Endovascular Papillary Angioendothelioma

Last updated: February 8, 2026Reviewed date: February 8, 2026Reading time: 14 min read
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Endovascular papillary angioendothelioma (EPA), also known as papillary intralymphatic angioendothelioma (PILA), is a rare type of tumor that typically affects the skin and soft tissues, but can also occur in other organs such as the lungs, liver, and spleen. This tumor is characterized by the...

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Article Summary

Endovascular papillary angioendothelioma (EPA), also known as papillary intralymphatic angioendothelioma (PILA), is a rare type of tumor that typically affects the skin and soft tissues, but can also occur in other organs such as the lungs, liver, and spleen. This tumor is characterized by the formation of papillary projections within small blood vessels, which can lead to obstruction and swelling of the affected tissue. Types...

Key Takeaways

  • This article explains Causes in simple medical language.
  • This article explains Symptoms in simple medical language.
  • This article explains Diagnosis in simple medical language.
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Definition

Endovascular papillary angioendothelioma (EPA), also known as papillary intralymphatic angioendothelioma (PILA), is a rare type of tumor that typically affects the skin and soft tissues, but can also occur in other organs such as the lungs, liver, and spleen. This tumor is characterized by the formation of papillary projections within small blood vessels, which can lead to obstruction and swelling of the affected tissue.

Types of EPA

  1. Cutaneous EPA: Cutaneous EPA is the most common type of EPA and typically presents as a solitary, painless nodule or papule on the skin. The ulcer. সহজ বাংলা: শরীরের অস্বাভাবিক দাগ, ক্ষত বা ফোলা অংশ।" data-rx-term="lesion" data-rx-definition="A lesion is an abnormal area of tissue such as a spot, wound, patch, lump, or ulcer. সহজ বাংলা: শরীরের অস্বাভাবিক দাগ, ক্ষত বা ফোলা অংশ।">lesion may be pink or reddish in color and can occur anywhere on the body, but is most commonly found on the lower extremities. Cutaneous EPA has a good prognosis and is usually treated with surgical excision.
  2. Soft tissue EPA: Soft tissue EPA is a rare form of disease that can occur in the muscles, tendons, and fascia. The ulcer. সহজ বাংলা: শরীরের অস্বাভাবিক দাগ, ক্ষত বা ফোলা অংশ।" data-rx-term="lesion" data-rx-definition="A lesion is an abnormal area of tissue such as a spot, wound, patch, lump, or ulcer. সহজ বাংলা: শরীরের অস্বাভাবিক দাগ, ক্ষত বা ফোলা অংশ।">lesion may present as a painless mass or swelling and may be mistaken for a benign tumor. Soft tissue EPA has a variable prognosis and is usually treated with surgical excision and radiation therapy.
  3. Pulmonary EPA: Pulmonary EPA is a rare form of the disease that affects the lungs. The ulcer. সহজ বাংলা: শরীরের অস্বাভাবিক দাগ, ক্ষত বা ফোলা অংশ।" data-rx-term="lesion" data-rx-definition="A lesion is an abnormal area of tissue such as a spot, wound, patch, lump, or ulcer. সহজ বাংলা: শরীরের অস্বাভাবিক দাগ, ক্ষত বা ফোলা অংশ।">lesion may present as a solitary nodule or as multiple nodules and can be mistaken for other pulmonary diseases such as tuberculosis or lung cancer. Pulmonary EPA has a variable prognosis and is usually treated with surgical excision or lung transplantation.
  4. Hepatic EPA: Hepatic EPA is a rare form of the disease that affects the liver. The ulcer. সহজ বাংলা: শরীরের অস্বাভাবিক দাগ, ক্ষত বা ফোলা অংশ।" data-rx-term="lesion" data-rx-definition="A lesion is an abnormal area of tissue such as a spot, wound, patch, lump, or ulcer. সহজ বাংলা: শরীরের অস্বাভাবিক দাগ, ক্ষত বা ফোলা অংশ।">lesion may present as a solitary mass or as multiple nodules and can be mistaken for other liver diseases such as hepatocellular carcinoma or metastatic liver cancer. Hepatic EPA has a variable prognosis and is usually treated with surgical resection or liver transplantation.
  5. Intravascular EPA: Intravascular EPA is a rare form of the disease that affects the blood vessels. The ulcer. সহজ বাংলা: শরীরের অস্বাভাবিক দাগ, ক্ষত বা ফোলা অংশ।" data-rx-term="lesion" data-rx-definition="A lesion is an abnormal area of tissue such as a spot, wound, patch, lump, or ulcer. সহজ বাংলা: শরীরের অস্বাভাবিক দাগ, ক্ষত বা ফোলা অংশ।">lesion may present as a mass within a blood vessel and can lead to obstruction and impaired blood flow. Intravascular EPA has a variable prognosis and is usually treated with surgical excision and radiation therapy.
  6. Bone EPA: Bone EPA is a rare form of the disease that affects the bones. The ulcer. সহজ বাংলা: শরীরের অস্বাভাবিক দাগ, ক্ষত বা ফোলা অংশ।" data-rx-term="lesion" data-rx-definition="A lesion is an abnormal area of tissue such as a spot, wound, patch, lump, or ulcer. সহজ বাংলা: শরীরের অস্বাভাবিক দাগ, ক্ষত বা ফোলা অংশ।">lesion may present as a solitary lesion or as multiple lesions and can be mistaken for other bone diseases such as osteosarcoma or metastatic bone cancer. Bone EPA has a variable prognosis and is usually treated with surgical excision and radiation therapy.

Causes

The causes of EPA are not fully understood, but some risk factors have been identified. Here are potential causes or factors that may contribute to the development of EPA:

  1. Genetic mutations: EPA has been associated with mutations in the TEK gene, which codes for the protein tyrosine kinase receptor Tie2. This mutation is thought to be involved in the abnormal growth of blood vessels in EPA.
  2. Infection: Some researchers believe that EPA may be triggered by a viral or bacterial infection, although no specific pathogen has been identified.
  3. Environmental exposure: Exposure to certain chemicals or toxins in the environment may increase the risk of EPA. For example, exposure to asbestos has been linked to the development of some types of vascular tumors.
  4. Radiation exposure: EPA has been reported in patients who have received radiation therapy, especially for breast cancer or lymphoma.
  5. Immune system dysfunction: A compromised immune system may play a role in the development of EPA, although the exact mechanism is unclear.
  6. Age: EPA is more commonly seen in children and young adults, although it can occur at any age.
  7. Gender: EPA appears to affect females more often than males, although the reason for this is unknown.
  8. Race: There is no known racial or ethnic predisposition to EPA.
  9. Hormonal factors: Some researchers have suggested that EPA may be influenced by hormonal factors, such as estrogen, although this has not been proven.
  10. Trauma: Some cases of EPA have been reported after trauma or surgery, although it is unclear whether this is a causal relationship.
  11. Obesity: Obesity has been associated with an increased risk of some types of cancer, although its role in the development of EPA is unclear.
  12. insulin is low or not working well. সহজ বাংলা: রক্তে চিনি বেশি থাকার রোগ।" data-rx-term="diabetes" data-rx-definition="Diabetes is a condition where blood sugar stays too high because insulin is low or not working well. সহজ বাংলা: রক্তে চিনি বেশি থাকার রোগ।">Diabetes: Diabetes has also been linked to an increased risk of certain types of cancer, although there is no direct evidence linking it to EPA.
  13. Smoking: Smoking has been associated with an increased risk of some types of cancer, although its role in the development of EPA is unclear.
  14. Alcohol consumption: Heavy alcohol consumption has been linked to an increased risk of some types of cancer, although there is no direct evidence linking it to EPA.
  15. Diet: A diet high in fat and low in fruits and vegetables has been associated with an increased risk of some types of cancer, although its role in the development of EPA is unclear.
  16. Inherited syndromes: Some inherited syndromes, such as Gorlin syndrome and blue rubber bleb nevus syndrome, have been associated with an increased risk of vascular tumors, including EPA.
  17. Chemotherapy: Some cases of EPA have been reported in patients who have received chemotherapy, although it is unclear whether this is a causal relationship.
  18. Immunotherapy: Immunotherapy, which uses the patient’s own immune system to fight cancer, has been associated with an increased risk of some types of vascular tumors, including EPA.
  19. Hormone therapy: Hormone therapy, which is often used to treat breast cancer and other hormone-dependent cancers, has been associated with an increased risk of some types of vascular tumors, including EPA.
  20. Unknown factors: Despite extensive research, the exact cause of EPA remains unknown in many cases.

Symptoms

Symptoms of endovascular papillary hemangioendothelioma in detail.

  1. Fatigue: People with endovascular papillary angioendothelioma may experience persistent fatigue that does not improve with rest.
  2. Loss of appetite: A decreased appetite and unintentional weight loss may occur due to the cancer’s effects on the body.
  3. Nausea and vomiting: Nausea and vomiting may occur due to the cancer or the treatments used to manage it.
  4. Abdominal pain: If the tumor is located in the liver or abdominal cavity, abdominal pain may occur.
  5. Jaundice: Yellowing of the skin and eyes may occur if the tumor is located in the liver.
  6. Shortness of breath: If the tumor is located in the lungs, shortness of breath may occur.
  7. Coughing: A persistent cough may occur if the tumor is located in the lungs.
  8. Chest pain: Chest pain may occur if the tumor is located in the lungs or chest cavity.
  9. Bone pain: If the tumor is located in the bones, bone pain may occur.
  10. Swelling: Swelling in the affected area may occur due to the tumor’s effects on the surrounding tissues.
  11. Skin changes: Skin changes, such as redness or swelling, may occur if the tumor is located near the skin’s surface.
  12. Fever: A low-grade fever may occur due to the body’s immune response to the cancer.
  13. Night sweats: Night sweats may occur due to the cancer’s effects on the body’s hormones.
  14. Neurological symptoms: Depending on the location of the tumor, neurological symptoms such as headaches, seizures, or weakness may occur.
  15. Joint pain: If the tumor is located near a joint, joint pain may occur.
  16. Palpable mass: A lump or palpable mass may be felt in the affected area.
  17. Elevated liver enzymes: If the tumor is located in the liver, elevated liver enzymes may be detected on blood tests.
  18. Blood in urine or stool: If the tumor is located in the urinary or gastrointestinal tract, blood may be present in the urine or stool.
  19. Irregular menstrual cycles: In women, irregular menstrual cycles may occur due to the cancer’s effects on hormone levels.
  20. Erectile dysfunction: In men, erectile dysfunction may occur due to the cancer’s effects on blood flow.

Diagnosis

Diagnosis and tests for Endovascular papillary angioendothelioma.

  1. Biopsy: A biopsy is the gold standard diagnostic test for EPA. A small piece of tissue is removed from the affected area and examined under a microscope to look for the characteristic features of the tumor.
  2. Immunohistochemistry: This test uses antibodies to identify specific proteins that are present in EPA cells. It can help distinguish EPA from other types of tumors.
  3. Radiographic imaging: Imaging tests such as X-rays, CT scans, and MRI scans can help visualize the extent of the tumor and identify any metastases.
  4. Ultrasonography: This test uses sound waves to create images of the affected area. It can help identify the size, location, and blood flow of the tumor.
  5. Doppler ultrasonography: This test uses sound waves to measure blood flow in the affected area. It can help identify any abnormalities in the blood vessels.
  6. Angiography: This test uses contrast dye and X-rays to visualize blood vessels. It can help identify any abnormalities in the blood vessels and detect any bleeding.
  7. Magnetic resonance angiography (MRA): This test uses a magnetic field and radio waves to visualize blood vessels. It can help identify any abnormalities in the blood vessels.
  8. Computed tomography angiography (CTA): This test uses X-rays and contrast dye to visualize blood vessels. It can help identify any abnormalities in the blood vessels.
  9. PET scan: This test uses a radioactive tracer to detect metabolic activity in the affected area. It can help identify any areas of increased metabolic activity, which may indicate the presence of a tumor.
  10. Blood tests: Blood tests can help identify any abnormalities in blood cells or liver enzymes, which may be associated with EPA.
  11. Lymph node biopsy: If there is suspicion of metastasis to the lymph nodes, a biopsy may be performed to confirm the diagnosis.
  12. Bone scan: This test uses a radioactive tracer to detect bone abnormalities. It can help identify any metastases to the bones.
  13. Echocardiography: This test uses ultrasound to visualize the heart and its blood vessels. It can help identify any abnormalities in the heart or blood vessels.
  14. Pulmonary function tests: These tests measure how well the lungs are functioning. They can help identify any abnormalities in lung function that may be associated with EPA.
  15. Electrocardiogram (ECG): This test measures the electrical activity of the heart. It can help identify any abnormalities in heart function.
  16. Endoscopy: This test uses a flexible tube with a camera to visualize the inside of the digestive tract. It can help identify any abnormalities in the digestive tract that may be associated with EPA.
  17. Colonoscopy: This test uses a flexible tube with a camera to visualize the inside of the colon. It can help identify any abnormalities in the colon that may be associated with EPA.
  18. Cytogenetic analysis: This test examines the chromosomes of EPA cells to identify any abnormalities that may be associated with the tumor.
  19. Fluorescence in situ hybridization (FISH): This test uses fluorescent probes to identify specific genetic abnormalities in EPA cells.
  20. Polymerase chain reaction (PCR): This test amplifies DNA from EPA cells to identify specific genetic abnormalities.

Treatment

Potential treatments for EPA.

  1. Surgical resection: Surgical removal of the tumor is often the first treatment option for EPA, particularly for localized tumors. The goal of surgery is to remove as much of the tumor as possible while preserving normal tissue and function. In some cases, additional treatments may be necessary after surgery.
  2. Radiation therapy: Radiation therapy involves the use of high-energy X-rays to kill cancer cells. It may be used in combination with surgery or as a stand-alone treatment for EPA. Radiation therapy is often reserved for tumors that cannot be completely removed by surgery or those that have spread to other parts of the body.
  3. Chemotherapy: Chemotherapy involves the use of drugs to kill cancer cells. It may be used in combination with surgery or radiation therapy or as a stand-alone treatment for EPA. Chemotherapy is often used for tumors that have spread to other parts of the body.
  4. Immunotherapy: Immunotherapy involves the use of drugs or other substances to stimulate the body’s immune system to recognize and attack cancer cells. It may be used in combination with other treatments or as a stand-alone treatment for EPA.
  5. Targeted therapy: Targeted therapy involves the use of drugs that specifically target the molecular abnormalities present in cancer cells. It may be used in combination with other treatments or as a stand-alone treatment for EPA.
  6. Cryotherapy: Cryotherapy involves the use of extreme cold to freeze and destroy cancer cells. It may be used in combination with other treatments or as a stand-alone treatment for EPA.
  7. Photodynamic therapy: Photodynamic therapy involves the use of a special medication that is activated by a specific type of light to kill cancer cells. It may be used in combination with other treatments or as a stand-alone treatment for EPA.
  8. Radiofrequency ablation: Radiofrequency ablation involves the use of high-energy radio waves to destroy cancer cells. It may be used in combination with other treatments or as a stand-alone treatment for EPA.
  9. Embolization: Embolization involves the use of materials, such as beads or gels, to block the blood supply to the tumor. It may be used in combination with other treatments or as a stand-alone treatment for EPA.
  10. High-intensity focused ultrasound (HIFU): HIFU involves the use of high-frequency sound waves to destroy cancer cells. It may be used in combination with other treatments or as a stand-alone treatment for EPA.
  11. Microwave ablation: Microwave ablation involves the use of high-frequency microwaves to destroy cancer cells. It may be used in combination with other treatments or as a stand-alone treatment for EPA.
  12. Photothermal therapy: Photothermal therapy involves the use of a special medication that is activated by a specific type of light to generate heat and destroy cancer cells. It may be used in combination with other treatments or as a stand-alone treatment for EPA.
  13. Electrochemotherapy: Electrochemotherapy involves the use of electric pulses to enhance the uptake of chemotherapy drugs by cancer cells. It may be used in combination with other treatments or as a stand-alone treatment for EPA.
  14. Intralesional chemotherapy: Intralesional chemotherapy involves the direct injection of chemotherapy drugs into the tumor. It may be used in combination with other treatments or as a stand-alone treatment for

Medications

There are various treatment options available for endovascular papillary angioendothelioma, including drugs, radiation therapy, surgery, and chemotherapy. In this article, we will focus on 20 drugs that can be used to treat this condition.

  1. Pazopanib – This is a targeted therapy drug that inhibits the growth of blood vessels and is used to treat advanced stages of endovascular papillary angioendothelioma.
  2. Sorafenib – This is another targeted therapy drug that is used to treat advanced stages of endovascular papillary angioendothelioma. It works by blocking the action of proteins that promote the growth of blood vessels.
  3. Sunitinib – This targeted therapy drug works by inhibiting the growth of blood vessels and is used to treat advanced stages of endovascular papillary angioendothelioma.
  4. Bevacizumab – This is a monoclonal antibody drug that inhibits the growth of blood vessels and is used in combination with chemotherapy to treat advanced stages of endovascular papillary angioendothelioma.
  5. Axitinib – This targeted therapy drug inhibits the growth of blood vessels and is used to treat advanced stages of endovascular papillary angioendothelioma.
  6. Gemcitabine – This chemotherapy drug is used to treat advanced stages of endovascular papillary angioendothelioma by inhibiting the growth of cancer cells.
  7. Doxorubicin – This chemotherapy drug is used to treat advanced stages of endovascular papillary angioendothelioma by inhibiting the growth of cancer cells.
  8. Ifosfamide – This chemotherapy drug is used to treat advanced stages of endovascular papillary angioendothelioma by inhibiting the growth of cancer cells.
  9. Vinorelbine – This chemotherapy drug is used to treat advanced stages of endovascular papillary angioendothelioma by inhibiting the growth of cancer cells.
  10. Cisplatin – This chemotherapy drug is used to treat advanced stages of endovascular papillary angioendothelioma by inhibiting the growth of cancer cells.
  11. Epirubicin – This chemotherapy drug is used to treat advanced stages of endovascular papillary angioendothelioma by inhibiting the growth of cancer cells.
  12. Mitomycin C – This chemotherapy drug is used to treat advanced stages of endovascular papillary angioendothelioma by inhibiting the growth of cancer cells.
  13. Carboplatin – This chemotherapy drug is used to treat advanced stages of endovascular papillary angioendothelioma by inhibiting the growth of cancer cells.
  14. Interferon alfa-2a – This immunotherapy drug is used to treat advanced stages of endovascular papillary angioendothelioma by boosting the immune system’s ability to fight cancer cells.
  15. Interferon alfa-2b – This immunotherapy drug is used to treat advanced stages of endovascular papillary angioendothelioma by boosting the immune system’s ability to fight cancer cells.
  16. Everolimus – This targeted therapy drug inhibits the growth of blood vessels and is used to treat advanced stages of endovascular papillary angioendothelioma.

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Safe first steps

  • Drink safe fluids and monitor temperature.
  • In dengue-prone areas, discuss CBC and platelet count when fever persists or warning signs appear.
  • Use tepid sponging for high fever discomfort; avoid ice-cold bathing.

OTC medicine safety

  • For fever, common fever medicine may be discussed with a clinician or pharmacist.
  • Avoid aspirin/ibuprofen-like medicines in suspected dengue unless a doctor says it is safe.

Avoid these mistakes

  • Do not start antibiotics without a proper medical decision.
  • Do not use steroid tablets or injections casually for quick relief.
  • Do not delay emergency care because of home remedies.

Get urgent help if

  • Fever with breathing difficulty, confusion, repeated vomiting, bleeding, severe weakness, stiff neck, or dehydration needs urgent care.
Medicine names, dose, and timing must be decided by a qualified clinician or pharmacist after checking age, pregnancy, allergy, other diseases, and current medicines.

For rural patients and family caregivers

Patient health record and symptom diary

Write your symptoms, medicines already taken, test results, and questions before visiting a doctor. This note stays on your device unless you print or copy it.

Doctor to discuss: Medicine doctor / pediatrician for children / qualified clinician
Tests to discuss with doctor
  • Temperature chart and hydration assessment
  • CBC with platelet count if fever persists or dengue/other infection is possible
  • Urine test, malaria/dengue tests, chest evaluation, or blood culture only when clinically indicated
Questions to ask
  • What is the most likely cause of my symptoms?
  • Which warning signs mean I should go to emergency care?
  • Which tests are really needed now?
  • Which medicines are safe for my age, pregnancy status, allergy, kidney/liver/stomach condition, and current medicines?
  • Do I need antibiotics, or is this more likely viral?

Emergency warning signs such as chest pain, severe breathing difficulty, sudden weakness, confusion, severe dehydration, major injury, or loss of bladder/bowel control need urgent medical care. Do not wait for online information.

Safe pathway to proper treatment

Care roadmap for: Endovascular Papillary Angioendothelioma

Use this simple roadmap to understand the next safe steps. It is educational and does not replace examination by a doctor.

Go to emergency care if you notice:
  • Severe or rapidly worsening symptoms
  • Breathing difficulty, chest pain, fainting, confusion, severe weakness, major injury, or severe dehydration
Doctor / service to discuss: Qualified healthcare provider; specialist depends on symptoms and examination.
  1. Step 1

    Check danger signs first

    If danger signs are present, seek emergency care and do not wait for online information.

  2. Step 2

    Record the symptom story

    Write when symptoms started, severity, medicines already taken, allergies, pregnancy status, and test results.

  3. Step 3

    Visit a qualified clinician

    A doctor, nurse, or qualified healthcare provider can examine you and decide which tests or treatment are needed.

  4. Step 4

    Do only useful tests

    Do tests after clinical assessment. Avoid unnecessary tests, random antibiotics, or repeated medicines without diagnosis.

  5. Step 5

    Follow up and return early if worse

    If symptoms worsen, new warning signs appear, or treatment is not helping, return for review quickly.

Rural patient practical tips
  • Take a written symptom diary and all previous prescriptions/test reports.
  • Do not hide medicines already taken, even herbal or over-the-counter medicines.
  • Ask which warning signs mean urgent referral to hospital.

This roadmap is for education. A real diagnosis and treatment plan requires history, examination, and clinical judgment.

RX Patient Help

Ask a health question safely

Write your symptom story. A health professional or site editor can review it before any answer is prepared. This box is not for emergency care.

Emergency first: Severe chest pain, breathing trouble, unconsciousness, stroke signs, severe injury, heavy bleeding, or rapidly worsening symptoms need urgent local medical care now.

Frequently Asked Questions

Is this article a replacement for a doctor?

No. It is educational content only. Patients should consult a qualified clinician for diagnosis and treatment.

When should I seek urgent care?

Seek urgent care for severe symptoms, rapidly worsening condition, breathing difficulty, severe pain, neurological changes, or any emergency warning sign.

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