Hepatic Lymphangioma - A Benign Tumour of the Liver

Introduction

Lymphangioma is a non-cancerous fluid-filled under the skin caused by overgrown lymph vessels. They are a category of benign tumors. Lymph vessels move white blood cells and lymphatic fluid through the blood and the tissues. The common sites of lymphangioma are the head and neck, but they can form in any part of the body. It usually affects infants between birth and 5 years.

What Are Lymphangiomas?

• Lymphangiomas are vascular tumor-like conditions. Lymphangiomas are acquired and developmental tumor-like conditions of the vascular system (blood circulating system). Lymphangiomas are endothelial cells (blood cells) lined in capillary channels filled with lymphatic fluid ( a clear fluid that carries cells made up of white blood cells, lymphocytes that fight against infections).

• They are tumor-like conditions in which some tissues are jumbled up. They are endothelial cells lined channels with lymphatic fluid in their channels.

• The lymphangiomas are histologically classified into three main types- capillary, cystic, and cavernous.

• They are most commonly found in the head, neck, and axillae, where the loose connective tissue allows easy expansion of lymphatic channels.

• Intraabdominal cases account for less than five percent of all lymphangiomas. Small lymphatic channels are present subcutaneously (1-2 cm). Clinically they look like fluid-filled blisters. The fluid component of lymphangiomas can be serous (pale yellow or transparent) depending on the location. In the presence of hemorrhage, it can become bloody or purulent.

• Cavernous lymphangiomas are present in the base of the neck. They look like humps and are sometimes present in the axilla. They are very large lymphatic-filled endothelial lined spaces. They are also called cystic hygroma. They are massively dilated, and their margins are not well-defined.

Cavernous lymphangiomas are associated with turner’s syndrome. The condition occurs in children with genetic conditions such as:

• Down syndrome (Chromosome 21 genetic disorder that causes developmental and intellectual delays).

• Trisomy 18 (a condition that causes severe developmental delays due to an extra chromosome 18).

• Trisomy 13 (A condition in which the person has extra chromosome 13).

• Trisomy 21 (a genetic chromosome 21 disorder causing developmental and intellectual delays).

• Turner syndrome (only one X chromosome is present in the female).

• Noonan syndrome (a genetic condition that affects various parts of the body).

What Are Hepatic Lymphangiomas?

• Hepatic lymphangiomas are rare congenital tumors that occur in young adults and children. They occur mainly in abdominal regions.

• Hepatic lymphangioma is a disease associated with the liver.

• Hepatic lymphangiomas are rare benign neoplasia characterized by congenital cystic dilatation of lymphatic vessels in the liver cells.

• Solitary hepatic lymphangioma is rare and can cause a clinical misinterpretation as a malignant tumor. Hepatic lymphangiomas are extremely rare.

• In most cases reported, the hepatic lesion is part of a diffuse involvement of multiple organs, including the liver, spleen, kidneys, skeleton, gastrointestinal tract, mesentery, mediastinum, lungs, pleura, pericardium, as well as the somatic soft tissues.

• The condition starts with epigastric pain. The etiology of lymphangiomas is unclear.

• Dysplasia of lymphatic tissue and abnormal development of lymph vessels are the mechanism for obstruction of lymphatic channels due to inflammation or trauma.

• Hepatic lymphangiomas are a subset of many abdominal lymphangiomas. A similar abnormality in other organs, including kidneys, spleen, lungs, gastrointestinal tract, and soft tissues, usually accompanies it.

• These are usually asymptomatic. Abdominal pain is noticed. There is no bleeding associated with cystic lymphangioma of the liver. Lympahngiomas carry the risk of malignant transformation.

What Are the Causes of Hepatic Lymphangioma?

The exact cause is unknown. It is due to abnormal development of the child’s lymphatic system during fetal development. The lymphatic system contains lymphatic fluid that contains white blood cells that pass through the bloodstream and tissues. It helps the immune system function properly. The fluid in the lymph vessel forms a pool of lymph fluid. The fluid pool appears as a liquid-filled bump on the skin(cyst). It occurs when the lymph backs up in the lymph vessels surrounding the body's tissues. They are not apparent at birth but can be more noticeable when the children grow older. Congenital developmental abnormality of the lymphatic tissues is the main cause. Localized lymphatic obstruction and dilatation o the abnormal channels are some of the causes. The other causes are vascular endothelial permeability disorders that include inflammatory processes.

What Is the Diagnosis of Hepatic Lymphangioma?

Most hepatic lymphangiomas are detected during routine physical examinations. An abdominal CT (computed tomography) or MRI (magnetic resource imaging) is used to help differentiate it from similar lesions. The abdominal CT shows a poorly limited, normal-sized liver. The diagnosis can be made before birth with an ultrasound exam. The lymphangioma becomes more noticeable by the age of 2, if the lymphangioma is not visually noticeable at birth.

What Is the Treatment for Hepatic Lymphangioma?

The treatment will be necessary if it prevents the child’s moving or is blocking the liver's function. The lymphangiomas tend to reoccur. Multiple surgeries or treatments may require to manage this condition. Reoccurrence rates vary according to the type of lymphangioma. Many lymphangiomas cause no health problems. The treatment options include:

1. Sclerotherapy- In this therapy, a chemical is injected into the mass, which shrinks and collapses. It has more success rate and fewer complications.

2. Laser Therapy or Radiofrequency Ablation- A needle or laser delivers the current to destroy the mass.

3. Surgery- The cyst is cut out. It becomes difficult if it is closely associated with the nerves and organs.

What Are the Complications of the Treatment?

The cyst can return after the treatment because removing all the cells that caused it to grow is difficult. The cysts are large and deep and are difficult to remove and grow back after the treatment. There is a risk of infection after the surgery. This would complicate the process of healing. Taking care of the surgical site while operating is essential for the best outcome. There is no prevention for lymphangiomas since it is due to an abnormal lymphatic system that happens when the child is growing.

Conclusion

The majority of lymphangiomas are non-life threatening. Since hepatic lymphangiomas are associated with a vital organ, they can cause the organs not to work effectively. It is always better to reach out to a doctor when the child has abdominal pain or changes in movement. Lympahgiomas result from genetic changes, so it is better to do genetic testing to understand the risk of having a child if a genetic condition.