A lymphangioma is a benign vascular malformation. It is a tumor disease of the lymphatic vessels that occurs rather rarely.
What is a lymphangioma?
Lymphangiomas owe their name to their site of origin, the lymphatic vessels. The counterpart of vascular malformations in the blood system are the so-called hemangiomas. Lymphangiomas are divided into three different classes:
- Cavernous lymphangiomas (lymphangioma cavernosum) usually occur on the face, under the armpits, arms or legs.
- Capillary lymphangioma (lymphangioma circumscriptum) is found mainly in the skin and mucous membrane of the mouth and lips. This form may also appear in the genital area or in the groin.
- Vascular malformations in one or more sites of the neck, throat, axillae, or mediastinum are most likely to be cystic lymphangiomas. These are also known as lymphangioma cysticum or hygroma cysticum colli.
Occasionally, a fourth form is also 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.
Causes
The exact origin of lymphangiomas is still unclear. Presumably, the vascular malformations are based on an inherited malformation of the lymphatic system. The foundations for the later disease are probably laid as early as the second month of pregnancy. In the mesoderm, the middle germ layer, a misdifferentiation then takes place in the affected patients. Apparently, the lymphangiomas arise from outpouchings of the venous and lymphatic system. The jugular vein, the two iliac veins and the mesenteric vein in the lower abdomen are particularly affected. Normally, connections are formed between the lymphatic system and the venous system to allow lymph to drain into the veins. However, in patients with lymphangiomas, these connections are partially absent. As a result, the lymph cannot drain and dilatation of the lymphatic vessels occurs. This is also known as lymphangiectasia. The tumor that then forms in these dilations consists of various cyst-like tissue structures. These are filled with a proteinaceous fluid that contains many eosinophilic granulocytes.
Symptoms, complaints, and signs
Lymphangioma usually first appears in early childhood. In nearly 90 percent of all cases, the vascular malformations show up immediately after birth, but no later than by the age of five. In most cases, the tumor is found on the neck or in the nape of the neck. In 20 percent of 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, pleura, pericardium, vulva, penis, groin, bones, pancreas, ovaries or throughout the rest of the abdomen. In most cases, lymphangiomas are light colored or skin colored. However, hemorrhages are often found in the cavities of the cavernous lymphangioma. In these areas, the lymphangioma then becomes darker and appears bluish to purple. While capillary lymphangioma tends to present as small vesicles, cystic hygroma and especially lymphangiomatous gigantism can cause larger growths. Depending on their size and location, the vascular malformations may also displace other organs and cause corresponding displacement symptoms. For example, narrowing of the airway may cause breathing difficulties, difficulty swallowing, or forced posture of the head.
Diagnosis and course of the disease
Diagnosis always begins with a careful taking of the patient’s medical history. This part of the diagnostic process is also called the medical history. This is followed by a physical examination. However, to reliably differentiate lymphangioma from other abnormalities of the vessels, an ultrasound examination (sonography) must be performed. The ultrasound also checks whether other organ or tissue structures are constricted or displaced by the location of the lymphangioma. In the case of lymphangiomas inside the body, magnetic resonance imaging (MRI) may be required.
Complications
The lymphangioma causes very serious malformations in the patient, which occur mainly in the vessels and can significantly restrict the daily life and quality of life of the affected person. In many cases, the affected person suffers from hemorrhages and thus from a discoloration of the skin. Furthermore, tumors and growths also form, which in a very unfavorable case can also spread to other regions of the body. The malformations on the vessels can have a negative effect on the organs and possibly displace them. This can lead to respiratory problems or swallowing difficulties. The swallowing difficulties often cause 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 lymphangioma to lead to poor posture of the head, resulting in tension or other uncomfortable symptoms. Lymphangioma is treated by removing the tumor. Furthermore, the affected persons are dependent on various therapies even after the removal. However, a positive course of the disease cannot always be guaranteed.
When should one go to the doctor?
Lymphangiomas must be diagnosed and treated promptly. Otherwise, the growths can lead to malformations, chronic pain and further complications. Parents who discover a swelling or lump in their child are best to inform the pediatrician. General symptoms such as fever, loss of appetite or breathing problems may also indicate a vascular malformation and should be investigated. Lymphangiomas are normally unproblematic, provided they are recognized and treated at an early stage. Diseased children must be examined regularly even after the malformation has been removed, as there is an increased risk of recurrence. If one parent has malformations of the lymphatic system, the child should be examined routinely. Because it is a hereditary disease, a specific diagnosis is possible, often while the child is still in the womb. Lymphangiomas are diagnosed and treated by an internist or lymphologist. An initial tentative diagnosis can be made by the family physician. If the growth is located in the head, spine, or limbs, the patient must also receive physical therapy to preserve or restore essential body functions.
Treatment and therapy
Unlike hemangiomas, lymphangiomas do not usually regress spontaneously. In most cases, the vascular tumors are completely removed. This complete removal of the tumor is also called extirpation. For complete healing, the tumor really must be completely removed. Otherwise, recurrences will always occur. However, only about one third of all lymphangiomas can be completely removed directly during the first operation. Tiny vesicles often remain along the nerves or organs. They then fill with fluid again later 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 surgery and also only small scars remain. However, several treatments are usually necessary for complete removal. Drug therapies usually remain unsuccessful. Only sclerotherapy with Picibanil is suitable for medical treatment. In this procedure, a specially treated strain of bacteria (Streptococcus pyogenes) is repeatedly injected into the lymphangioma. The procedure is used when the risk of surgery is too great. If organs are affected by the lymphangioma, the insertion of a stomach tube or a tracheostomy may also be necessary. In principle, however, the prognosis of lymphangioma can be considered good. It is a benign tumor, so complete removal also means complete cure. The mortality rate is about three percent.
Outlook and prognosis
The extent of malformations ultimately determines the prognosis for lymphangioma. A good prognosis is given in a large number of patients because the available options for therapy are often sufficient with current scenic knowledge. In the case of mild changes in the vessels, there may be a lifelong absence of symptoms. Frequently, in these cases, the diagnosis is made possible only 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 an elimination of the malformations. Normally, the tissue changes as well as the vessels have to be treated surgically. Tumors develop and must be removed to prevent further complications. Laser therapy is often used to minimize scarring. If the interventions proceed without further disturbances, a significant relief of the symptoms can be expected. Nevertheless, controls should take place at regular intervals throughout life to check the functional activity of the vessels. Despite all efforts, lifestyle management is limited in the case of lymphangioma. The physical capacity should be adapted to the organism’s specifications and overexertion should be avoided. There are always risks for the development of acute situations in this disease, which should be minimized.
Prevention
Since the exact causes of lymphangioma are still unclear, it is not possible to prevent the disease.
Aftercare
The type and duration of follow-up care depends on the location and treatment of the lymphangioma. Often, no aftercare measures are necessary. If the benign tumor was surgically removed, healing of the surgical scars should be monitored. If postoperative complications occur, intensive follow-up care becomes necessary. As a rule, after successful removal, several control examinations are performed to determine whether a lymphangioma has recurred. In some areas of the body, such as the breast, patients can detect this themselves by regular palpation. Nevertheless, regular examinations by a physician are also advisable in these cases. The exact interval of the controls 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. Affected persons should be closely monitored in the event of recurring abnormalities.
What you can do yourself
Lymphangioma has not been fully studied medically. For this reason, the options for self-help are not sufficiently known. To protect against further skin irritation, direct exposure to sunlight should be avoided. Likewise, cosmetic products should be selected and used only in consultation with the physician. Care should be taken not to scratch the benign tumors on the face or neck unintentionally, as this can lead to inflammation. In addition, if the mucous membranes in the mouth are affected, the consumption of acidic or spicy foods should be avoided. Patients as well as relatives can take advantage of self-help groups as well as forums to exchange information with other affected persons. Everyday tips and advice on dealing with the disease in everyday life are discussed in a trusting atmosphere. This stabilizes the psychological condition of many of those affected, alleviates fears and leads to clarification of open questions. Recent experiences are discussed with each other in this way and can help to improve general well-being. Regular massages help to ease tension. Depending on the location and extent, the patient himself or with the help of relatives can prevent and relieve muscle complaints through circular movements. For the shoulders and neck area, an adequate and continuous supply of heat is also beneficial.