Papillary Intralymphatic Angioendothelioma

Papillary intralymphatic angioendothelioma (PILA) is a rare and low-grade tumor that affects the lymphatic vessels. It is also known as the Dabska tumor, named after Maria Dabska, who first described it in 1969. PILA typically occurs in children and young adults, and it often presents as a soft tissue mass on the skin or in the subcutaneous tissue. It is rare cancer that affects the lymphatic system. It is also known as PILA typically affects children and young adults, and it can occur in various parts of the body, including the skin, soft tissues, and bones.

PILA is classified as a vascular tumor, meaning that it involves the blood vessels or the lymphatic vessels. It is considered to be a borderline tumor, which means that it is between benign and malignant. While it is low-grade and does not typically metastasize, it has the potential to recur locally.

Types

The basic types of PILA in detail.

Superficial PILA: This type of PILA is located on the surface of the skin or mucous membranes. It typically presents as a reddish or bluish nodule that may bleed or ulcerate. Superficial PILA is usually slow-growing and may not cause any symptoms.
Deep PILA: This type of PILA is located deep within the tissues of the body, such as in the muscles, bones, or organs. Deep PILA is more aggressive than superficial PILA and can cause pain, swelling, or other symptoms depending on its location.
Combined PILA: This type of PILA involves both superficial and deep components. It is the rarest type of PILA and can be more difficult to treat.
Recurrent PILA: This type of PILA is characterized by the tumor coming back after treatment. Recurrent PILA can be more difficult to treat than the initial tumor and may require more aggressive treatment.
Metastatic PILA: This type of PILA occurs when cancer spreads to other parts of the body, such as the lungs, liver, or bones. Metastatic PILA is the most advanced stage of the disease and can be difficult to treat.

Causes

While the exact cause of PILA is not fully understood, several factors have been associated with its development. In this article, we will discuss the most common causes of PILA.

Genetics: Some cases of PILA may be caused by genetic mutations that affect the development of the lymphatic vessels.
Infections: Infections with certain viruses, such as human herpesvirus-8, have been associated with the development of PILA.
Trauma: Trauma or injury to the lymphatic vessels may increase the risk of developing PILA.
Radiation: Exposure to ionizing radiation may increase the risk of developing PILA.
Chemical exposure: Exposure to certain chemicals, such as vinyl chloride, may increase the risk of developing PILA.
Immune system disorders: People with autoimmune disorders or other immune system disorders may have an increased risk of developing PILA.
Hormonal imbalances: Hormonal imbalances, such as those seen in women with polycystic ovary syndrome (PCOS), may increase the risk of developing PILA.
Chronic inflammation: Chronic inflammation in the lymphatic vessels may contribute to the development of PILA.
Age: PILA is most commonly diagnosed in middle-aged adults, although it can occur at any age.
Gender: PILA occurs more frequently in females than in males.
Obesity: Obesity may increase the risk of developing PILA.
Smoking: Smoking has been associated with an increased risk of developing PILA.
Diabetes: People with diabetes may have an increased risk of developing PILA.
High blood pressure: High blood pressure may increase the risk of developing PILA.
Exposure to ultraviolet (UV) radiation: Exposure to UV radiation from the sun or tanning beds may increase the risk of developing PILA.
Poor diet: A poor diet that is high in fat, sugar, and processed foods may increase the risk of developing PILA.
Lack of exercise: Lack of exercise may increase the risk of developing PILA.
Chronic lymphedema: Chronic lymphedema, a condition in which the lymphatic vessels become blocked and fluid accumulates, may increase the risk of developing PILA.
Family history: A family history of PILA may increase the risk of developing the condition.
Unknown factors: In some cases, the cause of PILA may be unknown.

Symptoms

Symptoms of PILA and explain each symptom in detail.

Skin Lesions: PILA can present as a skin lesion, which is a change in the appearance or texture of the skin. These lesions are usually red or purple and can be raised or flat.
Swelling: Swelling, also known as edema, can occur in the affected area due to the accumulation of fluid.
Pain: Pain can occur in the affected area, especially if the tumor is located near a nerve.
Numbness: Numbness or tingling sensation can occur if the tumor compresses a nerve.
Lymphadenopathy: Enlarged lymph nodes can occur in the area near the tumor.
Fatigue: Fatigue is a feeling of tiredness or weakness and can occur due to the body’s response to the tumor.
Fever: A low-grade fever can occur in some cases.
Weight Loss: Unexplained weight loss can occur in some cases.
Joint Pain: Joint pain can occur if the tumor is located near a joint.
Limb Weakness: Weakness in the affected limb can occur if the tumor compresses a nerve.
Headache: Headache can occur if the tumor is located in the head or neck region.
Visual Disturbances: Visual disturbances can occur if the tumor is located in the eye or orbit.
Seizures: Seizures can occur if the tumor is located in the brain.
Respiratory Symptoms: Respiratory symptoms such as coughing, shortness of breath, and chest pain can occur if the tumor is located in the chest.
Abdominal Pain: Abdominal pain can occur if the tumor is located in the abdomen.
Jaundice: Jaundice, which is a yellowing of the skin and eyes, can occur if the tumor is located in the liver.
Gastrointestinal Symptoms: Gastrointestinal symptoms such as nausea, vomiting, and diarrhea can occur if the tumor is located in the gastrointestinal tract.
Back Pain: Back pain can occur if the tumor is located in the spine.
Bone Pain: Bone pain can occur if the tumor spreads to the bones.
Neurological Symptoms: Neurological symptoms such as confusion, memory loss, and difficulty speaking can occur if the tumor is located in the brain.

Diagnosis

Diagnosis of PILA can be challenging, as it can be mistaken for other types of cancers or inflammatory conditions. Here are tests and diagnostic procedures that may be used to diagnose PILA:

Biopsy – A sample of tissue is taken from the affected area and examined under a microscope to look for abnormal cells.
Immunohistochemistry – A test that uses antibodies to detect specific proteins in tissue samples. This can help determine the type of cancer present.
Ultrasound – A non-invasive test that uses high-frequency sound waves to produce images of the affected area. It can be used to look for abnormalities in the lymphatic vessels.
MRI – A test that uses a magnetic field and radio waves to produce detailed images of the body. It can be used to look for tumors and evaluate the extent of the disease.
CT scan – A test that uses X-rays and computer technology to produce detailed images of the body. It can be used to look for tumors and evaluate the extent of the disease.
PET scan – A test that uses a radioactive tracer to produce images of the body. It can be used to look for tumors and evaluate the extent of the disease.
Lymphangiogram – A test that involves injecting dye into the lymphatic vessels and taking X-rays to produce images of the lymphatic system. It can be used to look for abnormalities in the lymphatic vessels.
Lymphoscintigraphy – A test that involves injecting a small amount of radioactive material into the affected area and using a special camera to produce images of the lymphatic system. It can be used to look for abnormalities in the lymphatic vessels.
Fine needle aspiration (FNA) – A procedure that involves using a thin needle to extract a small sample of tissue from the affected area. It can be used to diagnose cancer and other conditions.
Blood tests – Blood tests can be used to look for markers of cancer or inflammation in the body.
Skin biopsy – A biopsy of the skin may be taken to look for abnormal cells.
Bone marrow biopsy – A biopsy of the bone marrow may be taken to look for cancer cells that have spread from the primary tumor.
Endoscopy – A procedure that involves inserting a thin, flexible tube with a camera into the affected area. It can be used to look for tumors and evaluate the extent of the disease.
Colonoscopy – A type of endoscopy that is used to look for abnormalities in the colon.
Bronchoscopy – A type of endoscopy that is used to look for abnormalities in the lungs.
Cystoscopy – A type of endoscopy that is used to look for abnormalities in the bladder.
Laparoscopy – A minimally invasive procedure that involves inserting a thin, flexible tube with a camera into the abdomen. It can be used to look for tumors and evaluate the extent of the disease.
Angiography – A test that involves injecting dye into the blood vessels and taking X-rays to produce images of the blood vessels. It can be used to look for abnormalities in the blood vessels.
Echocardiogram – A test that uses sound waves to produce images of the heart. It can be used to look for abnormalities in the heart.
Electrocardiogram (ECG) – A test that measures the electrical activity of the heart. It can be used to look

Treatment

Treatment options for PILA vary depending on the stage and extent of the tumor, as well as the patient’s overall health and medical history.

Here is a list of treatments that may be used to manage PILA:

Observation and monitoring: In some cases, doctors may recommend closely monitoring the tumor to see if it grows or causes any symptoms. This approach is typically used for small tumors that are not causing any problems.
Surgery: Surgery is the primary treatment for PILA. The goal of surgery is to remove the entire tumor and any surrounding tissue that may be affected. Depending on the size and location of the tumor, the surgeon may use a variety of techniques, including excision, Mohs micrographic surgery, or lymph node dissection.
Radiation therapy: Radiation therapy uses high-energy beams of radiation to kill cancer cells. This treatment may be used in conjunction with surgery or as a standalone treatment option.
Chemotherapy: Chemotherapy involves the use of drugs to kill cancer cells. This treatment is typically reserved for cases where the tumor has spread to other parts of the body.
Immunotherapy: Immunotherapy uses the body’s immune system to attack cancer cells. This treatment may be used in conjunction with other treatments or as a standalone option.
Targeted therapy: Targeted therapy uses drugs that are designed to target specific molecules that are involved in the growth and spread of cancer cells. This treatment may be used in conjunction with other treatments or as a standalone option.
Cryotherapy: Cryotherapy uses extreme cold to destroy cancer cells. This treatment may be used for small tumors that are located near the surface of the skin.
Electrodesiccation and curettage: This technique involves the use of an electric current to destroy cancer cells, followed by the removal of the dead tissue using a curette.
Photodynamic therapy: Photodynamic therapy uses a special type of light to activate a photosensitizing drug that has been applied to the skin. The activated drug then destroys cancer cells.
Laser therapy: Laser therapy uses a high-intensity beam of light to destroy cancer cells. This treatment may be used for small tumors that are located near the surface of the skin.
Topical therapies: Topical therapies involve the application of a cream or ointment directly to the skin. These therapies may include steroids, retinoids, or imiquimod.
Sclerotherapy: Sclerotherapy involves the injection of a sclerosing agent into the tumor to cause it to shrink and eventually disappear.
Lymphovenous anastomosis: This surgical procedure involves creating a connection between a lymphatic vessel and a vein to bypass the blocked or damaged lymphatic vessels.
Lymphaticovenular bypass: This surgical procedure involves creating a connection between a lymphatic vessel and a vein to redirect lymphatic fluid away from the affected area.
Lymph node transplant: This surgical procedure involves transplanting healthy lymph nodes into the affected area to replace damaged or diseased lymph nodes.
Complementary and alternative therapies: Complementary and alternative therapies may include acupuncture, massage, meditation, or herbal supplements. These therapies are used in conjunction with traditional medical treatments to manage symptoms and improve quality of life.
Pain management: Pain management techniques may include medication, nerve blocks, or other procedures to manage pain caused by PILA.
Palliative care: Palliative care focuses on managing symptoms

Medications

Drugs that can be used to treat PILA.

Vincristine: Vincristine is a chemotherapy drug that is often used to treat PILA. It works by disrupting the growth and division of cancer cells.
Doxorubicin: Doxorubicin is another chemotherapy drug that can be used to treat PILA. It is a type of anthracycline that works by interfering with DNA replication.
Cyclophosphamide: Cyclophosphamide is a chemotherapy drug that is often used in combination with other drugs to treat PILA. It works by suppressing the immune system and preventing the growth of cancer cells.
Etoposide: Etoposide is a chemotherapy drug that is often used to treat PILA. It works by inhibiting DNA synthesis and preventing the growth of cancer cells.
Ifosfamide: Ifosfamide is a chemotherapy drug that is often used in combination with other drugs to treat PILA. It works by interfering with DNA replication and preventing the growth of cancer cells.
Methotrexate: Methotrexate is a chemotherapy drug that can be used to treat PILA. It works by inhibiting DNA synthesis and preventing the growth of cancer cells.
Gemcitabine: Gemcitabine is a chemotherapy drug that can be used to treat PILA. It works by inhibiting DNA synthesis and preventing the growth of cancer cells.
Bevacizumab: Bevacizumab is a monoclonal antibody that can be used to treat PILA. It works by blocking the growth of new blood vessels, which is necessary for the growth and spread of cancer cells.
Imatinib: Imatinib is a tyrosine kinase inhibitor that can be used to treat PILA. It works by blocking the activity of certain enzymes that are necessary for the growth and division of cancer cells.
Sorafenib: Sorafenib is a tyrosine kinase inhibitor that can be used to treat PILA. It works by blocking the activity of certain enzymes that are necessary for the growth and division of cancer cells.
Sunitinib: Sunitinib is a tyrosine kinase inhibitor that can be used to treat PILA. It works by blocking the activity of certain enzymes that are necessary for the growth and division of cancer cells.
Pazopanib: Pazopanib is a tyrosine kinase inhibitor that can be used to treat PILA. It works by blocking the activity of certain enzymes that are necessary for the growth and division of cancer cells.
Nivolumab: Nivolumab is a monoclonal antibody that can be used to treat PILA. It works by blocking a protein called PD-1, which can prevent the immune system from attacking cancer cells.
Pembrolizumab: Pembrolizumab is a monoclonal antibody that can be used to treat PILA. It works by blocking a protein called PD-1, which can prevent the immune system from attacking cancer cells.

References