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Hemangiopericytoma

Disease Details

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Description: Hemangiopericytoma is a rare vascular tumor arising from pericytes, which are contractile cells surrounding capillaries and venules.
Type: Hemangiopericytoma is a type of soft tissue tumor. It does not have a specific pattern of genetic transmission as most cases are sporadic, meaning they occur by chance and are not usually inherited.
Signs And Symptoms: Symptoms of hemangiopericytoma vary greatly depending on both tumor stage and affected organs. Most patients report pain and mass-related symptoms, while others also report vascular disease-related symptoms, and some have no symptoms until late in the disease process. Hemangiopericytomas are most commonly found in the meninges, lower extremities, retroperitoneum, pelvis, lungs, and pleura.
Prognosis: Hemangiopericytoma is a rare vascular tumor that arises from pericytes, cells that surround blood vessels. The prognosis for hemangiopericytoma varies based on several factors, including the size, location of the tumor, and whether it has metastasized. Generally, smaller tumors that are completely resectable have a better prognosis. Tumors that have metastasized or recur after treatment typically have a poorer prognosis. Long-term follow-up is necessary due to the potential for late recurrence.
Onset: Hemangiopericytoma is a rare vascular tumor that can occur at any age but is most commonly diagnosed in adults between their 30s and 50s. The exact time of onset is often difficult to determine because the tumor can grow slowly and may not cause symptoms until it reaches a significant size.
Prevalence: Hemangiopericytomas, now often grouped under the term solitary fibrous tumors (SFTs), are rare, accounting for less than 1% of all vascular tumors. Their exact prevalence is challenging to determine due to their rarity and heterogeneous presentation.
Epidemiology: In one series, the median age of affected individuals was 45 years, with a 10-year survival rate of 70 percent. In another study, age over 45 and female sex were associated with worse survival rates in hemangiopericytomas.
Intractability: Hemangiopericytoma, now often referred to as solitary fibrous tumor, is not universally intractable but can be challenging to treat due to its potential for recurrence and metastasis. Treatment typically involves surgical resection, and the success of the surgery greatly influences the prognosis. In cases where the tumor is not completely resectable or has metastasized, additional therapies such as radiation or chemotherapy may be considered, though these are less consistently effective.
Disease Severity: Hemangiopericytoma is a rare vascular tumor that can vary in severity depending on its location and aggressiveness. These tumors can range from benign to malignant, and malignant forms have the potential to metastasize to other parts of the body. Factors such as the size of the tumor, its growth rate, and invasiveness into surrounding tissues also influence disease severity and prognosis. Early detection and treatment are crucial in managing the disease effectively.
Healthcare Professionals: Disease Ontology ID - DOID:264
Pathophysiology: Hemangiopericytoma is a rare vascular tumor that originates from pericytes, which are contractile cells surrounding the blood capillaries. The pathophysiology involves the abnormal proliferation of these pericytes, leading to the formation of a tumor. The exact cause of this abnormal growth is not well understood, but genetic and molecular factors, such as chromosomal aberrations and mutations, may play a role. Hemangiopericytomas can occur anywhere in the body but are most commonly found in the lower extremities, pelvis, and retroperitoneum. They may exhibit varying degrees of malignancy, with some cases metastasizing to other tissues and organs.
Carrier Status: Hemangiopericytoma is not characterized by a specific carrier status as it is a type of rare vascular tumor that originates from pericytes, the contractile cells surrounding capillaries and venules. Its exact cause is not well understood, and it is not typically inherited or associated with a genetic carrier state.
Mechanism: Hemangiopericytoma is a rare soft tissue tumor that originates from pericytes, the contractile cells that wrap around the endothelial cells of capillaries and venules.

Mechanism:
The exact mechanism underlying the development of hemangiopericytoma is not fully understood. Generally, it is believed to involve abnormal proliferation and migration of pericytes due to genetic and possibly environmental factors.

Molecular Mechanisms:
1. **Genetic Mutations**:
- Genetic alterations, including mutations and chromosomal translocations, have been implicated. One common genetic event is the NAB2-STAT6 gene fusion, which is considered a hallmark in solitary fibrous tumors, a broader category that includes hemangiopericytomas.
2. **Growth Factors and Signaling Pathways**:
- Abnormal activation of growth factor signaling pathways, such as PDGF (platelet-derived growth factor) and VEGF (vascular endothelial growth factor), is thought to play a role.
3. **Angiogenesis**:
- Since hemangiopericytomas are vascular tumors, dysregulated angiogenesis, or new blood vessel formation, is a significant factor. Abnormal signaling in pathways regulating angiogenesis, such as the VEGF pathway, may contribute to tumor growth and sustainability.
4. **Epigenetic Changes**:
- Changes in DNA methylation and histone modification may also play roles in the abnormal regulation of pericyte proliferation and differentiation.

These molecular insights are important not only for understanding the tumor biology but also for developing targeted therapies for patients with hemangiopericytoma.
Treatment: Depending on the grade of the sarcoma, it is treated with surgery, chemotherapy, and/or radiotherapy. Though surgery is the current standard of care for hemangiopericytomas, metastasis and tumor recurrence occur in more than 30% of patients, in particular recurrence in the pelvis and retroperitoneum and metastasis in bone and lungs. Radiotherapy does not appear to provide a significant survival benefit but is recommended for use in patients with tumors greater than 5 cm in diameter or with inadequate resection margins after surgery. Clinical benefits of chemotherapy in soft tissue tumors remains unclear. However, the combination of surgery and chemotherapy appears to worsen survival in hemangiopericytoma patients.More research is needed to determine efficacy of different types of treatment.
Compassionate Use Treatment: Hemangiopericytoma is a rare vascular tumor that can be challenging to treat, especially in advanced stages. Compassionate use, off-label, or experimental treatments for hemangiopericytoma may include:

1. **Pazopanib**: An off-label usage, Pazopanib is a tyrosine kinase inhibitor that has shown efficacy in treating soft tissue sarcomas, including hemangiopericytomas.

2. **Bevacizumab**: Another off-label option, Bevacizumab is a monoclonal antibody targeting vascular endothelial growth factor (VEGF), potentially inhibiting tumor angiogenesis.

3. **Temozolomide**: Though primarily used for gliomas, Temozolomide may be used off-label for its alkylating properties.

4. **Immunotherapy**: Experimental treatments such as Pembrolizumab or Nivolumab, PD-1 inhibitors, are being explored in clinical trials for various cancers, including rare tumors like hemangiopericytoma.

Consulting with a healthcare professional specializing in oncology is essential to explore these options and determine the most appropriate course of action based on the individual patient's condition.
Lifestyle Recommendations: Lifestyle recommendations for individuals with a hemangiopericytoma, a rare vascular tumor, typically focus on overall health and managing symptoms:

1. **Regular Medical Follow-up:** Regular check-ups and imaging scans to monitor the tumor and detect any changes or recurrences early.
2. **Healthy Diet:** Maintain a balanced diet rich in fruits, vegetables, whole grains, and lean proteins to support overall health and recovery.
3. **Physical Activity:** Engage in regular, moderate exercise as advised by a healthcare provider to improve overall fitness and well-being.
4. **Stress Management:** Practice stress-reducing techniques such as meditation, yoga, or counseling to manage anxiety and emotional stress.
5. **Avoid Smoking and Limit Alcohol:** Avoid smoking and limit alcohol consumption as these can negatively impact overall health and recovery.
6. **Symptom Monitoring:** Keep track of any new symptoms or changes in existing symptoms and report them to a healthcare provider promptly.
7. **Follow Treatment Plans:** Adhere to the treatment regimen prescribed by healthcare providers, including medications, radiation, or any other recommended therapies.
8. **Support Networks:** Engage with support groups or counseling services for emotional and psychological support.

Each individual's situation is unique, so these recommendations should be tailored to fit personal health conditions and discussed with a healthcare provider.
Medication: Hemangiopericytoma is a rare type of vascular tumor arising from pericytes. Treatment typically involves surgical resection as the primary intervention. There isn't a specific medication exclusively for hemangiopericytoma, but in some cases, radiation therapy and chemotherapy might be considered, especially in cases where complete surgical removal is not possible. Tyrosine kinase inhibitors, such as sunitinib or pazopanib, might be used in metastatic or unresectable cases. Always consult an oncologist for personalized treatment options.
Repurposable Drugs: There is no widely recognized list of repurposable drugs specifically for hemangiopericytoma, as treatment typically focuses on surgery and, in some cases, radiation therapy or chemotherapy. However, off-label use of certain drugs designed for other cancers might be considered in refractory cases. Consulting a specialized oncologist for updated and personalized treatment options is advisable.
Metabolites: Hemangiopericytoma is a rare vascular tumor originating from pericytes, which are contractile cells surrounding capillaries and venules. Research on specific metabolites associated with hemangiopericytoma is limited, and metabolomic profiling is not routinely used in clinical practice for this condition. Diagnosis and monitoring typically rely on imaging, histopathology, and immunohistochemical analysis rather than metabolic markers.
Nutraceuticals: For hemangiopericytoma, evidence regarding the efficacy of nutraceuticals is currently limited. Hemangiopericytoma is a rare vascular tumor, and its treatment usually involves surgical resection, radiation therapy, and sometimes chemotherapy. Integrative approaches, such as the use of nutraceuticals, have not been well-studied in clinical settings for this specific condition. Always consult a healthcare professional before considering any supplements or alternative treatments in addition to standard medical care.
Peptides: Hemangiopericytoma is a rare vascular tumor that arises from pericytes, the contractile cells surrounding capillaries and venules. Treatment and research into this tumor may involve various methods including peptides and nanoparticles (nan).

Peptides can be used for targeted therapy as they can serve as ligands for receptors overexpressed in tumor cells, aiding in drug delivery or diagnostics. Nanotechnology, involving nanoparticles, can be employed to enhance imaging, deliver therapeutic agents specifically to the tumor site, and improve the efficacy and safety of treatment modalities.

These approaches are part of ongoing research aimed at improving diagnosis, monitoring, and treatment of hemangiopericytoma.