Rom anian Journal of Morphology and Em bryology 2006, 47(4):373–377
C CA AS SE E R RE EP PO OR RT T
Lymphangioma of the oral cavity
LIGIA STĂNESCU1), E. F. GEORGESCU2),
CRISTIANA SIMIONESCU3), IULIANA GEORGESCU4)
1)Department of Pediatry
2)Department of Internal Medicine
“Filantropia” University Hospital of Craiova
3)Department of Morphopathology,
Emergency County Hospital of Craiova
University of Medicine and Pharmacy of Craiova
4)Mediplus Diagnostica Gastroenterology Center,
Department of Dermatology, Craiova
Lymphangiomas are uncommon congenital hamartomas of the lymphatic system, usually diagnosed in infancy and early childhood.
Commonly located at head and neck, they are rarely situated in the oral cavity. Preferred site of oral involvement is the tongue. In the
absence of proper therapy, lymphangiomas of the tongue are extremely recurrent, leading to serious complications such as hemorrhage or
obstruction of the upper respiratory airways. The authors present the case of eleven years old boy with pseudo-vesicles, and smooth,
glossy lesions on the tongue, and a red prominent pulsative sublingual mass located at the base of the tongue. Both the macroscopic
structure and the histological aspect sustain the diagnosis of lymphangioma. CT established that is a profound lymphangioma with a
narrow communication with the superficial planes. Conclusions. Though rarely met in the oral cavity, lymphangiomas are an eventuality to
take into consideration by the clinician. Early recognition is of utmost importance to initiation of proper treatment, and avoiding serious
Lymphangioma is a benign hamartomatous tumor of
lymphatic vessels with a marked predilection for the
head and the neck region .
Described for the first time by Redenbacher in 1828,
currently the lymphangiomas are classified as
malformations and not as neoplasms .
Lymphangiomas represent about 6% of the total
number of benign tumours of the smooth tissue in
patients aged less than 20 years .
The oral localization of lymphangiomas is less
usual, and the most common site in this case is the
tongue, especially the 2 third anterior .
Lymphangiomas usually affect the ventral part of the
tongue and are solitaire or circumscribed. Occasionally,
they are associated with cystic hygroma .
We present a case of superficial lymphangioma of
the tongue associated with a profound lymphangioma
situated at the base of the tongue.
? Patient and methods
At presentation in the Paediatric department, the
patient was 9 years of age and reported abdominal pain,
nausea, vomiting, and inconstant dyspeptic tools. At the
same time he accused pain in the tongue at the contact
with some aliments, leading sometimes to difficult
alimentation. The parent informed us that in early
childhood (at the age of 1) appeared little, asymptomatic
prominences on the ventral part of the tongue (the first
third and the tip). Then the lesions extended, and
became more evident after ingestion of warm aliments.
He was examined several times by physicians, and the
lesions were diagnosed either as candidosis, either oral
papilomatosis. Different antifungal treatments could not
prevent the extension of the lesions.
The patient had no history of systemic disease, and
the familial background excluded the existence of the
similar clinical manifestations at any other family
At physical examination, the child was in good
general condition and good nutrition state, without signs
of obstruction of the superior respiratory tract. There
were no cardio-pulmonary
Abdominal tenderness was present at superficial
palpation of the epigastric and umbilical regions.
Recently, the child reported difficult appetite, vomiting
and dyspeptic stools.
Dermatological examination showed an enlarged
reddish tongue, with numerous granular lesions,
smooth, glossy, vesicle-like conglomerated in a placard
affecting the two anterior thirds of the tongue including
the tip, dorsal and lateral surfaces, with lesser
involvement of the inferior one. The color of the lesion
ranged from yellow to purple (Figures 1 and 2).
The mycological exam was positive for Candida
infection, and recommended the administration of the
Ligia Stănescu et al.
fragments of the lesions from the tip of the tongue
showing squamous epithelium
papillomatosis and parakeratosis, and solitaire and
clustered cystic spaces. Enlarged, clustered cystic
spaces of the papillary derma presented a sheath of
endothelial cells and included eosinophilic material.
Lymphatic vessels, some of them with the thick walls
were present in the profound derma and subcutaneous
(Figures 3 and 4).
Computerized tomography showed at the base of the
tongue the presence of a well delimitated mass of
tissular density, protruding in the oropharynx, with
slight inhomogeneous contrast substance retention.
The CT aspect suggested profound lymphangiomatous
tumor with a narrow communication with the superficial
planes (Figure 5).
Local ultrasound showed normal Doppler arterial
pattern in the common and internal carotid artery,
billateral. There were no arterio-venous fistulas.
Abdominal ultrasound was done to exclude hepatic or
pancreatic lymphangiomas and showed no modification.
Digestive endoscopy also excluded the involvement of
gastrointestinal tract. The clinical and paraclinical data
sustain the diagnosis of oral lymphangioma.
examination was performed on
hamartomas of the lymphatic system, usually diagnosed
in infancy and early childhood as lobular masses or
cystic lesions .
They may be present anywhere on the skin and
mucosa. The most usual locations are the head and
neck, followed by the proximal extremities, buttocks
and trunk. Sometimes they can be located at intestinal,
pancreatic and mesenteric level. Lymphangioma rarely
affect the oral cavity. Affected sites in the oral cavity
may include the tongue, palate, gingival and oral
mucosa, lips, and alveolar ridge of the mandible .
The classifications of the lymphangioma are not
standardized. Usually, they are divided depending on
depth and size of the abnormal lymphatic vessels in two
groups: a superficial one, including the circumscribed
angyoma and another more profound, represented by
cavernous lymphangioma and cystic hygroma. Many
authors consider cystic hygroma as a cavernous
lymphangioma variant. Lymphangioma circumscriptum
is a localized congenital hamartoma of lymphatic
vessels consisting of deep cavernous cisterns within the
subcutaneous tissue, with secondary formation of more
dilated lymphatics .
Two major theories have been proposed to explain
the origin of lymphangiomas .
The first theory is that the lymphatic system
develops from five primitive sacs arising from the
venous system. Concerning the head and the neck,
endothelial outpouchings from the jugular sac spread
centrifugally to form the lymphatic system. Another
theory proposes that the lymphatic system develops
from mesenchymal clefts in the venous plexus reticulum
and spread centripetally towards the jugular sac.
are uncommon congenital
Finally, lymphangioma develop from congenital
obstruction or sequestration of the primitive lymphatic
enlargement [7, 8].
These complex abnormal lymphatic patterns running
from the cisterns to the skin surface, adopting a
meandering course with frequent anastomosis and
branching, were firstly described by Whimster IW
et al. . On the base of the three-dimensional
lymphatic models rebuild post-mortem; he demonstrated
the concept of “iceberg” in the case of lymphangiomas,
the lymphatic involvement being much more important
in depth than in surface. The lesions are composed from
ectopic lymphatic system of lymphatics separated from
the normal network of lymphatic vessels, yet
communicating with the superficial lymphatics that
become dilated by the continuous rise and fall of the
pressure that is transmitted from the muscular walls of
the deep cisterns. Thus, the blockage can determine the
growth of the hydrostatic pressure with consecutive
expansion of the lesions, untill an equal pressure with
the contiguous tissues will be realized. The importance
of the contiguous tissues is evident, as microcystic
lesions are more common on the tongue, whereas
macrocystic lesions predominate in the relatively
compliant tissues of the neck .
Clinically, lymphangiomas of the oral cavity have a
characteristic aspect. Usually, there is a plaque
constituted from small vesicles with thin walls,
translucide like frog eggs. Part of the vesicles are full
with clear content (lymph), part has a blood content
suggesting co-existence of the involvement of the
lymphatic anomalies with abnormalities of the blood
vessels. Sometimes, like in the case we present,
they are associated with a diffuse, profound
lymphangioma that appear like a submucous mass.
This association explains the frequent recurrence,
almost constant after excision
lymphangiomas of the tongue are rare, this must be
recognized early for optimal therapeutic result [11, 12].
circumscriptum is characterized by solitary and grouped
dilated cystic spaces in the papillary derma. The cystic
spaces are lined by endothelial cells often containing red
blood cells, as well as lymphatic fluid. In the deep
derma and subcutaneous fat, dilated lymphatic vessels
are also seen, some of it containing thickened muscular
A classification of the lymphangioma of head and
neck on the base of the spread the anatomical
involvement had been proposed by De Serres LM :
1. Stage/class I – infrahyoid unilateral lesions;
2. Stage/class II – suprahyoid billateral lesions;
3. Stage/class III – suprahyoid or
4. Stage/class IV – suprahyoid bilateral lesions;
5. Stage/class V – suprahyoid or infrahyoid bilateral
6. Stage/class IV – infrahyoid bilateral lesions.
Ricciardelli LJ and
demonstrated that suprahyoid lymphangiomas had a
significantly higher rate of recurrence than infrahyoid
Richardson MA 
Lymphangioma of the oral cavity
Figure 1 – Dorsal aspect of the tongue. Enlarged, reddish
tongue with multiple granular lesions. Smooth, glowing
glossy-shiny vesicle-like lesions affecting the tip and
dorso-lateral surfaces of the tongue
Figure 5 – Well-delimitated mass with tissue density
protruding in the oropharynx, with discrete
contrast substance retention
Figure 2 – Inferior aspect of the tongue covered by
vesicle-like lesions. Areddish, prominent mass
is visible at the base of the tongue
Figure 3 – Squamous epithelium with acanthosis,
papillomatosis, parakeratosis and solitaire
clustered cystic spaces
Figure 4 – Enlarged, clustered cystic spaces of the papillar
derma, presenting a sheath of endothelial cells and
included eosinophyllic material. Lymphatic vessels are
present in the profound derma and subcutaneous tissue
Ligia Stănescu et al.
Lymphatic malformations have a growing trend with
the age of the child and rarely regress; clinically, their
location determines the symptoms. While oral cavity is
a specialized structure for speaking, deglutition,
mastication and respiration,
malformations can affect all these functions. Many
patients may have no other symptoms except some
The commonest complication of the lymphatic
malformations is infection, associated with the growth
of the lesion. Lymphocytopenia has been documented in
all these patients, although a clear correlation with the
infection risk must be established .
The rapid growth of lymphangiomas can be
associated with hemorrhage or can lead to obstruction of
the upper respiratory tract , 50% from the children
with these lesions requiring tracheotomy .
The diagnostic of lymphangiomas is not difficult,
being mainly clinical. Sixty percent of lymphangiomas
are diagnosed at birth and 80–90% in the second year of
Lymphangioma may resemble to a number of oral
lesions including hemangioma, teratoma, dermoid cyst,
thyroglossal duct cyst, heterotopy of gastric mucosal
cyst and granular cell tumor . The differential
diagnosis must also include, like in the case we
presented, oral florid papillomatosis.
For inexperienced clinicians
with atypical clinical features a definitive diagnosis
should be made trough biopsy and histopathologic
Imagistic studies are important for the confirmation
of the diagnosis. Prenatal ultrasound may be use for
identifying cystic hygroma and also can monitored
regression or recurrence. MRI is important for
differentiate complex vascular malformations. Fetal
MRI evaluates oral and cervical lesions previously
detected by prenatal ultrasound, being also used to
determine the potential of airway obstruction .
CT is superior to MRI for evaluation of the bone
deformation and in detecting febolites caused probably
by anterior intralesional hemorrhage.
Therapeutically, many approaches have been
proposed. Spontaneous regression of the lesion is rarely
encountered. Aspiration of the cystic content is
temporary used as a measure to relieve airway
obstruction, paying the price for the risk of introducing
infection. Sclerozing agents are ineffective, probably as
a result of the discontinuous basement membrane of the
lymphatic vessels .
Neodymium–yitrium–aluminum Garnet (Nd-YAG)
laser surgery has become widely preferred because of its
advantages as less bleeding and edema versus standard
methods of surgical resection .
Surgical excision is the usual treatment of
lymphangioma. However, some clinicians do not
recommend surgery for non-enlarging lymphangiomas
of the tongue because of difficulties in removal and the
high recurrence rate .
In choosing the ablative therapy of lymphangiomas
the exact knowledge of the anatomy and spread of
lesions are very important. In this case, in which
or in lesions
CT proved the existence of a narrow communication
between the lymphangioma from the base of the tongue
and the superficial planes, the risk of recurrences after a
surgical simple excision are high. We left to the
surgeons the choice of the most adequate procedure.
Although rarely encountered in the oral cavity,
lymphangiomas represent a condition that must be
recognized. Their early recognition allows proper
initiation of treatment and prevents the occurrence of
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A unified concept of
Lymphangioma of the oral cavity Download full-text
 BRENNAN T. D., MILLER A. S., CHEN S. Y., Lymphangiomas
of the oral cavity: a clinicopathologic immunohistochemical
and electronomicroscopic study, J Oral Maxillofacial Surg,
Ligia Stănescu, MD, PhD, Department of Pediatry, “Filantropia” University Hospital of Craiova, University of
Medicine and Pharmacy of Craiova, 2–4 Petru Rareş Street, 200349 Craiova, Romania; Phone +40748–182 406,
Fax +40251–420 896, E-mail: firstname.lastname@example.org
Received: March 6th, 2007
Lymphangioma treatment with Nd-YAG laser, Turk J
Pediatr, 1995, 37(3):253–256.
Accepted: April 10th, 2007