Head and Neck Pathology (2020) 14:512–515

https://doi.org/10.1007/s12105-019-01108-z

SINE QUA NON CLINICOPATHOLOGIC CORRELATION

Lymphangioma of the Dorsal Tongue

Brenda L. Nelson1 · Eric L. Bischoff2 · Ashwin Nathan3 · Liana Ma4

Received: 1 November 2019 / Revised: 23 November 2019 / Accepted: 29 November 2019 / Published online: 10 December 2019
© This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply 2019

Abstract
Lymphangiomas are rare, congenital malformations of the lymphatic system which have a marked predilection for the head
and neck. In this region, they most commonly occur on the dorsum of the tongue, followed by the lips, buccal mucosa, soft
palate, and floor of the mouth. Lymphangiomas of the tongue are commonly present at birth; however, they may go unnoticed
until after eruption of the dentition or even puberty. They may present as a defined mass or as macroglossia with impaired
speech, difficulty in mastication, and, in extreme cases, airway obstruction. Clinically, lymphagiomas of the tongue are char-
acterized by clusters of pebbly, vesicle-like nodules. A benign proliferation of lymphatic vessels is identified histologically.
A classic case of a lymphangioma of the dorsal tongue is presented.

Keywords Lymphangioma · Vascular · Soft tissue · Tongue

Clinical Features
A 24 year old female presented to a Malaysian Community
Health Engagement Clinic during Pacific Partnership 2019,
a joint health operation of the Unites States Navy and Malay-
sian Armed Forces. Her chief complaint was of a toothache
for which she requested an extraction. Intraoral examina-
tion revealed partial edentulism and numerous grossly cari-
ous teeth including the one causing her pain. The patient’s
dorsal tongue was notable for a mass surfaced by pebbly,
vesicle-like nodules (Fig. 1). The lesion was partially trans-
lucent with areas that appeared red, purple, and yellow.
The elevated mass was soft to palpation and non-tender.
The patient was aware of the lesion since early childhood
with her parents relaying that it had been present for over 20
years. She expressed little concern for the mass and reported
only mild discomfort when eating hot and spicy foods. Due
to the classic appearance, a working clinical diagnosis of
lymphangioma was established.
The patient consented to extraction of the tooth cited
in the chief complaint, and the procedure was performed
without incident. She returned 4 days later for an incisional
biopsy of the tongue lesion under local anesthetic (Fig. 2).
There were no complications, and the tissue was submitted
in formalin for microscopic review.

Disclaimer The opinions and assertions expressed herein are

those of the author and are not to be construed as official or
representing the views of the Department of the Navy or the
Department of Defense.
* Brenda L. Nelson
Brenda.l.nelson24.mil@mail.mil
1
Department of Anatomic Pathology, Naval Medical
Center San Diego, 34800 Bob Wilson Drive, San Diego,
CA 92134‑5000, USA
2
Department of Oral and Maxillofacial Surgery, Naval
Medical Center San Diego, San Diego, CA, USA
3
Dental Services Malaysian Armed Forces, 96 Armed Forces
Hospital, Lumut Naval Base, Perak, Malaysia
4
Tuanku Mizan Armed Forces Hospital, Kuala Lumpur,
Malaysia
Histologic Features
The tissue specimen was received by the Department of
Pathology, Tuanku Mizan Armed Forces Hospital, Kuala
Lumpur. It measured 1.0 cm in greatest dimension. His-
tologic review revealed a collection of dilated lymphatic
vessels surfaced by normal stratified squamous epithelium
(Fig. 3). The lymphatic channels were intimately associated
with the overlying epithelium reflecting the translucent and
pebbly clinical presentation. The endothelial lining of the
channels was thin, and the spaces contained proteinaceous
fluid, scant lymphocytes, and red blood cells (Fig. 4). The
red blood cells were attributed to the surgical procedure and/

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Fig. 3  Dilated lymphatic vessels intimately associated with the over-

Fig. 1  Clinical photo of the lymphangioma shows the classic pebbly, lying epithelium (low power, hematoxylin and eosin stain).
vesicle-like appearance of the tumor.

Fig. 4  The endothelial lined spaces of the lymphangioma are readily

identified (high power, hematoxylin and eosin stain).

Clinical Outcome

The patient was informed of the diagnosis and the healing of

Fig. 2  Intraoperative photo shows the tumor’s clear to translucent the biopsy site was uneventful. Regular follow-up intervals
vesicles mixed with red to purple-hued areas.
were established

or previous trauma. Extension into deep connective tissues

and muscle was focally identified. The microscopic diagno-
sis of lymphangioma was rendered.

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Discussion
Lymphangioma is a congenital malformation of lymphatic
channels. The proliferation of lymph vessels is hamartoma-
tous and not considered neoplastic. Lymphangiomas are
relatively uncommon but show a strong predilection for the
head and neck [1, 2]. While skin is the most common site,
intraoral lesions are not infrequent and mostly commonly
affect the anterior two-thirds of the dorsal tongue. The lips,
buccal mucosa, soft palate, and floor of mouth are other sites
of occurrence [3]. Most lymphangiomas are present at birth
or develop within the first 2 years of life [4, 5].
The patients’ signs and symptoms are dependent on the
size and precise intraoral location. If small, lymphangiomas
of the tongue may go unnoticed. Larger lesions may compro-
mise speech and/or eating. Bleeding may occur with minor
trauma associated with normal oral functions such as eating,
speaking, and hygiene, and some patients report a burning
sensation associated with their lesion [4]. In extreme cases,
lymphagiomas can be life threatening by causing airway
obstruction or if secondarily infected [4].
Most lymphangiomas of the tongue have a classic clinical
appearance. Lesions of this location are usually superficial,
or have a superficial component, that corresponds to the
characteristic pebbly, translucent, and yellow vesicles said
to resemble frog eggs [4]. Additionally, lesions frequently
have a red or purple hue that results from hemorrhage into
the lymphatic channels.
Histologically, lymphagiomas consist of lymphatic chan-
nels lined by endothelial cells. The size and shape of the
channels are variable and lesions may be classified accord-
ingly. Cavernous lymphangiomas comprise primarily dilated
vessels, capillary lymphangiomas have capillary-sized ves-
sels, and large, cyst-like spaces are characteristic of cystic
lymphangiomas [2]. Lesions tend to be a mixture of sizes,
however, and these distinctions may not be contributory to
the management of the case. The endothelial cells of lym-
phangiomas are reactive for CD31, CD34, and D2-40 [6, 7].
The clinical differential diagnosis for a lymphangioma
may include any “bump of the tongue.” As in this case, a
dorsal location may suggest entities including, but not lim-
ited to, granular cell tumor, lingual thyroid, and mesenchy-
mal tumors. Of the mesenchymal linage, vascular lesions,
including hemangiomas, vascular malformations, and arte-
riovenous malformations are the main consideration. Addi-
tionally, pyogenic granuloma, a reactive lesion, part of the
differential diagnosis. Despite the classic clinical presenta-
tion of lymphangioma of the dorsal tongue, histopathologic
review will assist in providing a precise diagnosis.
When considering the differential diagnosis, hemangio-
mas, particularly infantile hemangiomas, share a similar
clinical profile and natural history with lymphangiomas [8].
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Head and Neck Pathology (2020) 14:512–515
They are frequently congenital or develop soon after birth
and also favor the head and neck. Hemangiomas have the
same sites of predilection and are considered common [8].
Head and neck hemangiomas of the mucosa or skin, when
first are noticed, may appear as flat red macules or raised
masses. This is frequently followed by a proliferative stage
in which the lesion grows rapidly. Over the course of years
infantile hemangiomas involute. As stated previously, tongue
lymphangiomas may develop soon after birth but regression
is not a feature. Characteristically, tongue lymphangiomas
demonstrate a classic frog egg clinical appearance, this find-
ing is absent in hemangiomas.
Histologically, hemangiomas display multiple vessels
lined by endothelial cells. Similarly to lymphangiomas, the
vessels may be of varying sizes. Infantile hemangiomas
may show marked endothelial proliferation, however. The
intimate relationship with the overlying epithelium that is
characteristic of lymphangiomas is not generally seen in
hemangiomas. Additionally, infantile hemangiomas are
overwhelmingly reactive (over 95%) for GLUT-1 while
lymphaniomas are non-reactive [8, 9].
Of the vascular lesions, venous malformation is the next
diagnostic consideration. These lesions tend to be present
at birth (although they may not be noticed) and persist like
lymphangiomas. Clinically, venous malformations may be
red or blue. Lesions may be nodular or have blebs (a blister-
like appearance) that is similar to a lymphangioma. Venous
malformations are typically compressible and may demon-
strate a thrill or bruit whereas lymphangiomas will not [8].
Histologically, venous malformations are composed of aber-
rant vessels often times dilated, filled with red blood cells.
Endothelial cell proliferation is generally not a feature of
vascular malformations.
A less common and perhaps a remote consideration is
arteriovenous malformation, particularly in this tongue. This
lesion is of concerning clinical consequence due to their
high flow. Additionally, they usually do not present until
adulthood. A working diagnosis is often established by clini-
cal presentation then verified with advanced imagining. Due
to concerns for excessive bleeding biopsy is not performed.
Tissue may be submitted after surgical intervention and
may show numerous vessels of varying sizes, thrombosis,
calcification and emblolizing material [10]. The clinically
worrisome presentation of this entity generally excludes
lymphangioma.
Finally, pyogenic granuloma may be part of the differ-
ential diagnosis. This lesion is not a neoplasm, it is a local-
ized reaction to trauma or irritation. Common in children
and young adults, intraorally these tumor-like growths favor
the gingiva, however, are frequently found on the tongue.
Clinically, pyogenic granulomas may present as a sessile
or pedunculated red to pink mass, frequently ulcerated.
Histologically, they may resemble granulation tissue with
Head and Neck Pathology (2020) 14:512–515
a vague lobular architecture of the vessels. Pyogenic granu-
lomas may be very inflamed, demonstrating both acute and
chronic inflammation with or without ulceration.
Lymphangiomas of the tongue are generally not a diag-
nostic dilemma. The histologic features are characteris-
tic and the clinical appearance often classic. A detailed
review of the vascular lesions in the differential diagnosis
is beyond the scope of this paper, however, differentiating a
lymphangioma from these entities is assisted by identifying
the contents of the vessels. The channels of lymphagiomas
are filled with proteinaceous fluid and scant lymphocytes
whereas the other vascular lesions contain red blood cells.
It is important to remember that surgical trauma or even nor-
mal functional trauma may result in red blood cells within
the vessels of a lymphangioma, so their presence does not
exclude this diagnosis. Additionally, immunohistochemical
markers CD31 and CD34 are expressed in the endothelial
cells of all vascular lesions, but only lymphatic endothelial
cells are reactive to D2-40 [6, 7]. While this marker may
be helpful to differentiate the type of vessels, it should be
stressed that all the entities considered in the differential
diagnosis are usually best interpreted with hematoxylin and
eosin stained sections.
Treatment for lymphangiomas of the tongue is usually
surgical excision; however, complete removal may be com-
plicated by the size and infiltrative nature of the lesion. Sev-
eral treatment modalities have also been utilized including
injection of sclerosing agents, radiofrequency ablation, elec-
trocoagulation, cryotherapy, embolization, steroid adminis-
tration, radiation, and laser surgery [11, 12].
Post-operative complications are reported in as many
as 12–33% of cases and recurrence is seen in 15–53% of
cases [13]. Common complications associated with surgi-
cal resection of lingual lymphangioma include hemorrhage
that can be difficult to control due to the vascular nature of
the lesions. Incomplete resection of the lesion is another
complication frequently encountered due to the infiltrative
properties of lymphangiomas which contributes to the high
rate of recurrence. Due to the high rates of complication and
recurrence, surgery is typically driven by symptomology and
functionality. After diagnosis, lesions that are asymptomatic
and do not impair function can be left with routine follow up
and scheduled monitoring.
Acknowledgements The authors wish to express their mutual grati-
tude for the opportunity to work together as a result of the combined
efforts of the United States Navy and the Malaysian Armed Forces.
Lasting professional and personal friendships were forged among the
contributing teams.
515
Funding This study has no funding.
Compliance with Ethical Standards
Conflict of interest The authors declare that they have no conflict of
interest.
Ethical Approval This article does not contain any studies with human
participants or animals performed by any of the authors.
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