What is Endovascular Papillary Angioendothelioma?
Endovascular papillary angioendothelioma (EPA), also known as Dabska tumor (DT) and papillary intralymphatic angioendothelioma (PILA), is a condition that is somewhere between a benign blood vessel tumor called hemangioma and a type of cancer called angiosarcoma. EPA generally has a good outlook, although there is a chance it could come back after treatment and can spread, albeit at a low level. This condition is most common in children and usually appears in various skin locations, below the skin surface, or deeper within the body. However, EPA can also occur in adults and affect internal organs.
The affected areas or lesions are generally around two to three centimeters in size when they’re first identified. The diagnosis of EPA is confirmed through a biopsy, which is a procedure where a small sample of tissue is taken for examination. The standard treatment for EPA is a type of surgery called wide surgical excision, which involves removing the tumor and some healthy tissue around it. Because EPA is so rare, most of the information we have about it comes from reports of individual cases or collections of cases.
What Causes Endovascular Papillary Angioendothelioma?
Endovascular papillary angioendothelioma, a type of blood vessel tumor, can appear from nowhere or develop in a region that has chronic, long-term swelling (or lymphedema), or an irregularity in the blood vessels like a hemangioma (a birthmark that appears at birth or in the first or second week of life) or lymphangioma circumscriptum (a rare skin condition characterized by clusters of translucent vesicles).
There are similar-looking conditions, such as retiform hemangioendothelioma (a very rare, slow-growing cancer that starts in the cells lining the blood vessels), angiosarcoma (a cancer that forms on the inner lining of blood vessels), reactive angioendotheliomatosis (a rare condition characterized by the growth of blood vessels), and intravascular endothelial hyperplasia (a benign, non-cancerous growth that occurs inside the blood vessels).
Researchers think this type of tumor might originate in the lymphatic system — network of tissues and organs that help rid the body of toxins, waste, and other unwanted materials — because of similarities between this tumor and nearby lymphatics or lymphangioma, as well as because of the presence of proteins known as “vascular endothelial growth factor” and “D2-40”. These proteins are found in blood vessels and lymphatic tissue, respectively, and may play a role in their growth.
Risk Factors and Frequency for Endovascular Papillary Angioendothelioma
The Dabska tumor was first identified in a 1969 study involving six kids. Since then, the number of reported cases remains fairly low, with fewer than 40 described in medical literature. The tumor doesn’t appear to affect one gender more than the other, and it’s mostly found in children, making up about 75% of patients. However, it has also shown up in older adults. In a group of 12 patients, the ages ranged from 8 to 59, with an average age of 30. This suggests that the tumor can affect a broader age range. The Dabska tumor usually appears as a skin lesion, but reports show it can also arise in the spleen, testicle, tongue, and bone.
- The Dabska tumor was first identified in 1969 in a study involving six children.
- Less than 40 cases have been reported since its discovery.
- It doesn’t favor males or females.
- About 75% of the cases are in children, but it can also affect older adults.
- Ages of patients can range from 8 to 59, with an average of 30 years old.
- While it mostly appears as a skin lesion, it has been found in the spleen, testicle, tongue, and bone.
Signs and Symptoms of Endovascular Papillary Angioendothelioma
Endovascular papillary angioendothelioma is a condition that manifests as a slow-growing and painless skin growth, usually situated in the tissue beneath the skin of the arms or legs, the skin itself, or less commonly, the torso, head, and neck. In rare instances, the growth can occur in deeper tissues such as bone, brain, spleen, tongue, or testicles. In these cases, no other primary sources have been found, nor was there any involvement of the lymph nodes.
Typically, skin tumors from this condition are noticed when they reach about two to three centimeters in size. However, some have been reported to grow as large as 40 centimeters in diameter. The appearance of the tumors can vary and may be described as an undefined mass, a skin plaque, or a nodule that projects into surrounding tissues. The skin covering the tumor might have a pink, blue, or violet color, and the skin layer might appear thin or shrunk.
Symptoms can also differ among individuals. Some people may experience pain, skin ulcerations, or bleeding at the affected site. Although these growths are typically low grade, cases of involvement of the regional nodes and even spread to lungs have been reported. Therefore, a thorough physical examination is crucial, including tests to check for metastasis.
Testing for Endovascular Papillary Angioendothelioma
If a doctor suspects Ectomesenchymal chondromyxoid tumor of the anterior tongue (EPA), the final way to confirm it is through a procedure called a biopsy. Since EPA is extremely rare, there are no established guidelines yet for tracking and handling the disease. However, if a patient is experiencing lung-related symptoms, it might be useful to get a chest X-ray. This is because there has been a reported case where a patient died from lung metastases, where the disease spread to the lungs from the tongue. If there are reasons to believe the lymph nodes could be involved, these can also be checked.
Treatment Options for Endovascular Papillary Angioendothelioma
Surgery is generally recommended to remove Dabska tumors. The outlook is usually good even if the tumor has invaded deeper structures. That being said, since the tumor has the potential to become cancerous, regular check-ups are recommended.
When the tumor has reached the surrounding lymph nodes, an additional procedure to remove these nodes might be needed. This procedure is called a regional lymphadenectomy.
In one instance, doctors followed a specific set of cancer care guidelines known as the National Comprehensive Cancer Network (NCCN) practice guidelines. These guidelines were for dealing with soft tissue sarcoma located in the limbs or trunk of the body, categorized as stage IIA (T1bN0M0G2). Following these guidelines, doctors recommended imaging tests for the chest, such as x-rays or CT scans, every 3 to 6 months for the first 2 to 3 years. Then, check-ups needed to be done every six months for the next two years, and then every year for continual monitoring.
What else can Endovascular Papillary Angioendothelioma be?
When diagnosing this particular type of tumor, the doctors have to consider different conditions that appear similar under the microscope. This way of diagnosing is called ‘histologic’. The conditions that could be mistaken for this tumor are:
- Intravascular papillary endothelial hyperplasia (also known as Masson tumor)
- Epithelioid hemangioendothelioma
- Angiosarcoma
- Lymphangioma-like Kaposi sarcoma
- Retiform hemangioendothelioma
This kind of tumor has unique features, like tiny finger-like growths similar to those found in certain stages of Kaposi sarcoma (a type of cancer). These growths can also appear in other variants of the tumor. However, these other variants would also display other distinct features, like more abnormal cells and uncontrolled growth beyond the blood vessels.
Another condition to consider is the Masson phenomenon, which are organized blood clots that show finger-like processes covered in a layer of cells. Unlike this tumor, the Masson phenomenon are contained within blood vessels, and visible intravascular blood clots are often seen. It lacks the presence of a certain type of cells and has a single layer of cells surrounding solidified tissue cores.
The condition called epithelioid hemangioendothelioma displays cells with pink, clear spaces within them. These tumor cells arrange in different patterns with a jelly-like or hardening stroma (the supportive framework of a cell), and often grow beyond the blood vessel.
It’s important to note that there have been recorded instances of benign (non-cancerous) growths inside blood vessels that show features similar to this tumor, giving a false appearance of cancer. This means that seeing abnormal features in a usually benign growth should not lead to unnecessary aggressive treatment.
Surgical Treatment of Endovascular Papillary Angioendothelioma
The standard treatment for these tumors is a widespread surgical removal. This method is often enough for most patients, and they don’t experience the return of the tumor. However, there have been a few instances of the cancer spreading to the lymph nodes and lungs, suggesting the need for regular check-ups and long-term monitoring after treatment.
What to expect with Endovascular Papillary Angioendothelioma
In simple terms, endovascular papillary angioendothelioma, also known as Dabska tumors, are usually low-grade tumors that don’t often behave aggressively. They usually have positive outcomes when treated with surgery alone. Looking at the long-term health of patients with these tumors, the results are generally excellent.
However, there have been instances where the tumor has spread to the lymph nodes or lungs, so it’s crucial for patients to have regular check-ups and evaluations to monitor their health.
Possible Complications When Diagnosed with Endovascular Papillary Angioendothelioma
Endovascular papillary angioendothelioma, also known as a type of blood vessel tumor, tends to grow slowly and generally has a good prognosis or expected outcome. However, it’s important not to overlook these tumors as there have been instances of them spreading to the lungs. So, these tumors need timely evaluation and treatment.
Potential Risks:
- Slow-growing endovascular papillary angioendothelioma
- Good prognosis, but requires careful monitoring
- Potential for tumors to spread to the lungs
- Importance of timely evaluation and treatment to prevent complications
Preventing Endovascular Papillary Angioendothelioma
Endovascular papillary angioendothelioma is a medical term for a specific type of skin lump. It’s important for this condition to be considered, especially in children who have slow-growing lumps in the skin. The lumps should be examined properly to make sure the correct diagnosis is made. This is particularly important for skin doctors because children with these lumps will likely see them first. So, it’s crucial for these doctors to be aware of this condition and how to treat it.