A lymphangioma is a benign vascular malformation. It is a tumor disease of the lymphatic vessels, which occurs rather rarely.
What is a lymphangioma?
Lymphangiomas owe their name to their place of origin, the lymph vessels. The counterpart of the vascular malformations in the blood system are the so-called haemangiomas. Lymphangiomas are divided into three different classes:
- Cavernous lymphangiomas ( Lymphangioma cavernosum ) usually appear on the face, under the armpits, on the arms or legs.
- The capillary lymphangioma ( lymphangioma circumscriptum ) is mainly found in the skin and mucous membrane area of the mouth and lips. This form can also appear in the genital area or in the groin area.
- A vascular malformation in one or more sites of the neck, neck, armpits, or mediastinum is most likely to be a cystic lymphangioma. These are also known as lymphangioma cysticum or hygroma cysticum colli.
A fourth form is also occasionally described in the medical literature. This lymphangiomatous gigantism is similar in appearance to elephantiasis, an abnormal enlargement of a body part caused by lymphatic congestion. See AbbreviationFinder for abbreviations related to Lymphangiom.
Causes
The exact origin of the lymphangiomas is still unclear. The vascular malformations are probably based on an inherited malformation of the lymphatic system. The foundations for the subsequent illness are probably laid in the second month of pregnancy. In the mesoderm, the middle germ layer, an incorrect differentiation then takes place in the affected patients. Apparently, the lymphangiomas arise from bulges in the venous and lymphatic systems.
The neck vein, the two pelvic veins and the mesenteric vein in the lower abdomen are particularly affected. Connections are normally formed between the lymphatic system and the venous system so that the lymph can flow into the veins. In patients with lymphangiomas, however, these connections are sometimes missing. As a result, the lymph cannot drain away and the lymphatic vessels expand.
This is also known as lymphangiectasia. The tumor that then forms in these extensions consists of various cyst-like tissue structures. These are filled with a protein-like fluid containing many eosinophilic granulocytes.
Symptoms, Ailments & Signs
Lymphangiomas usually appear for the first time in early childhood. In almost 90 percent of all cases, the vascular malformations appear immediately after birth, but by the age of five at the latest.
In most cases, the tumor is found on the neck or neck. In 20 percent of the cases it is located under the armpits. Other parts of the body are rarely affected. However, the benign tumors can also occur in the mediastinum, on the pleura, on the pericardium, on the vulva, on the penis, in the groin region, in the bones, on the pancreas, on the ovaries or in the entire remaining abdomen.
In most cases, lymphangiomas are light-colored or skin-colored. However, bleeding is often found in the cavities of the cavernous lymphangioma. In these places, the lymphangioma then becomes darker and appears bluish to purple. While the capillary lymphangioma is more likely to appear in the form of small vesicles, the cystic hygroma and especially the lymphangiomatous gigantism can cause larger tumors.
Depending on their size and location, the vascular malformations can also displace other organs and cause the corresponding symptoms of displacement. For example, narrowing of the airways can lead to breathing disorders, swallowing difficulties or a forced posture of the head.
Diagnosis & course of disease
At the beginning of the diagnosis there is always a careful collection of the medical history. This part of the diagnosis is also referred to as anamnesis. The physical examination follows. However, in order to be able to reliably differentiate the lymphangioma from other anomalies of the vessels, an ultrasound examination (sonography) must be carried out. The ultrasound also checks whether other organ or tissue structures are being constricted or displaced due to the position of the lymphangioma. Magnetic resonance imaging (MRI) may be needed for internal lymphangiomas.
Complications
The lymphangioma causes very serious malformations in the patient, which mainly occur in the vessels and can significantly restrict the patient’s everyday life and quality of life. In many cases, those affected suffer from bleeding and thus discoloration of the skin. Furthermore, tumors and growths form, which in a very unfavorable case can also spread to other regions of the body.
The malformations in the vessels can have a negative effect on the organs and possibly displace them. This can lead to breathing disorders or swallowing difficulties. It is not uncommon for the swallowing difficulties to result in difficulties in taking food or liquids. In the worst case, the breathing difficulties can also lead to a loss of consciousness.
It is not uncommon for a lymphangioma to lead to a misaligned head position, resulting in tension or other unpleasant symptoms. Lymphangioma is treated by removing the tumor. Furthermore, those affected are dependent on various therapies even after the removal. However, a positive course of the disease cannot always be guaranteed.
When should you go to the doctor?
Lymphangiomas must be diagnosed and treated promptly. Otherwise, the growths can lead to deformities, chronic pain and other complications. Parents who discover a swelling or lump in their child are best informed by the pediatrician. General symptoms such as fever, loss of appetite or breathing problems can also indicate a vascular malformation and should be examined. Normally, lymphangiomas are unproblematic as long as they are recognized and treated early. Diseased children must also be examined regularly after the removal of the malformation, as there is an increased risk of recurrence.
If a parent has a malformation of the lymphatic system, the child should be examined routinely. Since it is a hereditary disease, a targeted diagnosis is possible, often in the womb. Lymphangiomas are diagnosed and treated by an internist or lymphologist. Your general practitioner can make an initial suspected diagnosis. If the growth is in the area of the head, spine or limbs, the patient must also be treated with physiotherapy in order to preserve or restore essential bodily functions.
Treatment & Therapy
In contrast to haemangiomas, lymphangiomas usually do not regress spontaneously. In most cases, the vascular tumors are completely removed. This complete removal of the tumor is also known as extirpation. For a complete cure, the tumor must really be completely removed. Otherwise, recurrences will always occur. However, only about a third of all lymphangiomas can be completely removed in the first operation.
Tiny blisters often remain along the nerves or organs. They then later refill with fluid and form a new lymphangioma. Laser therapy is often used as an alternative or in combination with surgery. The advantage of laser treatment is that the procedure is not as stressful as an operation and only small scars remain. However, multiple treatments are usually required for complete removal.
Drug therapies are usually unsuccessful. Only sclerotherapy with Picibanil is suitable for drug treatment. A specially treated strain of bacteria (Streptococcus pyogenes) is repeatedly injected into the lymphangioma. The procedure is used when the risk of an operation is too great. If organs are affected by the lymphangioma, it may also be necessary to insert a gastric tube or a tracheostomy.
In principle, however, the prognosis of the lymphangioma can be considered good. It is a benign tumor, so complete removal means complete healing. Mortality is about three percent.
Outlook & Forecast
The extent of the malformation is ultimately decisive for the prognosis of a lymphangioma. A good prognosis is made for a large number of patients, since the available therapy options with current scenic knowledge are often sufficient. Slight changes in the blood vessels can lead to a lifelong freedom from symptoms. In these cases, the diagnosis is often only made possible by an incidental finding. Nevertheless, no spontaneous healing of the disease is to be expected, since the malformations remain for life. There is no treatment option that leads to a complete recovery and thus to the elimination of the malformations.
Normally, the tissue changes and the vessels must be treated surgically. Tumors develop that have to be removed to prevent further complications. Laser therapy is often used to minimize scarring. If the interventions proceed without further disturbances, a significant alleviation of the symptoms can be expected. Nevertheless, checks should be carried out at regular intervals throughout life in order to check the functional activity of the vessels.
Despite all efforts, lifestyle choices are limited in the case of a lymphangioma. The physical resilience should be adapted to the requirements of the organism and overexertion should be avoided. With this disease, there are always risks for the development of acute situations, which must be minimized.
Prevention
Since the exact causes of the lymphangioma are still unclear, the disease cannot be prevented.
Aftercare
The type and duration of aftercare depends on the location and treatment of the lymphangioma. Follow-up measures are often not necessary. If the benign tumor was surgically removed, the healing of the surgical scars should be observed. If there are postoperative complications, intensive follow-up care is necessary.
As a rule, after successful removal, several check-ups are carried out to determine whether a lymphangioma will recur. In some areas of the body, such as the chest area, those affected can determine this themselves through regular palpation. Nevertheless, regular examinations by a doctor are also advisable in these cases.
The exact interval of the checks is determined by the respective specialist. As soon as those affected notice changes again, they should therefore consult a specialist, regardless of the agreed check-up intervals. Those affected should be closely monitored in the event of recurring abnormalities.
You can do that yourself
The lymphangioma has not yet been fully researched medically. For this reason, the possibilities of self-help are not sufficiently known. Direct sunlight should be avoided to protect against further skin irritation. Likewise, cosmetic products should only be selected and used in consultation with a doctor. It is important to ensure that the benign tumors on the face or neck are not accidentally scratched, as this can lead to inflammation. In addition, if the mucous membranes in the mouth are infected, you should refrain from consuming acidic or spicy foods.
Patients and relatives can take advantage of an exchange with other affected people in self-help groups and forums. Everyday tips and information on how to deal with the disease in everyday life are discussed in a trusting atmosphere. This stabilizes the mental state of many of those affected, reduces fears and leads to clarification of open questions. Recent experiences are discussed with one another in this way and can help to improve general well-being.
Regular massages help to relieve tension. Depending on the location and extent, the patient himself or with the help of relatives can prevent or solve the muscle problems with circular movements. Sufficient and continuous heat supply is also beneficial for the shoulders and neck area.
