Overview

Angiosarcoma of the bone is an extremely rare and aggressive form of cancer that develops in the cells lining blood vessels within bones. This malignant vascular tumor represents less than 1% of all primary bone cancers, making it one of the rarest bone cancers diagnosed today.

This rare cancer primarily affects older adults, with men being twice as likely to develop it compared to women. The disease typically targets the long bones of the arms and legs, including the thighbone (femur), shinbone (tibia), and upper arm bone (humerus), as well as the pelvis and spine.

What makes angiosarcoma of the bone particularly challenging is its aggressive nature and high tendency to spread (metastasize) to other parts of the body. The cancer grows rapidly and often has a poor prognosis, especially when diagnosed after it has already spread to other organs.

Symptoms

In its early stages, angiosarcoma of the bone may not cause any noticeable symptoms, making early detection difficult. When symptoms do appear, they vary depending on the tumor’s size and location within the bone.

The most common symptoms include:

• Bone pain – This may start as a dull ache but can become sharp and stabbing
• Swelling or a lump – A noticeable mass or swelling in the affected area
• Fatigue and general weakness
• Unexplained tiredness that doesn’t improve with rest

In rare cases (about 10% of patients), the tumor can weaken the bone structure, leading to pathological fractures. These are breaks that occur with minimal trauma or stress. If the angiosarcoma develops near the spine, it may press on the spinal cord or nerves, causing:

• Numbness in the arms or legs
• Weakness in the limbs
• Pins-and-needles sensations
• Loss of coordination

It’s important to note that these symptoms can be similar to other, less serious conditions. However, persistent bone pain that worsens over time should always be evaluated by a healthcare professional.

Causes

The exact cause of angiosarcoma of the bone remains largely unknown to medical researchers. Scientists classify most cases as idiopathic, meaning they occur without an identifiable cause.

What researchers do understand is that cancer develops when endothelial cells (cells that line blood and lymph vessels) undergo genetic changes or mutations. These DNA changes cause the cells to:

• Multiply uncontrollably
• Live longer than normal healthy cells
• Accumulate and form tumors
• Invade surrounding healthy tissues
• Potentially spread to distant parts of the body

While most cases are considered primary (occurring without a known cause), some angiosarcomas are classified as secondary, meaning they develop as a result of specific risk factors or previous medical treatments.

Risk Factors

Although the cause of most angiosarcomas of the bone is unknown, several factors may increase a person’s risk of developing this rare cancer:

Previous Radiation Therapy: A history of radiation therapy for other cancers or medical conditions is a well-established risk factor. The cancer can develop years or even decades after radiation exposure, particularly in areas previously treated for breast cancer or Hodgkin lymphoma.

Chronic Lymphedema: Lymphedema is a condition involving chronic swelling caused by a buildup of lymph fluid. This can occur after lymph nodes are removed during cancer surgery or due to infections and other medical conditions.

Chemical Exposure: Exposure to certain industrial chemicals has been linked to increased angiosarcoma risk, including:

• Vinyl chloride
• Thorium dioxide
• Arsenic
• Radium

Genetic Syndromes: Certain inherited genetic conditions may predispose individuals to angiosarcoma, including:

• Neurofibromatosis
• Maffucci syndrome
• Klippel-Trenaunay syndrome
• Mutations in BRCA1, BRCA2, and POT1 genes

Bone-Specific Conditions: For angiosarcoma of the bone specifically, pre-existing bone conditions may increase risk:

• Bone infarction (death of bone tissue due to poor blood supply)
• Paget’s disease of the bone
• Chronic osteomyelitis (persistent bone infection)

Diagnosis

Diagnosing angiosarcoma of the bone is challenging due to its rarity and the non-specific nature of its symptoms. A comprehensive evaluation by a multidisciplinary team of specialists is essential for accurate identification.

The diagnostic process typically involves several steps:

Physical Examination: The doctor will examine the affected area, looking for swelling, lumps, or other abnormalities. They will also review the patient’s medical history and symptoms.

Imaging Tests: Various imaging studies help determine the tumor’s size, location, and whether it has spread:

• X-rays – Initial imaging that may show bone destruction and undefined margins
• MRI – (Magnetic Resonance Imaging) – Provides detailed images of soft tissues and bone
• CT scan – (Computed Tomography) – Used for whole-body staging to detect metastasis
• PET scan – (Positron Emission Tomography) – Highlights areas of high metabolic activity

Biopsy: The definitive diagnosis requires a biopsy, where a small tissue sample is taken from the suspicious area. This can be done through:

• Needle biopsy (guided by imaging)
• Open surgical biopsy (less common)

Laboratory Analysis: The biopsy sample undergoes detailed examination, including:

• Histological analysis under a microscope
• Immunohistochemical testing using specific markers to identify cancer cells

Tests to differentiate angiosarcoma from other similar conditions

Types

Angiosarcoma of the bone can present in different ways:

Epithelioid Angiosarcoma: This variant is characterized by cancer cells that look like epithelial cells under a microscope rather than typical blood vessel cells. These tumors are particularly aggressive and are always considered high-grade.

Presentation Patterns: The cancer can appear as:

• A single lesion in one bone
• Multiple lesions within the same bone (most common)
• Multiple lesions in different bones

Stages

While there isn’t a formal staging system specifically for angiosarcoma of the bone, doctors typically classify the disease based on its extent:

Localized Disease: The tumor is confined to its original location without evidence of spread to other parts of the body.

Metastatic Disease: The cancer has spread from its primary site to other distant locations, such as:

• Lungs
• Other bones
• Liver
• Lymph nodes
• Brain (less common)

The presence of metastatic disease at diagnosis significantly affects the prognosis and treatment options.

Treatment

Due to its aggressive nature, angiosarcoma of the bone requires prompt and intensive treatment. A multidisciplinary team including surgical oncologists, radiation oncologists, and medical oncologists works together to develop a personalized treatment plan.

Surgery: Surgery is often the cornerstone of treatment, with the goal of complete tumor removal. Key aspects include:

• Achieving wide surgical margins (removing tumor plus surrounding healthy tissue)
• Limb-sparing surgery when possible to preserve function
• Amputation may be necessary in severe cases
• Surgical complete remission is strongly associated with better outcomes

Radiation Therapy: Radiation therapy uses high-energy beams to destroy cancer cells and may be used:

• After surgery to eliminate remaining cancer cells
• Before surgery to shrink tumors
• As primary treatment when surgery isn’t possible
• For pain relief in advanced cases

Chemotherapy: Chemotherapy involves powerful drugs that target cancer cells throughout the body. Common medications include:

• Taxanes (such as paclitaxel)
• Doxorubicin
• Ifosfamide
• Gemcitabine

Targeted Drug Therapy: These treatments focus on specific molecular pathways within cancer cells:

• VEGF pathway inhibitors (sorafenib, pazopanib)
• Beta-adrenergic receptor blockers (propranolol)

Immunotherapy: This newer approach harnesses the body’s immune system to fight cancer, particularly useful for advanced cases.

Rehabilitation

Following intensive treatment for angiosarcoma of the bone, rehabilitation plays a crucial role in helping patients regain strength, mobility, and independence.

A comprehensive rehabilitation program may include:

• Physiotherapists – Help restore strength, movement, and physical function
• Occupational therapists – Assist with daily activities and adaptation to limitations
• Dietitians – Provide nutritional support during recovery
• Prosthetists – Design artificial limbs for patients who underwent amputation
• Orthotists – Provide supportive devices like splints or specialized footwear

The goal is to help patients return to their daily activities and maintain the highest possible quality of life.

Complications

Angiosarcoma of the bone is associated with several potential complications:

High Recurrence and Metastasis Rates: Despite aggressive treatment, there is a significant risk of:

• Local recurrence (tumor returning in the same area)
• Distant metastasis to lungs, liver, other bones, or lymph nodes

Poor Prognosis: The outlook is generally challenging, especially for patients with metastatic disease at diagnosis. Many patients with aggressive forms face significant challenges within the first year after diagnosis.

Treatment-Related Side Effects

• Radiation therapy can cause skin problems, bone damage, and organ complications

Chemotherapy may cause significant systemic side effects, particularly challenging for older patients

Prevention

Prevent frozen shoulder by maintaining shoulder movement after injury or surgery, regular stretching, and early medical consultation for shoulder pain.

Living With Frozen Shoulder

Living with angiosarcoma of the bone presents significant challenges that require comprehensive, ongoing care.

Follow-up Care: Regular monitoring is essential and includes:

• Periodic imaging tests (MRI, CT scan)
• Whole-body scans to check for recurrence
• Cardiac function monitoring for patients who received certain chemotherapy drugs
• Long-term surveillance for treatment-related complications

Symptom Management: Patients work closely with their healthcare team to manage:

• Ongoing pain and discomfort
• Fatigue and weakness
• Side effects from treatments
• Emotional and psychological challenges

Support Systems: Access to support services is crucial:

• Psychological counseling
• Support groups for patients and families
• Social work services
• Palliative care when appropriate

Quality of Life: Maintaining the best possible quality of life involves:

• Staying active within physical limitations
• Pursuing meaningful activities and relationships
• Working with rehabilitation specialists
• Open communication with the healthcare team

Key Takeaways

• Angiosarcoma of the bone is an extremely rare and aggressive cancer affecting blood vessel cells within bones
• Early symptoms may be minimal, making early detection challenging
• The disease primarily affects older adults, with men at higher risk than women
• Risk factors include previous radiation therapy, chronic lymphedema, and certain genetic conditions
• Diagnosis requires a combination of imaging tests and biopsy confirmation
• Treatment typically involves surgery, radiation therapy, and chemotherapy in a multidisciplinary approach
• Rehabilitation services are essential for recovery and maintaining quality of life
• The prognosis remains challenging, particularly for metastatic disease
• Ongoing follow-up care and comprehensive support are crucial for patients and families

At Sancheti Hospital, we understand the complexity and challenges associated with rare bone cancers like angiosarcoma of the bone. Our multidisciplinary team of orthopedic oncologists, medical oncologists, radiation oncologists, and rehabilitation specialists work collaboratively to provide comprehensive care for patients facing this challenging diagnosis. 

We offer state-of-the-art diagnostic facilities, advanced treatment options, and comprehensive rehabilitation services to help patients achieve the best possible outcomes. Our team is committed to providing personalized care that addresses not only the medical aspects of the disease but also the emotional and physical challenges that patients and families face. 

Through our integrated approach to cancer care, we strive to support patients throughout their treatment journey and help them maintain the highest quality of life possible.