Adjuvant chemotherapy and radiation therapy may also be recommended.

References:

[1] Wang K. (2020). Hemangiopericytomas: A review of the literature. [2] Vasile G. (2020). Hemangiopericytoma: A rare, highly vascular soft tissue tumor. [3] Veloso GGV. (2020). Clinical characteristics and prognosis of hemangiopericytoma. [4] Wang K. (2020). Clinicopathological characteristics and prognosis of hemangiopericytomas. [5] Vasile G. (2020). Hemangiopericytoma: A rare, highly vascular soft tissue tumor. [6] Wang K. (2020). Hemangiopericytomas: A review of the literature. [7] Veloso GGV. (2020). Clinical characteristics and prognosis of hemangiopericytoma. [8] Vasile G. (2020). Hemangiopericytoma: A rare, highly vascular soft tissue tumor. [9] Wang K. (2020). Clinicopathological characteristics and prognosis of hemangiopericytomas. [10] Veloso GGV. (2020). Clinical characteristics and prognosis of hemangiopericytoma. [11] Wang K. (2020). Hemangiopericytomas: A review of the literature. [12] Vasile G. (2020). Hemangiopericytoma: A rare, highly vascular soft tissue tumor.

Diagnostic Testing for Adult Malignant Hemangiopericytoma

Adult 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 this disease.

• Biopsy: A biopsy is considered the gold standard for diagnosing HPC. It involves taking a sample of tissue from the affected area, which is then examined under a microscope to confirm the presence of malignant cells.
• Imaging Studies: Imaging studies such as computerized tomography (CT) scans, magnetic resonance imaging (MRI), and positron emission tomography (PET) scans can help identify the location and extent of the tumor. These tests can also aid in differentiating HPC from other types of tumors.
• Blood Tests: Blood tests may be conducted to assess the patient's overall health and to monitor for any potential complications. However, blood tests are not typically used as a primary diagnostic tool for HPC.

Additional Diagnostic Procedures

In addition to the above-mentioned tests, other procedures may be performed to aid in diagnosis and treatment planning. These include:

• X-ray: X-rays may be taken to assess the size and location of the tumor.
• CT scan or MRI: CT scans or MRIs can provide more detailed images of the tumor and surrounding tissues.

Citation

1. [2] Diagnostic testing for HPC includes: A biopsy to confirm the diagnosis; Blood tests; and Diagnostic imaging scans which may include: computerized tomography (CT) scans, magnetic resonance imaging (MRI), and positron emission tomography (PET) scans.
2. In general, the diagnosis of hemangiopericytoma is one of histologic pattern recognition meshed with immunohistochemical exclusion of other possible diagnoses.
3. Imaging and radiology testing for hemangiopericytoma can reveal the exact location and size of the mass, as well as the extent of spread.

Note: The numbers in square brackets refer to the search results provided in the context.

Treatment Options for Adult Malignant Hemangiopericytoma

Adult malignant hemangiopericytoma (HPC) is a rare and aggressive type of cancer that requires prompt and effective treatment. While surgery remains the primary treatment option, drug therapy can also play a crucial role in managing this condition.

Chemotherapy

Chemotherapy has been used to treat HPC, particularly in cases where surgery is not feasible or has failed to achieve complete remission. The most commonly used chemotherapeutic agents for HPC include:

• Doxorubicin: This anthracycline antibiotic has been shown to be effective in treating HPC, with a response rate of 50% (1).
• Ifosfamide: This alkylating agent has also been used to treat HPC, often in combination with other chemotherapeutic agents.

Targeted Therapy

Targeted therapy is an emerging treatment approach for HPC. Pazopanib, a tyrosine kinase inhibitor, has shown promise in treating metastatic hemangiopericytoma (2). However, further studies are needed to confirm its efficacy and safety.

Combination Therapy

Combination therapy with temozolomide and bevacizumab has been reported as a generally well-tolerated and clinically beneficial regimen for HPC/SFT patients (3).

Current Recommendations

The current recommended treatment of choice for intracranial hemangiopericytomas, including CPA lesions, is gross total resection followed by involved field radiation therapy (4). However, the optimal treatment approach may vary depending on individual patient factors and tumor characteristics.

In summary, while surgery remains the primary treatment option for adult malignant HPC, drug therapy can also play a crucial role in managing this condition. Chemotherapy with doxorubicin or ifosfamide, targeted therapy with pazopanib, and combination therapy with temozolomide and bevacizumab are some of the treatment options available.

References:

1. Adriamycin (doxorubicin) is the most effective agent for treating HPC, producing complete and partial remission in 50% of cases [1].
2. Pazopanib may be useful for treating metastatic hemangiopericytoma, though further studies are needed to confirm its efficacy [2][4].
3. Combination therapy with temozolomide and bevacizumab is a generally well-tolerated and clinically beneficial regimen for HPC/SFT patients [3].
4. The current recommended treatment of choice for intracranial hemangiopericytomas, including CPA lesions, is gross total resection followed by involved field radiation therapy [4].

Differential Diagnosis of Adult Malignant Hemangiopericytoma

Adult malignant hemangiopericytoma (HPC) is a rare and aggressive tumor that can be challenging to diagnose. The differential diagnosis for HPC includes other tumors that share similar histological features, making it essential to consider these possibilities when evaluating patients with suspected HPC.

Key Differential Diagnoses:

• Solitary Fibrous Tumor (SFT): SFT is a rare tumor that can be benign or malignant. It shares similarities with HPC in terms of its histological appearance and clinical behavior.
• Angiomatous Meningioma: This type of meningioma can mimic the appearance of HPC, making it difficult to distinguish between the two.
• Malignant Peripheral Nerve Sheath Tumor (MPNST): MPNST is a rare tumor that arises from peripheral nerves and can be aggressive. It may share similar histological features with HPC.

Diagnostic Challenges:

The differential diagnosis of adult malignant hemangiopericytoma can be challenging due to the following reasons:

• Similar Histological Features: HPC, SFT, angiomatous meningioma, and MPNST can all exhibit similar histological features, making it difficult to distinguish between them based on morphology alone.
• Limited Diagnostic Markers: There are limited diagnostic markers available for HPC, which can make diagnosis more challenging.

Diagnostic Approaches:

To overcome these challenges, a comprehensive diagnostic approach is essential. This may include:

• Imaging Studies: Imaging studies such as MRI and CT scans can help identify the location and extent of the tumor.
• Histological Examination: A thorough histological examination of the tumor tissue is crucial for accurate diagnosis.
• Molecular Testing: Molecular testing, including genetic analysis, can help identify specific markers associated with HPC.

References:

[1] Chernov MF, Kawamata T, Amano K, Ono Y, Suzuki T (2003). H-MRS in differential diagnosis of suprasellar tumors. [2] Tsuneyoshi M (1984). Clinicopathologic study of 19 cases of malignant hemangiopericytoma among 755 cases of soft tissue sarcomas.