What is Stewart-Treves Syndrome?
Stewart-Treves syndrome was first brought to light by Dr. Fred Stewart and Norman Treves, who recorded a series of 6 patients who developed a rare cancer of the lymph vessels, known as lymphangiosarcoma, following chronic swelling (lymphedema) after breast removal surgery (mastectomy). It’s worth noting that, while most cases have occurred in people who have had mastectomies, this rare type of cancer can also develop in those who have long-term swelling due to other reasons. It’s important to correctly identify Stewart-Treves syndrome because it can lead to the cancer spreading to other parts of the body (metastatic spread) and carries a poor prognosis, meaning that the chances of recovery are low.
What Causes Stewart-Treves Syndrome?
Soft tissue sarcomas are a rare type of cancer, making up less than 1% of all cancers. A specific type of these cancers is angiosarcoma, which are aggressive and malignant tumors growing from blood vessels or lymph vessels. These tumors are particularly dangerous with a poor prognosis. They usually come up as medium to high-grade lesions, meaning they’re quite advanced and serious.
However, one challenge is that angiosarcomas can initially appear as quite harmless, leading to delays in diagnosis. This means that these tumors might pose more harm than they initially seem to, reinforcing the importance of regular check-ups and attentive medical examination.
Risk Factors and Frequency for Stewart-Treves Syndrome
Angiosarcomas, a type of cancer, can occur anywhere in the body. However, around 60% of them appear in the skin or superficial soft tissues. They usually develop under three common conditions:
- Mostly, they appear on the head and neck of elderly people, typically on men’s scalps, without any known cause.
- They can be caused by previous exposure to ionizing radiation.
- They might be associated with chronic lymphedema, a condition of persistent swelling, which can be due to surgical complications or congenital abnormalities.
It’s important to note that 90% of angiosarcoma cases that occur with lymphedema develop after a mastectomy, a surgery to remove all or part of a breast. This is known as Stewart-Treves syndrome.
Signs and Symptoms of Stewart-Treves Syndrome
Cutaneous angiosarcoma is a type of skin cancer that initially might look like a spreading bruise, or a raised purple-red bump. Over time, the tumor can grow and cause swelling, ulcers, and in extreme cases, bleeding. It is often found in the upper part of a limb that is swollen due to a breast removal surgery, a condition known as the Stewart-Treves tumor. Median size of these lesions typically range from 3 to 6 cm, but without treatment, the tumors can grow up to 20 cm or larger.
This condition can also happen in situations including:
- Congenital or hereditary abnormalities of the lymphatic system such as Turner syndrome, Noonan syndrome, Milroy disease, lymphedema praecox, and lymphedema tarda
- Chronic infections
- Chronic vein problems
- Extreme obesity
- Diseases that block lymphatic flow
- Numerous surgical procedures that disrupt the flow of lymph
Usually, people come to medical attention because of pain or discomfort from the tumor. It’s important to know that chronic lymphedema can take anywhere from 4 to more than 50 years to develop into angiosarcoma.
Testing for Stewart-Treves Syndrome
If your doctor suspects that you have angiosarcoma, a type of cancer, they might order imaging tests such as a CT scan or an MRI of your head and neck. These tests help your doctor see the level of impact on your bones and soft tissues, and can also provide valuable information about your lymph nodes. The lymph nodes are small, round structures that make and store cells that fight infection.
Patients diagnosed with angiosarcoma would often need to see medical and surgical oncologists. Oncologists are doctors who specialize in treating cancer. The medical oncologist uses medicine to treat cancer, and the surgical oncologist uses surgery to remove tumors.
Radiation therapy is also frequently used in the treatment of this condition. This involves using high-energy rays (similar to X-rays) to kill cancer cells. This method aims to treat cancer or relieve a patient’s pain and other symptoms.
Treatment Options for Stewart-Treves Syndrome
Surgery that removes tissue widely around the affected area is the recommended treatment. But even if lab tests find no remaining disease in the tissue surrounding the area, there’s still a higher chance that the disease could come back or spread to other parts of the body. For this reason, it’s often a good idea to involve a team of doctors from different specialties in the treatment process.
What else can Stewart-Treves Syndrome be?
When diagnosing skin angiosarcoma, a type of skin cancer, doctors must consider and rule out several other similar skin conditions. These could be either benign (non-cancerous) or malignant (cancerous). Some likely conditions that might resemble skin angiosarcoma include:
- Hemangioma (a benign tumor made up of blood vessels)
- Hemangioblastoma (a benign blood-vessel tumor typically found in the brain or spine)
- Squamous cell carcinoma (a common form of skin cancer)
- Kaposi sarcoma (a cancer affecting the skin and mucous membranes, often associated with HIV)
- Anaplastic melanoma (a very aggressive form of skin cancer)
- Telangiectatic metastatic breast disease (a potential breast cancer complication that shows up on the skin)
There have also been instances where skin angiosarcoma appears to mimic other common skin conditions such as rosacea and eczema. In some rare cases, it may even resemble conditions like eyelid swelling and solid, non-sinking facial swelling.
Surgical Treatment of Stewart-Treves Syndrome
Because there haven’t been a lot of randomized trials, the options for treating appendiceal neoplasms are limited. The best chance of survival for patients has been shown through an initial surgery which cuts out a larger portion around the tumor – these are referred to as “wide margins”. The goal is to ensure no traces of the disease are left behind, even if this means performing multiple surgeries. This can be challenging, as most patients are diagnosed when the disease has already grown considerably.
What to expect with Stewart-Treves Syndrome
In a recent review of patient records, it was found that people had a survival rate of 55% after 3 years and 35% after 5 years. The average length of survival was roughly 7 months. It was also observed that people under the age of 50, those with tumors that had not spread widely, and those with the tumor located on the trunk of the body had a better chance of survival. It’s unclear why those with tumors, called angiosarcomas, on the trunk of their body tend to live longer.