Composite hemangioendothelioma (CHE) is a rare type of vascular tumor that is composed of different cell types. This tumor was first described in 2001 and since then, only a few cases have been reported in the medical literature. In this article, we will explore the various types of CHE, its symptoms, diagnosis, and treatment options.
Composite hemangioendothelioma is a rare type of vascular tumor that is composed of several different types of endothelial cells. These tumors can occur in various parts of the body, including the skin, soft tissue, liver, lung, bone, and lymph nodes.
Types
Types of Composite Hemangioendothelioma:
There are four types of CHE, each with different characteristics and cell types. These are:
- Dermal CHE: Dermal CHE is a type of tumor that is located in the skin. It is usually slow-growing and may appear as a reddish or bluish lump on the skin. This type of CHE is composed of different cell types including spindle cells, epithelioid cells, and vascular endothelial cells.
- Soft tissue CHE: Soft tissue CHE is a rare tumor that is located in the soft tissues of the body such as the muscles, tendons, and ligaments. It is composed of spindle cells, epithelioid cells, and vascular endothelial cells. This type of tumor may cause pain and swelling in the affected area.
- Hepatic CHE: Hepatic CHE is a tumor that is located in the liver. It is a rare type of liver tumor and is composed of spindle cells, epithelioid cells, and vascular endothelial cells. This type of tumor may cause liver dysfunction, abdominal pain, and weight loss.
- Pulmonary CHE: Pulmonary CHE is a tumor that is located in the lungs. It is composed of spindle cells, epithelioid cells, and vascular endothelial cells. This type of tumor may cause shortness of breath, cough, and chest pain.
Causes
The exact causes of CHE are not fully understood, but there are several factors that are believed to contribute to its development. In this article, we will discuss the causes of CHE in detail.
- Genetic mutations: Some genetic mutations have been linked to the development of CHE. These mutations may affect the genes that control the growth and division of cells, leading to the formation of tumors.
- Environmental factors: Exposure to certain environmental factors, such as radiation or chemicals, may increase the risk of developing CHE.
- Hormonal imbalances: Hormonal imbalances, such as those caused by pregnancy or menopause, may contribute to the development of CHE.
- Immune system disorders: Disorders of the immune system, such as autoimmune diseases, may increase the risk of developing CHE.
- Infections: Infections, such as hepatitis B or C, have been linked to the development of CHE.
- Age: CHE is more common in adults than in children, and the risk of developing the tumor increases with age.
- Gender: Females are more likely to develop CHE than males.
- Race: There is a higher incidence of CHE in Asian populations compared to other races.
- Previous cancer treatment: CHE has been reported in patients who have previously received radiation therapy or chemotherapy.
- Previous surgery: There have been cases of CHE developing at the site of previous surgeries.
- Family history: There may be a genetic predisposition to the development of CHE in some families.
- Blood vessel abnormalities: Abnormalities in the blood vessels, such as aneurysms or malformations, may contribute to the development of CHE.
- Lymphatic abnormalities: Abnormalities in the lymphatic system, such as lymphangiomas or lymphedema, may increase the risk of developing CHE.
- Chronic inflammation: Chronic inflammation, such as that caused by certain autoimmune diseases, may contribute to the development of CHE.
- Trauma: Trauma to the affected area may increase the risk of developing CHE.
- Obesity: Obesity has been linked to an increased risk of developing CHE.
- Diabetes: Diabetes has also been linked to an increased risk of developing CHE.
- Liver disease: Liver disease, such as cirrhosis, has been linked to an increased risk of developing CHE.
- Heart disease: Heart disease, such as coronary artery disease, has been linked to an increased risk of developing CHE.
- Smoking: Smoking has been linked to an increased risk of developing CHE.
Symptoms
Symptoms of CHE in detail.
- Abdominal pain: CHE in the liver can cause abdominal pain, which may be constant or intermittent. The pain can range from mild to severe.
- Enlarged liver: CHE can cause the liver to enlarge, which can be felt by a doctor during a physical examination.
- Fatigue: CHE can cause fatigue, which can be debilitating and affect a person’s daily activities.
- Weight loss: CHE can cause weight loss, which can be significant and unexplained.
- Loss of appetite: CHE can cause a loss of appetite, which can contribute to weight loss and malnutrition.
- Nausea and vomiting: CHE can cause nausea and vomiting, which can be intermittent or persistent.
- Jaundice: CHE in the liver can cause jaundice, which is a yellowing of the skin and whites of the eyes.
- Itching: CHE in the liver can cause itching, which can be severe and persistent.
- Ascites: CHE can cause the accumulation of fluid in the abdominal cavity, known as ascites.
- Edema: CHE can cause swelling in the legs and feet, known as edema.
- Shortness of breath: CHE can cause shortness of breath, which can be mild to severe.
- Chest pain: CHE can cause chest pain, which may be sharp or dull.
- Cough: CHE can cause a persistent cough, which may be dry or productive.
- Bone pain: CHE can spread to the bones, causing bone pain.
- Headaches: CHE can cause headaches, which can be mild to severe.
- Seizures: CHE can cause seizures, which can be a result of the spread of the cancer to the brain.
- Neurological symptoms: CHE can cause a range of neurological symptoms, including weakness, numbness, and tingling.
- Skin lesions: CHE can cause skin lesions, which can be red or purple in color.
- Fever: CHE can cause a low-grade fever, which may be persistent or intermittent.
- Night sweats: CHE can cause night sweats, which can be severe and affect a person’s sleep.
Diagnosis
Most common diagnoses and tests used for composite hemangioendothelioma.
- Biopsy: The most common diagnostic test for composite hemangioendothelioma is a biopsy. This involves the removal of a small piece of tissue from the affected area, which is then examined under a microscope.
- Immunohistochemistry: Immunohistochemistry is a technique used to identify specific proteins in tissue samples. This test can help to confirm the diagnosis of composite hemangioendothelioma by identifying the presence of certain markers that are characteristic of this type of tumor.
- MRI: Magnetic resonance imaging (MRI) is a non-invasive imaging test that uses a magnetic field and radio waves to produce detailed images of the body. This test can help to identify the location and extent of the tumor.
- CT scan: A computed tomography (CT) scan is another imaging test that can be used to identify the location and extent of the tumor. This test uses X-rays and computer technology to produce detailed images of the body.
- PET scan: Positron emission tomography (PET) scan is an imaging test that can help to identify areas of the body that are affected by cancer. This test involves the injection of a small amount of radioactive material into the body, which is then detected by a scanner.
- Ultrasound: Ultrasound is a non-invasive imaging test that uses high-frequency sound waves to produce images of the body. This test can help to identify the location and size of the tumor.
- Fine-needle aspiration (FNA): Fine-needle aspiration is a procedure that involves the insertion of a thin needle into the tumor to collect a small sample of cells for examination. This test can help to confirm the diagnosis of composite hemangioendothelioma.
- Core biopsy: Core biopsy is a procedure that involves the removal of a small cylinder of tissue from the tumor for examination. This test can help to identify the specific type of cells that are present in the tumor.
- Surgical excision: Surgical excision is a procedure that involves the removal of the entire tumor for examination. This test can help to determine the extent of the tumor and whether it has spread to other parts of the body.
- Immunofluorescence: Immunofluorescence is a technique used to identify specific proteins in tissue samples. This test can help to confirm the diagnosis of composite hemangioendothelioma by identifying the presence of certain markers that are characteristic of this type of tumor.
- Flow cytometry: Flow cytometry is a technique used to analyze the physical and chemical characteristics of cells in a tissue sample. This test can help to identify the specific type of cells that are present in the tumor.
- Electron microscopy: Electron microscopy is a technique used to examine tissue samples at a very high magnification. This test can help to identify the specific type of cells that are present in the tumor.
- Genetic testing: Genetic testing is a test that can be used to identify changes in the DNA of cells that may contribute to the development of the tumor. This test can help to determine the risk of developing composite hemangioendothelioma in family members.
- Blood tests: Blood tests can be used to measure levels of certain proteins that may be elevated in people with
Treatment
Treatments for composite hemangioendothelioma in detail.
- Surgery – Surgery is the most common treatment option for composite hemangioendothelioma. It involves removing the tumors and surrounding tissues. The type of surgery depends on the location and size of the tumors.
- Radiation Therapy – Radiation therapy uses high-energy radiation to kill cancer cells. It can be used as a primary treatment for small tumors or as an adjuvant therapy after surgery to kill any remaining cancer cells.
- Chemotherapy – Chemotherapy uses drugs to kill cancer cells. It is usually used in combination with other treatments for composite hemangioendothelioma. The drugs are delivered either orally or intravenously.
- Immunotherapy – Immunotherapy is a type of treatment that stimulates the immune system to fight cancer cells. It can be used as a primary treatment for small tumors or as an adjuvant therapy after surgery.
- Targeted Therapy – Targeted therapy uses drugs that target specific molecules or pathways that are involved in the growth and spread of cancer cells. It can be used as a primary treatment for small tumors or as an adjuvant therapy after surgery.
- Embolization – Embolization is a minimally invasive procedure that involves blocking the blood vessels that supply the tumors with blood. This cuts off the blood supply to the tumors, causing them to shrink or die.
- Ablation Therapy – Ablation therapy uses heat or cold to destroy cancer cells. It can be used as a primary treatment for small tumors or as an adjuvant therapy after surgery.
- Cryotherapy – Cryotherapy is a type of ablation therapy that uses extreme cold to freeze and destroy cancer cells.
- Laser Therapy – Laser therapy uses high-energy light to destroy cancer cells. It can be used as a primary treatment for small tumors or as an adjuvant therapy after surgery.
- Photodynamic Therapy – Photodynamic therapy uses a special drug that makes cancer cells sensitive to light. The drug is then activated by a special light, which destroys the cancer cells.
- Radiofrequency Ablation – Radiofrequency ablation uses high-frequency electrical energy to destroy cancer cells. It can be used as a primary treatment for small tumors or as an adjuvant therapy after surgery.
- Watchful Waiting – Watchful waiting is a form of active surveillance where the patient is closely monitored for any changes in the tumors. This approach is usually taken for small, low-grade tumors that are not causing any symptoms.
- Chemoradiation Therapy – Chemoradiation therapy combines chemotherapy and radiation therapy to kill cancer cells. It is usually used as a primary treatment for large tumors or as an adjuvant therapy after surgery.
- Hormone Therapy – Hormone therapy is a type of targeted therapy that uses drugs to block the hormones that stimulate the growth of certain types of cancer cells.
- Stem Cell Transplant – Stem cell transplant involves replacing the patient’s bone marrow with healthy stem cells. This can be used as a treatment for some types of cancer, including composite hemangioendothelioma.
- Cryosurgery – Cryosurgery uses extreme cold to freeze and destroy cancer cells. It can be used as a primary treatment for small tumors or as an adjuvant therapy after surgery.
Medications
Drugs that are commonly used to treat composite hemangioendothelioma and their details.
- Sirolimus
Sirolimus is an immunosuppressive drug that is commonly used to prevent rejection after organ transplantation. It has also been found to be effective in treating composite hemangioendothelioma by inhibiting the growth of blood vessels. Sirolimus works by targeting a protein called mTOR, which is involved in the growth and proliferation of cells. By inhibiting mTOR, sirolimus can slow the growth of the tumor.
- Bevacizumab
Bevacizumab is a monoclonal antibody that targets vascular endothelial growth factor (VEGF). VEGF is a protein that stimulates the growth of blood vessels, and it is often overexpressed in composite hemangioendothelioma. By blocking VEGF, bevacizumab can slow the growth of the tumor and improve symptoms.
- Pazopanib
Pazopanib is a tyrosine kinase inhibitor that targets several receptors involved in the growth and proliferation of cells. It has been found to be effective in treating composite hemangioendothelioma by inhibiting the growth of blood vessels. Pazopanib is usually used to treat advanced renal cell carcinoma but has also been found to be effective in other types of tumors.
- Sorafenib
Sorafenib is another tyrosine kinase inhibitor that targets several receptors involved in the growth and proliferation of cells. It has been found to be effective in treating composite hemangioendothelioma by inhibiting the growth of blood vessels. Sorafenib is usually used to treat advanced liver cancer but has also been found to be effective in other types of tumors.
- Sunitinib
Sunitinib is a tyrosine kinase inhibitor that targets several receptors involved in the growth and proliferation of cells. It has been found to be effective in treating composite hemangioendothelioma by inhibiting the growth of blood vessels. Sunitinib is usually used to treat advanced kidney cancer but has also been found to be effective in other types of tumors.
- Everolimus
Everolimus is an immunosuppressive drug that is similar to sirolimus. It works by targeting mTOR, which is involved in the growth and proliferation of cells. By inhibiting mTOR, everolimus can slow the growth of the tumor.
- Interferon alpha
Interferon alpha is a protein that is naturally produced by the immune system to fight viruses and cancer. It has been found to be effective in treating composite hemangioendothelioma by inhibiting the growth of blood vessels and stimulating the immune system. Interferon alpha is usually given as an injection.
- Imatinib
Imatinib is a tyrosine kinase inhibitor that targets a protein called BCR-ABL, which is involved in the growth and proliferation of cells. It has been found to be effective in treating composite hemangioendothelioma by inhibiting the growth of blood vessels. Imatinib is usually used to treat chronic myeloid leukemia but has also been found to be effective in other types of tumors.
