Case Report - Cavernous lymphangioma of lower lip

CASE REPORT

Cavernous lymphangioma of lower lip

Bindhu PR, Justin Mathew 1, Priya Thomas, Jayanthi P, Rekha Krishnapillai

Department of Oral Pathology and Microbiology, 1Dept. of Oral and Maxillofacial Surgery
Annoor Dental College, Muvattupuzha, Ernakulam, Kerala, India
Corresponding author: hripa@rediffmail.com

Abstract
Lymphangiomas are benign tumours of
lymphatic
vessels
showing
marked
predilection for head and neck region. They
are extremely rare in the oral cavity. The
common
site
of
occurrence
for
lymphangioma in the oral cavity is the
anterior dorsum and lateral border of
tongue. Rarely has it been reported in other
parts of oral cavity such as the palate,
cheeks, floor of the mouth, gingiva and lips.
The most accepted treatment option for
lymphangioma is complete surgical excision.
There is only a minimal chance of
recurrence. We report a case of cavernous
lymphangioma of the lower lip which was

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excised and had satisfactory post- operative

healing.
Introduction
Lymphangiomas
are
congenital
malformations of lymphatic system that
involve skin and subcutaneous tissue. 1,2 This
hamartomatous lesion was first described by
Redenbacher in 1828.3 They are believed to
arise as a result of lymph sac sequestration
which fails to establish communication with
larger lymphatic channels. They enlarge due
to improper drainage or excessive secretion
of lining cells.4,5,6,7 It has been reported that
approximately
75%
of
cases
of
lymphangiomas occur in the head and neck
region.8 The most frequent site of
involvement in the oral cavity is the tongue. 7

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Case Report - Cavernous lymphangioma of lower lip

Less frequent sites of involvement include

areas like palate, buccal mucosa, gingiva,
floor of the mouth and lips.9 Current
treatment methods of lymphangioma
include surgery, sclerotherapy, laser therapy
or a combination of these.10 The present
report focuses on a rare case of
lymphangioma of the lower lip in a male
child with a review of the scientific
literature.
Case report
A 13 year old boy reported to the
Department of Oral Medicine and
Radiology with a chief complaint of a
growth on the lower lip for the past six
months. The patient noticed a rapid
enlargement of the growth following
orthodontic treatment. He gave a history of
a similar growth on the same site about one
year back which was removed by surgical
excision. On clinical examination, a painless
growth was seen on the right lower lip in
relation to 43, 44 region. The lesion
appeared as multiple reddish pink
transparent vesicles grouped together,
measuring approximately 1.5 1 cm in
dimension with an irregular surface and
borders (Figure 1). A differential diagnosis
of lymphangioma and mucocele were
considered based on the clinical features.
Wide surgical excision of the lesion was

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carried out and the biopsy specimen sent for

histopathological
examination.
Post
operative wound healing was satisfactory
without any complications.

Figure 1. Lymphangioma of right lower lip

of a thirteen year old boy (10x)
Histopathological examination showed large
dilated cavitary spaces lined by flattened
endothelial cells containing eosinophilic
fluid located just beneath the surface
epithelium (Figure 2). Numerous smaller
and dilated lymphatic channels, few blood
vessels and inflammatory cells were also
noticed in the deeper connective tissue
(Figure 3). The surface was covered by an
atrophic keratinized stratified squamous
epithelium. Based on the histopathological
features, a final diagnosis of lymphangioma
was made. Follow up of the patient was
recommended for at least two years since
this was a recurrent lesion.

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Case Report - Cavernous lymphangioma of lower lip

Figure 2. Photomicrograph of the lip lesion (H&E x10) Enlarged lymphatic vessels containing
lymph are seen in the superficial connective tissue

Figure 3. Photomicrograph of the lower lip lesion (H&Ex10). Smaller lymphatic vessels
containing lymph are seen in the deeper connective tissue

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Case Report - Cavernous lymphangioma of lower lip

Discussion
Lymphangiomas are hamartomatous lesions
of lymphatic vessels that show predilection
for head and neck region. They occur as a
result of structural defects in the lymphatic
pathway, which usually develop during the
sixth week of gestation.5Approximately
about 50% of the cases are noticed at the
time of birth and around 90% develop by 2
years of age.7 The age of diagnosis can range
from nineteen weeks of gestation to the
second decade of life. According to a
retrospective study of lymphangioma by
Alqahtani et al, the average age at diagnosis
was 3.3years.11 No definite sex predilection
is noticed. Determining the true incidence
of these lesions is difficult due to lack of
uniformity
in
classification
and
nomenclature.
Lymphangiomas
show
a
marked
predilection for the neck and head region
mostly the cervical area (posterior triangle,
anterior triangle, submandibular and parotid
region in descending order). 9 Other
common sites, outside the head and neck,
include the axilla, shoulder, chest wall,
mediastinum, abdominal wall, and thigh.10
Lymphangiomas of lateral and posterior
neck region are frequently associated with
chromosomal anomalies like Turners
syndrome, Noonans syndrome and foetal
hydrops.7
Primary
intra-osseous
lymphangioma is a very rare entity that
occurs as a result of gradual extension of a
peripheral soft tissue lymphangioma to the

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periosteum of adjacent bone and into the

marrow spaces.12
Lymphangioma rarely involve the oral
cavity. Most commonly involved site is the
dorsum of tongue. Lower lip is an
uncommon
site
comprising
of
approximately
6%
of
all
oral
lymphangiomas.6 Characteristic clinical
appearance of oral lymphangioma is a
superficial pebbly vesicular lesion resembling
frog egg / tapioca pudding.8 Deep seated
lesions appear as soft ill defined submucosal
masses that markedly distort the local
anatomy.13 Half of the cases may be
diagnosed with the help of clinical
manifestations alone. Early diagnosis may be
established
with
the
help
of
ultrasonography, MRI, arterogram and CT
angiography.14 In the oral cavity, lesions like
haemangioma, lingual thyroid, thyroglossal
duct cyst, granular cell tumour, cheilitis
granulomatosa, mucocele/ ranula and
cellulitis may be considered for differential
diagnosis.15
The lesions are capable of rapid growth with
infection or trauma. This may result in
disfigurement as well as severe impairment
of respiration, deglutition and speech
especially lymphangioma of the tongue.
Even though they are benign lesions,
spontaneous regression is rarely seen (1.6
16.0%). With adequate follow-up, it is seen
that a regression is usually followed by a
recurrence.16

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Case Report - Cavernous lymphangioma of lower lip

The case discussed here occurred on the

lower lip which is a rare site for
lymphangioma with a rapid growth rate in
response to trauma. It was a recurrent case
of lymphangioma within a period of one
year, following surgical excision.
Histopathologically, lymphangioma may be
classified into three types according to the
size of the lymphatic vessels- lymphangioma
simplex (capillary lymphangioma) which
consists of small capillary sized vessels,
cavernous lymphangioma composed of
larger dilated lymphatic vessels and cystic
lymphangioma (cystic hygroma) exhibiting
large macroscopic cystic spaces. The
lymphatic channels are usually lined by a
single layer of endothelial cells. 17Often all
three histological types may be found in the
same lesion.8Among these the most
common intraoral type is cavernous
lymphangioma.9
The structure of blood vessels and lymphatic
vessels show certain similarities and
differences. The supporting pericytes and
smooth muscle cells of blood vessels produce
a large amount of extracellular matrix,
whereas supporting cells are sparse in the
case of lymphatic channels and hence lack a
continuous basement membrane. Lymphatic
vessels also have fenestrations in their vessel
walls. These factors attribute to the
increased permeability of lymphatic
channels.
D2-40
is
an
excellent
immunohistochemical marker for lymphatic
vessels and helps in differentiation lymphatic

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endothelial cells from endothelial cells lining

the blood vessels.18
Though lymphangiomas are benign lesions,
the involvement of vital structures or
aesthetic /functional requirements may
necessitate treatment of these lesions. 6 The
most accepted treatment modality for
lymphangioma consists of complete surgical
excision with minimal chances of
recurrence.19 Complete excision is possible
only for small and localized lesions. The
management
of
head
and
neck
lymphangioma is challenging because of its
close association with adjacent vital
structures and poor demarcation of the
lesion. Hence, complete excision is difficult
to achieve in some cases and recurrence can
occur following incomplete removal. 11
Multiple treatment modalities have been
recommended for deep seated and large
lesions.
They
include
cryotherapy,
electrocauterization, sclerotherapy (OK432),
administration
of
steroids,
9
embolization and laser therapy. According
to Gilberth, CO2 laser technique was more
convenient with less chance for recurrence
than any other methods.3 The various
treatment
options
for
lymphatic
malformations have their advantages and
disadvantages and the method of treatment
should depend on the status of the patient,
available technology and expertise.
Conclusion

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Case Report - Cavernous lymphangioma of lower lip

Lymphangioma of the head and neck are

benign lymphatic malformations with a
marked predilection for head and neck. In
the oral and maxillofacial region diagnosis of
lymphatic malformations is easy but
management become difficult due to
involvement of vital structures and chances
for disfigurement. Though rarely seen in the
oral cavity lymphangiomas are an
eventuality to take into consideration by the
oral surgeon. Early recognition is of utmost
importance to initiation of proper treatment
and avoidance of complications.
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