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Mysteries of the lymphatic system: How do lymphatic malformations develop?
Lymphatic malformations are benign, slow-flowing vascular malformations of the lymphatic system characterized by the loss of lymphatic vessels to connect with the normal lymphatic circulation. Recent studies have updated this term to lymphoid malformation, replacing the outdated term lymphoma. Depending on the size and number of their cysts, lymphatic malformations can be classified as macrocystic, microcystic, or a combination of both. Large cystic lymphatic malformations have cysts that are larger than 2 cubic centimeters, while small cystic lymphatic malformations have cysts that are smaller than 2 cubic centimeters.
According to statistics, 90% of lymphatic malformations occur in children under 2 years old and are mainly concentrated in the head and neck area.
These deformities can be either congenital or acquired. Congenital lymphomas are often associated with chromosomal abnormalities (such as Turner syndrome), although they may also occur independently. Many lymphomas can be diagnosed before birth through fetal ultrasound, while acquired lymphomas may result from trauma, inflammation, or lymphatic obstruction.
Most lymphomas are benign and cause little more than a "soft" mass that grows slowly. Because they do not change into malignant tumors, lymphomas are usually treated for cosmetic reasons only. Although rare, lymphoma can cause complications, such as difficulty breathing due to airway compression, when it puts pressure on vital organs.
Clinical manifestations and causes
There are three distinct types of lymphatic malformations, each with its own unique symptoms depending on the depth and size of the abnormal lymph vessels. Lymphoma donut-shaped can be found on the surface of the skin, while the other two types usually occur just beneath the skin. Along with different types of lymphoma, they also vary in color and size, from pink to dark red, white, blue, purple, and even black.
"The secret cause of lymphatic malformation is still unclear. Is it a genetic error or a coincidence in the development process?"
The direct cause of lymphatic malformation is the obstruction of the lymphatic system during embryonic development, but the specific cause remains unknown. Studies have shown that cystic lymphoma occurring during the first and second trimesters is associated with some genetic abnormalities. These include Noonan syndrome and trisomy of chromosomes 13, 18, and 21. About 40% of patients with cystic lymphoma may also have chromosomal aneuploidies.
Diagnosis and treatment options
The diagnosis of lymphatic malformations mainly relies on histopathological examination. Prenatally, lymphatic malformations are usually diagnosed by ultrasonography during the late 1st or early 2nd trimester. Other imaging tests such as CT and MRI scans can help with treatment planning and clearly show the size of the lesion and the vital structures around it.
"With the advancement of imaging technology, the diagnosis of many lymphatic malformations has become increasingly convenient."
Treatment strategies often include surgical excision, but complete removal may not be easy, especially without damaging surrounding normal tissue. Many patients require at least two surgeries during their treatment, and recurrence is possible but unlikely in most cases. In recent years, non-invasive treatments such as microdialysis or injection of sclerosing agents have gradually gained attention. Sirolimus is used to treat lymphoma and has been shown to reduce pain and slow the growth of abnormal lymph vessels.
Looking to the future
The prognosis of lymphoid malformations is generally good, especially lymphoma annulare and cavernous lymphoma. With cystic lymphoma, large cysts may cause difficulty swallowing and breathing problems. Therefore, such patients should undergo cytogenetic analysis to determine whether there is a staining abnormality, and their parents should be offered genetic counseling.
"For future treatment options, how to more effectively avoid the potential problems caused by lymphatic malformations will be an important issue in the medical community."
Epidemiological data on lymphatic malformations show that they occur in approximately 4% of children. Although lymphoma can manifest at any age, 50% are detected at birth and 90% of cases are present before the age of 2 years. As our understanding of the lymphatic system grows, can we continue to explore solutions to this mysterious phenomenon?
