Lymphangioma is a benign tumor, which is usually based on a congenital anomaly of the lymphatic system. Lesions can occur on any part of the body, they look like soft-tissue formations with a bumpy surface of red-brown or bluish color. Malformation often proceeds asymptomatically, being a cosmetic problem, but, reaching large sizes, gives rise to dangerous conditions. The basis of the diagnosis is a clinical examination confirmed by instrumental methods (ultrasound, MRI, lymphography). Sclerotherapy, radical surgery, minimally invasive techniques are used in the treatment.
ICD 10
D18.1 Lymphangioma of any localization
Meaning
Lymphangioma is a fairly rare pathology. According to various data, it accounts for 1.3–10.6% of cases of all vascular tumors (in childhood – up to 25%), occurs with a frequency of 1 per 20-250 thousand admissions to surgical hospitals. Lymphatic malformations occupy the second place among soft tissue formations of the maxillofacial region. They are usually detected in newborns and infants (90% of cases), less often in the first 2-3 years of a child’s life, but they can be diagnosed in utero or much later in adulthood. Gender statistics are contradictory: while some researchers report the predominant development of superficial lymphangiomas in women, others observe pathology 3 times more often among men.
Causes
Most of the formations are considered as congenital vascular malformations, and not true tumors. They usually occur in the fetus at the end of the first or at the beginning of the second trimester of intrauterine development. Although the origin of the pathology remains not fully understood, etiological factors may be:
- Anomalies of embryogenesis. The occurrence of pathology is associated with malformations of the embryonic development of the lymphatic system (dysontogenesis). The role of vascular endothelial growth factor (VEGF-C) and its receptors (VEGFR-3) affecting the proliferative activity of cells is indicated.
- Genomic and gene mutations. It was revealed that more than half of the cases of angiomas are associated with chromosomal aberrations in the fetus. Lymphangiomas are often observed in Shereshevsky-Turner, Down syndrome, trisomy 13, 18. Autosomal dominant inheritance is observed in Noonan syndrome.
- Intoxication. Impaired development of lymphatic vessels may be mediated by intrauterine toxic damage. Cases of tumors in children born to women who were exposed to lead or its compounds during pregnancy are described.
Secondary tumors are caused by impaired lymphatic drainage (superficial anomalies in lymphangitis, lymphogranuloma, panniculitis) associated with malrotation and intestinal inversion (intra-abdominal). Vascular malformations can develop after mechanical injury, in particular, against the background of Gorham-Stoat syndrome.
Pathogenesis
The mechanisms of lymphangioma development have not been studied well enough. Many researchers call the main pathological process the union of lymphatic cisterns in the deep subcutaneous space. They are separated from the normal vascular network, but communicate with the surface capillaries through vertical dilated channels. Presumably, cisterns arise from a primitive lymphatic sac, unable to unite with the rest of the lymphatic system in embryogenesis.
Hypertrophied muscle fibers lining sequestered areas support rhythmic contractions of the wall, which leads to an increase in pressure with dilation of the channels ascending to the skin. Fibrosis processes occur around the altered lymphatic vessels of the superficial and deep parts of the dermis, the epidermis undergoes acanthosis, papillomatosis, hyperkeratosis. Some variants of lymphangiomas are accompanied by pronounced infiltrative growth, but without tissue destruction. Tumors do not metastases.
Classification
Based on the localization, there are superficial (skin, subcutaneous tissue) and deep lymphangiomas. The former can affect almost any area, especially often involving the head, neck, armpits. Deep ones are found in internal organs (liver, spleen, kidneys) and body cavities (abdominal, retroperitoneal space, mediastinum). Pathomorphological classification of tumors is common in clinical angiology:
- Capillary (simple). They are formed when the lymphatic capillaries of the skin and subcutaneous tissues grow. Tumor-like foci are small, thin-walled, and cover limited areas.
- Cavernous. They are characterized by the expansion of rather large ducts with the formation of cavities and crevices filled with lymphatic fluid. They have a looped structure due to the multitude of connective tissue partitions, they resemble spongy tissue on the incision.
- Cystic (hygromas). They are cavities of various sizes isolated from adjacent lymphatic vessels. Filled with serous, chyletic or hemorrhagic contents. There are single or multiple, communicating with each other.
In addition, there are mixed tumors (lymphangiofibroma, lymphhemangioma). According to etiology, malformations are primary (congenital) and secondary (acquired). The prevalence criterion makes it possible to distinguish local and diffuse forms, and taking into account the effect on nearby tissues and organs, lymphangiomas can occur without visible disorders or be accompanied by obvious dysfunction and structural deformities.
Symptoms
The clinical picture is determined by the localization, histological structure, spread of the tumor, anatomical and topographic features of the involved areas, and the effectiveness of the treatment carried out earlier. Angiomatous foci are located in any area where there is lymphoid tissue. The face (lips, tongue, parotid-chewing area), trunk are often affected. But lymph nodes, internal organs, and bone tissue may be involved in the pathological process. The development of the tumor is slow, synchronous with the growth of the child, in most cases asymptomatic.
Capillary lymphangioma begins as a small gradually compacting spot. The formation has indistinct borders and a bumpy surface covered with small (2-4 mm in diameter) nodular elements of elastic consistency, colored pink-red. The area of the affected skin expands over time. The anomaly usually does not bring any other discomfort, except aesthetic. Sometimes lymph leaks out of the nodular elements, they bleed when traumatized. Increasing in volume and acquiring a diffuse character, the tumor presses on the underlying structures, which may be accompanied by bone deformities in children.
With cystic or cystic-cavernous lymphangioma of the face and neck, there is a limited swelling from 3 to 30 cm in diameter, facing outward or towards the oral cavity. The skin above it is of the usual color or with a cyanotic tinge, stretched, loose or soldered to the tumor mass. The surface of the lesion is smooth or bumpy, the consistency is soft, palpation is painless. The symptom of fluctuation is characteristic.
A feature of extensive lymphangiomas in the oral cavity, neck, mediastinum is periodically occurring inflammation. This is accompanied by fever, general weakness, the appearance of local symptoms – redness, swelling, sharp soreness of the affected area. The inflammatory process lasts 1-2 weeks and gradually subsides, making the tumor more dense.
Intraabdominal lymphangioma can give a picture of volumetric formation, accompanied by abdominal pain, nausea and anorexia, signs of intestinal obstruction. Symptoms of an “acute abdomen” are often mistaken for a sign of a more common surgical pathology. In some children, an asymmetric increase in the abdomen is noted with a palpable painless tumor, a satisfactory general condition. Lymphangiomas of small size are asymptomatic, being detected only by additional methods.
Complications
Despite its benign nature, lymphangioma is considered a potentially dangerous formation. Vascular anomalies of large size in the neck and head area of the fetus block its exit through the birth canal of the mother, squeeze and displace neighboring structures. Diffuse damage to the bottom of the oral cavity can cause respiratory disorders, up to asphyxia, inability to eat. Malformations of the orbital zone lead to visual disturbances, and localized in the abdominal cavity are accompanied by intestinal obstruction, sometimes torn.
Superficial lymphangiomas are easily injured and bleed, inflamed angiodysplasia in the cervical-facial area can become a source of serious infectious complications. A higher risk of developing epithelioid hemangioendothelioma and lymphangiosarcoma in patients has been reported. That is, a lymphatic anomaly is not just a cosmetic defect – it can lead to pronounced functional disorders and cause conditions that pose a real threat to life.
Diagnostics
It is not difficult to assume a superficial lymphangioma based on the characteristic clinical picture, the expiration of lymph when it is punctured. In diagnostically difficult cases, including anomalies of deep localization, the lymphologist needs data from instrumental imaging methods:
- Ultrasonography of the tumor. Ultrasound of superficial lymphangiomas reveals an increase in the volume of soft tissues, an increase in their acoustic density with multiple hypo- and anechoic areas of rounded or oval shape, combined into a single conglomerate. Echography determines not only the structure and localization of the tumor, but also the state of the surrounding formations.
- MRI. According to the results of MRI of soft tissues, it is possible to accurately determine the degree of lesion and morphological characteristics of vascular malformation. The results of the study help to form the right therapeutic tactics, avoiding unjustified surgical interventions.
- Lymphography. Allows you to examine the condition of lymphatic vessels, determine the size of the focus and its connection with neighboring organs. Lymphography is performed by injecting a water-soluble contrast agent directly into the tumor and performing a series of targeted X-ray images in two projections.
Dermatoscopy helps in the diagnosis of superficial limited lymphangioma, which determines nodules with a clear liquid and a pronounced lacunar pattern. With the accumulation of bloody contents, differences from hemangiomas help to establish histological examination, supplemented with immunohistochemical tests (with factor VIII antigen, laminin).
Lymphoid formations have to be differentiated from other tumors – teratoma, lipoma, neck cysts. Some malformations should be distinguished from congenital elephantiasis, spinal hernia, limited scleroderma. With intraabdominal lesion, it is necessary to exclude acute surgical pathology.
Treatment
None of the conservative therapy strategies has proven to be effective in lymphatic malformations. Only invasive techniques can ensure the reduction of the tumor mass or its complete removal. The method of correction is chosen based on the localization of lymphangioma, size, depth of occurrence, spread, and the presence of complications. The following options are acceptable:
- Sclerotherapy. The introduction of sclerosing substances (bleomycin, picibanil, ethoxysclerol) into the vascular anomaly leads to a reduction in its size. Sclerotherapy gives a good cosmetic result, and therefore is more preferable when the cervical-facial area is affected. It is indicated before surgery or if it is impossible to perform a complete resection.
- Radical excision. When removing a tumor focus, traditional surgical intervention is most appropriate. Radical excision is considered the method of choice for large cavernous formations and the need for urgent surgery. However, the removal of deep malformations is accompanied by a high risk of relapse.
- Minimally invasive methods. Treatment of superficial lymphangiomas can be carried out with a carbon dioxide laser, cryotherapy, electrocoagulation. They are less traumatic in comparison with traditional excision, they allow to reduce the time of rehabilitation. Sometimes just one procedure is enough to get a significant effect.
In the postoperative period, control studies are performed to assess the results obtained and the risk of relapse. Nonsteroidal anti-inflammatory and antibacterial agents are prescribed, compression therapy is used when tumors are localized on the lower extremities.
Prognosis and prevention
Lymphangiomas are benign lymphatic malformations, not true tumors. But acquiring large sizes, they are able to provoke functional disorders and lead to the development of life-threatening conditions. Timely correction makes the prognosis favorable, although the risk of relapse still exists. Given the predominantly congenital nature of the anomalies, primary prevention measures have not been developed. The probability of a recurrent course is reduced by the optimal choice of therapeutic tactics.
Literature
- Wygant C.M., Cohle S.D. Fatal Airway Obstruction in a Man With a Cystic Hygroma // Am J Forensic Med Pathol. — 2018; 39(3): 250-2.
- Clement C., Snoekx R., Ceulemans P., Wyn I., Mathei J. An acute presentation of pediatric mesenteric lymphangioma: a case report and literature overview // Acta Chir Belg. — 2018; 118(5): 331-5.
- Amodeo I., Colnaghi M., Raffaeli G., Cavallaro G., Ciralli F., Gangi S., et al. The use of sirolimus in the treatment of giant cystic lymphangioma: Four case reports and update of medical therapy // Medicine (Baltimore). — 2017; 96(51): e8871.
- Dvorakova V., Rea D., O’Regan G.M., Irvine A.D. Generalized lymphatic anomaly successfully treated with long-term, low-dose sirolimus // Pediatr Dermatol. — 2018; 35(4): 533-4.
- Horbach SER, van de Ven J.S., Nieuwkerk P.T., Spuls P.I., van der Horst C., Reekers J.A. Patient-Reported Outcomes of Bleomycin Sclerotherapy for Low-Flow Vascular Malformations and Predictors of Improvement // Cardiovasc Intervent Radiol. — 2018; 41(10): 1494-504.
- Khurana A., Gupta A., Ahuja A., Sardana K., Malhotra P. Lymphangioma circumscriptum treated with combination of Bleomycin sclerotherapy and Radiofrequency ablation // J Cosmet Laser Ther. — 2018: 1-4.
- Khanwalkar A., Carter J., Bhushan B., Rastatter J., Maddalozzo J. Thirty-day perioperative outcomes in resection of cervical lymphatic malformations // Int J Pediatr Otorhinolaryngol. — 2018; 106: 31-4.
