What is Telangiectatic Osteosarcoma?
Osteosarcoma is the most common type of bone cancer that doesn’t originate from blood cells. There are many different forms of osteosarcoma that doctors can identify when they look at the cells under a microscope. These might include conventional, small cell, periosteal, parosteal, high-grade surface, low-grade central, giant cell, and telangiectatic types.
One specific type, telangiectatic osteosarcoma, is a rare and aggressive form of the disease, making up 2 to 12% of all osteosarcoma cases. This particular type has unique features that can be seen on x-rays and other imaging tests, as well as things that can be seen when the tumor is examined closely. However, these features also make it tricky to diagnose because it can look like other bone conditions such as bone cysts and giant cell tumors.
This specific form of osteosarcoma was first described by a doctor named Paget in 1853. However, it wasn’t until later that another doctor, Ewing, identified it as a type of osteosarcoma. Even though it’s been known for more than 150 years, doctors are still working to agree on the best way to treat these tumors.
What Causes Telangiectatic Osteosarcoma?
The cause of a rare bone cancer known as telangiectatic osteosarcoma isn’t clear, nor are the factors that might make someone more likely to get it. Since this cancer is so rare, it’s tough to do studies and investigate what might lead to it. There have been a few reports, however, that suggest genetics might play a role because the cancer has appeared in family members.
For example, one report mentioned an 11-year-old girl and an 8-year-old boy, who were cousins, both diagnosed with telangiectatic osteosarcoma. The girl had this type of cancer in her shinbone and the boy in his spine. When doctors examined tissue samples from these cancers under a microscope, they saw signs that confirmed the diagnosis of telangiectatic osteosarcoma, such as widened and multiplied blood vessels, irregular cancer cells, and excess bone-like tissue. Both children did well after treatment.
There were also three confirmed cases of telangiectatic osteosarcoma found in soft tissues, not bone. Interestingly, one of these cases was the mother whose daughter had already died from a highly malignant, or aggressive, type of bone cancer.
Risk Factors and Frequency for Telangiectatic Osteosarcoma
Telangiectatic osteosarcoma is a condition often seen in children, teenagers, and young adults. The average age of people when they find out they have this disease is 17.5 years old, but it can range from 15 to 20 years old. It also tends to affect males twice as often as females.
- The most common place to find this condition is the lower part of the thigh bone (42% of cases).
- Other locations include the top of the shin bone (17%), the top of the arm bone (9%), and the top of the femur (8%).
- It’s not often seen in flat bones, only about 8% of the time.
There are uncommon areas where it may appear, like the shoulder blade, ribs, breastbone, hip bone, the skull, and the jawbone. Telangiectatic osteosarcoma of the spine is quite rare, making up 2% of all primary vertebral osteosarcomas. It can also very rarely appear in soft tissues of the thigh, forearm, and popliteal fossa, which is the area behind the knee.
Signs and Symptoms of Telangiectatic Osteosarcoma
Telangiectatic osteosarcoma has similar symptoms as conventional osteosarcoma. The most common signs include local pain or a soft tissue mass. Some patients might experience a fracture due to disease progression. In some cases, you can hear a vascular bruit (abnormal sound made by blood flowing through an artery) over the tumor. Just like any other cancer, it’s crucial to undergo a thorough examination to check for any evidence of the disease spreading to other parts of the body.
Testing for Telangiectatic Osteosarcoma
When testing for a rare type of bone cancer called telangiectatic osteosarcoma, various imaging methods can be used. These include plain X-rays, CT (computed tomography) scans, and MRI (magnetic resonance imaging) scans.
Telangiectatic osteosarcoma often shows up on these tests as a type of lesion that causes the bone structure to appear disrupted or expanded. Despite its aggressive nature, this cancer can sometimes mimic the appearance of a less dangerous condition known as an aneurysmal bone cyst, tricking doctors with its clear borders and cyst-like appearance. Certain features, such as changes in the surrounding soft tissue or the existence of a soft tissue mass, usually give the cancer away.
MRI scans are particularly helpful in the diagnosis of telangiectic osteosarcoma. These scans can reveal detailed features such as the multi-chambered nature of the tumor (known as the ‘fluid level sign’). On these MRI scans, the disease can look similar to an aneurysmal cyst, but key differences like irregular walls and solid parts within the tumor can help distinguish it as a cancer.
Doctors use a few different criteria to diagnose telangiectatic osteosarcoma. The following things are signs of the cancer:
– A tumor that appears on imaging tests as a destructive, bone-dissolving growth with little or no new bone formation around it.
– Visible ‘cysts’ or hollow spaces in the tumor which are not densely filled with bone tissue.
– A pattern of tissue death and blood-filled spaces viewed under a microscope.
Although bone cancers often lead to high levels of an enzyme called alkaline phosphatase in the blood, telangiectatic osteosarcoma does not cause this. But other tests – looking at platelet count, white cell count, and levels of enzymes called lactate dehydrogenase and alkaline phosphatase – might help distinguish telangiectatic osteosarcoma from aneurysmal bone cysts.
Treatment Options for Telangiectatic Osteosarcoma
Telangiectatic osteosarcoma is a rare type of bone cancer that in the past had a poor survival rate. However, thanks to the introduction of chemotherapy treatments, survival rates have greatly improved to around 60% after ten years – similar to the more common type of bone cancer, known as conventional osteosarcoma.
Patients with this form of cancer usually undergo a type of chemotherapy known as neo-adjuvant chemotherapy, which is given before surgery to shrink the tumor. After this, the tumor is surgically removed. In some cases, additional chemotherapy or radiation therapy may be given after the surgery to kill any remaining cancer cells.
Before the introduction of chemotherapy, most patients with this type of cancer required an amputation. Thankfully, advances in medicine have made it possible for most patients to keep their limbs, even when the cancer causes a break or crack in the bone, known as a pathological fracture.
The extent of the surgery will depend on various factors, such as the size and location of the cancer and how well it reacts to the initial chemotherapy treatment. If the cancer has spread to other parts of the body, additional chemotherapy may be beneficial.
In a study, eight patients with telangiectatic osteosarcoma, who also had a pathological fracture, were found to have the same survival and recurrence rates as those without fractures. Similarly, in a review of 87 cases, 60% of patients survived ten years after treatment with chemotherapy and surgery. These studies suggest that the most important factor for survival is how well the cancer responds to chemotherapy.
What else can Telangiectatic Osteosarcoma be?
One condition that can be mistaken for telangiectatic osteosarcoma is an aneurysmal bone cyst. This is because both conditions show similar characteristics when examined through X-rays, physical examination, or under a microscope. This similarity can delay the correct diagnosis and the start of the best possible treatment. Other conditions that can be mistaken for telangiectatic osteosarcoma include:
- Giant cell tumor of the bone
- Brodie abscess
- Blood clot
- Malignant fibrous histiocytoma
- Fibrosarcoma
What to expect with Telangiectatic Osteosarcoma
Telangiectatic osteosarcoma, a type of bone cancer, generally responds better to chemotherapy compared to another type called conventional osteosarcoma. With current chemotherapy treatments, about 65% of patients survive after five years. The way in which telangiectatic osteosarcoma spreads in the body is similar to conventional osteosarcoma.
When more than 95% of the tumor has been destroyed (“tumor necrosis”), it’s a strong sign that the treatment is working. Clear neurovascular bundles (networks of nerves and blood vessels) and safe margins of soft tissue and bone removed during surgery are also important for a positive outcome.
Research indicates that levels of lactate dehydrogenase (an enzyme released when there is tissue damage) and the size of the tumor could be used to predict the patient’s prognosis. The study also indicated that the presence of a fracture in the diseased bone does not typically impact the recovery outcomes.
Possible Complications When Diagnosed with Telangiectatic Osteosarcoma
Dealing with telangiectatic osteosarcoma, a type of bone cancer, can come with several complications. These might include the cancer returning in the same area, the disease spreading to other parts of the body, reduced survival rates, failure of implanted medical devices, infections, and heart damage from chemotherapy treatment.
Potential complications:
- Cancer recurrence in the same place
- Cancer spreading to other parts of the body (metastases)
- Reduced survival rates
- Implanted medical device failure
- Infections
- Heart damage due to chemotherapy
Preventing Telangiectatic Osteosarcoma
Telangiectatic osteosarcoma is a rare and powerful type of bone cancer known as osteosarcoma. Even though earlier studies painted a gloomy picture, current understanding suggests that its chances of getting better are similar to those of the more common type of osteosarcoma. These tumors respond well to chemotherapy, a type of cancer treatment. However, these types of tumors have a higher chance of causing a condition called pathologic fracture. This is because they cause the bone to form into a cyst-like shape, which makes the bone weaker. A pathologic fracture is basically a broken bone caused by a disease.[4]
