Oral Lymphangiomas

Updated: Apr 18, 2023
  • Author: Kruti Parikh; Chief Editor: William D James, MD  more...
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Overview

Practice Essentials

Lymphangiomas and cystic hygromas are rare benign hamartomatous lesions of the lymphatic system. Determining the true incidence of these lesions is difficult because uniformity in classification and nomenclature is lacking. Moreover, lymphatic malformations are generally treated in tertiary and quaternary medical centers, distorting any view of their true incidence. [1]

These lesions, more correctly referred to as lymphatic malformations, have a marked predilection for the head and neck, with as many as 75% presenting in the oral cavity. [2]  No clear sex predilection is demonstrated, and Whites appear to be affected more often than people of other races.

Also see Lymphangioma, Cervicofacial Lymphangiomas, and Arterial Vascular Malformations including Hemangiomas and Lymphangiomas.

Signs and Symptoms

The clinical appearance of the lymphatic malformations generally depends on the depth of extension. Superficial lymphatic malformations have a pebbly surface and are usually pink or yellow, with the appearance of grouped translucent vesicles. Some may also be bluish, which may be caused by either a venous component or an intralesional hemorrhage.

See the images below.

Superficial lymphatic malformation. Superficial lymphatic malformation.
Superficial lymphatic malformation. Superficial lymphatic malformation.

Deeper lesions may have a superficial component. When a superficial component is absent, deeper lesions usually appear as soft, ill-defined masses that markedly distort the local anatomy. In rare cases, lymphangioma circumscriptum can cause gingival enlargement. [3]  This condition can also present as firm, skin-colored labial papules. [4]

See the images below.

Marked lingual enlargement caused by lymphatic mal Marked lingual enlargement caused by lymphatic malformation. Note the pebbly surface in areas not covered by materia alba. Also note the ecchymotic lesions protruding from the buccal mucosa in the mandibular vestibules.
Note the significant left buccal and submandibular Note the significant left buccal and submandibular swelling.
Profile view of a young adult with oral lymphangio Profile view of a young adult with oral lymphangioma (same patient as in Media File 2).

Macrocystic lesions may be fluctuant and readily transilluminating. 

 

Also see Presentation.

Diagnostics

Superficial lesions consist of dilated lymph vessels lined by flat endothelial cells in a discontinuous layer immediately subjacent to the oral epithelium. These lesions often replace the dermal papillae, but they can also extend into the deeper connective tissue.

Deeper lesions consist of irregular, dilated, and interconnected lymphatic vessels, some of which reveal macroscopic cyst formation (eg, cystic hygroma, cystic lymphangioma, lymphangioma cystoides). The walls of the vessels variably contain smooth muscle bundles. Lymphoid aggregates and germinal centers can be observed in both the surrounding tissues and the walls of the lesion. Cystic spaces generally contain a lightly eosinophilic, proteinaceous fluid with scant lymphocytes.

In contrast to vascular malformations, electron microscopy and immunohistochemical techniques demonstrate irregular and fragmented basal lamina in the lymphatic malformations.

Oral lymphangiomas do not express Wilms tumor 1 protein (WT1), which is consistent with the finding that oral lymphangiomas are not true neoplasms. Negative WT1 staining implies a nonproliferative nature of the oral lymphangiomas, thus growth is most likely secondary to enlargement of lymphatic vessels and not neoplastic proliferation of lymphatic endothelial cells. [5]

Two oral lymphangioma cases showed the following on polarized dermoscopy: pinkish-to-red lacunae surrounded by pale septa on a yellowish background. Some of the lacunae exhibited hypopyon-like features. [6]

Also see Workup.

Management

The mainstay of medical management for oral lymphangiomas is observation. Small oral lymphatic malformations that are not associated with any functional sequelae may be observed. All patients require evaluation of airway patency, speech, and nutritional concerns.

See Treatment for a full discussion.

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Background

Of lymphatic malformations, 50% are present at birth, and 90% are diagnosed by the time the individual is aged 2 years. However, the time of diagnosis can range from 19 weeks' gestation to the individual's second decade of life.

Of lymphatic malformations in the oral cavity, 40-50% involve the dorsum of the tongue, which is the preferred site of intraoral involvement, and they range in size from 1-2 cm. [7, 8] Other sites of involvement include lips, buccal mucosa, soft palate, and floor of the mouth. [9] These distinctions can be somewhat artificial because lesions that involve the oral cavity may extend from the orbits to the upper mediastinum and axillae.

Lymphatic malformations are typically classified as simple microcystic (cavities smaller than about 2 cm3), simple macrocystic (cavities larger than about 2 cm3), or mixed microcystic and macrocystic lesions, according to their predominant histologic features. [10]

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Pathophysiology

Although no clear consensus on the mechanism for lesion development exists, the lesions likely develop as a result of aberrant sequestration of portions of the primitive embryonic anlagen. The sequestered areas never achieve efficient anastomoses with the larger lymph channels and, as a result, they consist of little more than functionally blocked lymphatic tracts. This blockage may result in increased hydrostatic pressure with subsequent expansion of the lesion until a pressure equilibrium is achieved with the surrounding tissues. The importance of the surrounding tissues in determining the nature of the lesion is evident, as microcystic lesions are more common in the tongue, whereas macrocystic lesions predominate in the relatively compliant tissues of the neck.

At the molecular level, some have hypothesized overexpression of growth factors or their associated receptors essential to lymphatic development. Two examples would be vascular endothelial growth factor types 3 and C.

A neoplasm is classically defined as an abnormal mass of tissue. Its growth exceeds and is uncoordinated with that of healthy tissues and persists in an excessive manner after the inciting stimuli is removed. In contrast, lymphatic malformations and/or lymphangiomas tend to grow commensurately with the child's growth and rarely regress spontaneously. Rapid enlargement of the lesions (out of proportion with the surrounding tissues) is observed only in conjunction with infections of the upper respiratory tract or the lesion itself or with trauma and hemorrhage into the malformation. In addition, the lesions have a typical endothelial cell cycle.

For these reasons, lymphangiomas are considered to be malformations rather than neoplasms. In a teleologic sense, this determination remains rather unsatisfying because the predisposing event (ie, the sequestration of embryonic anlagen) occurs long before these lesions develop. As previously stated, only 50% of the lesions are diagnosed at birth, and a few reports of lesions developing in early adulthood exist.

Moreover, at prenatal ultrasonography, fetal lymphatic malformations are observed to develop and occasionally resolve in utero. Some unrecognized event must be superimposed on these earlier events resulting in the development of a clinically apparent lesion. These events are apparently reversible because the lesions occasionally spontaneously regress in fetuses and children. The inciting event must then be occurring during the maturation of the formed lymphatic systems.

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Etiology

Although no clear consensus on the mechanism for lesion development exists, the lesions likely develop as a result of aberrant sequestration of portions of these primitive embryonic anlagen. See Pathophysiology.

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Epidemiology

Frequency

Determining the true incidence of these lesions is difficult because uniformity in classification and nomenclature is lacking. Moreover, lymphatic malformations are generally treated in tertiary and quaternary medical centers.

A review of 1,243 oral lymphangioma cases revealed the tongue to be the most affected site. [11]

Race

Whites appear to be affected more than people of other races. One exceptional form of lymphatic malformation occurs on the alveolar ridge in approximately 4% of black neonates. This lesion, generally bilateral and small, resolves spontaneously and does not require treatment. [12]

Sex

No clear sex predilection is demonstrated; however, small lymphangiomas (< 1 cm) occurring at the alveolar ridge of the mandible do show a 2:1 male-to-female ratio. [13]

Age

Of lymphatic malformations, 50% are present at birth, and 90% are diagnosed by the time the individual is aged 2 years. However, the time of diagnosis can range from 19 weeks' gestation to the individual's second decade of life. [14]  Individuals older than the sixth decade of life are not commonly affected. [11]

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Prognosis

The oral cavity is a highly specialized structure that is important for speech, deglutition, mastication, and airway maintenance. The functional and anatomic aberrations that result from an oral lymphatic malformation may negatively affect each of these specialized functions. In addition, the aesthetic consequences of an oral lymphatic malformation can be severe and significantly impair a child's psychosocial development. See Complications.

Infection-related morbidity

Approximately 70-80% of patients with lymphatic malformations experience infections, often associated with significant increases in the size of lesions. [15] Infection of oral lymphangiomas have also been complicated by overlying erysipelas, a skin infection. [16] Management of these episodes requires aggressive, early institution of broad-spectrum antibiotic therapy. This typically consists of admission to hospital for a course of parenteral antibiotics followed by a prolonged course of oral antibiotics. Susceptibility to infection of these lesions probably pertains to the structural abnormalities of the tissues and the lymphatic system and its important role in mounting an immune response. Further, lymphocytopenias have been documented in this population, although they have yet to be definitely correlated to an increased risk of infection. [17]

Airway-related morbidity

Rapid enlargement of the lesion either as a result of intralesional hemorrhage or infection may lead to airway obstruction. The clinician must be very vigilant in this regard. As a result of these concerns, approximately 50% of children with such lesions require tracheostomy.

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