The Laryngoscope

VC 2012 The American Laryngological,

Rhinological and Otological Society, Inc.
Case Report
Symmetrical Lipomatosis of the TongueA Rare Cause of
Macroglossia: Diagnosis, Surgical Treatment, and Literature Review
Ioannis Vasileiadis, MD; Georgios Mastorakis, MD; Panagiotis Ieromonachou, MD; Ioannis Logothetis, MD
Symmetrical lipomatosis of the tongue is an extremely rare condition. To date, only eight cases have been reported in
the literature. We present an extremely rare case of intramuscular lipomatosis of the tongue in a 67-year-old male. The pres-
ent case is unique because of its infiltrating nature and the extension of the lesion. Glossectomy was performed to reduce the
size of the tongue and for diagnosis. The literature is reviewed and clinical characteristics, pathology, and surgical treatment
are discussed. Laryngoscope, 000:000000, 2012
Key Words: Tongue lipomatosis, benign tumor, multiple symmetrical lipomatosis, macroglossia.
Laryngoscope, 123:422425, 2013
INTRODUCTION
The term lipomatosis refers to a disorder character-
ized by multiple, nonencapsulated lipomas affecting
various areas.
1
The majority of tumors of the tongue are
malignant.
2
Tongue cancer is the most common oral can-
cer. The incidence rate is higher in males than females,
and the average age of incidence is approximately 60
years old. Of the benign tumors, lipomas comprise a
very small proportion, between 1% and 4.4% of all
benign oral lesions, and lipomatosis of the tongue
is extremely rare.
3
A case of large multiple lipomatosis of the tongue
with intramuscular invasion is presented, the current lit-
erature is reviewed, and the surgical treatment with
possible complications is discussed. To the best of our
knowledge, this is the ninth case of symmetrical lipoma-
tosis of the tongue presenting as macroglossia. After
extensive review of the literature, we believe that it is the
bulkiest lipomatous lesion of the tongue ever reported.
CASE REPORT
A 67-year-old male presented with a 2-year history
of a gradually progressive, diffuse, painless swelling on
both lateral borders of the tongue. He was aware of mod-
erate difficulty in swallowing and dysarthria for the last
5 months. There was also mild dyspnea for the last 6
months, but no stridor or ankyloglossia was observed.
He had diabetes mellitus type 2 and took an oral anti-
diabetic treatment with metformin 850 mg daily. The
remainder of his medical and social history was noncon-
tributory. The patient was not obese and did not abuse
alcohol, but he had chronic obstructive pulmonary
disease.
On clinical examination, the tongue was diffusely
and remarkably enlarged, with its borders protruding
symmetrically with Mallampati class IV (Fig. 1). Swel-
ling on the right lateral border measured 5.5 4 cm,
and swelling on the left measured 5 3.5 cm. The cen-
tral portion of dorsum had a normal appearance, and
the mucosa was smooth with normal surface, and no
lacerations or erosions were present. A moderate
impairment in the tongues movement was observed
because of the increased size of the tongue. No tumor
masses could be identified on the head, neck, trunk, or
extremities.
Computed tomography scan demonstrated a lesion
with reduced signal that involved both sides of the
tongue symmetrically. The lesion extended from the tip
to the root of the tongue and epiglottis, causing moder-
ate narrowing of the upper airway (Fig. 2). Magnetic
resonance imaging (T1 weighted) showed that the entire
muscular mass of the tongue had been replaced by adi-
pose tissue (Fig. 3).
Bilateral partial glossectomy and tongue debulking
were performed under general anesthesia. A spindle-
shaped incision was made in both lateral edges of the
tongue, extending from the tip of the tongue to the level
of the circumvallate papillae. Intraoperatively, adipose
tissue was found deeply invading the lingual muscles.
From the Department of OtolaryngologyHead and Neck Surgery
(I.V.), Department of Oral and Maxillofacial Surgery (G.M., I.L..), and
Department of Pathology (P.I.), Venizeleio-Pananeio General Hospital,
Heraklion, Greece.
Editors Note: This Manuscript was accepted for publication
August 15, 2012.
The authors have no funding, financial relationships, or conflicts
of interest to disclose.
Send correspondence to Ioannis Vasileiadis, MD, Department of
Otolaryngology, Head and Neck Surgery, Venizeleio-Pananeio General
Hospital, Heraklion, Greece., 30, Alexandroupolis, Evros, Greece.
E-mail: j.vasileiadis@yahoo.gr
DOI: 10.1002/lary.23724
Laryngoscope 123: February 2013 Vasileiadis et al.: Symmetrical Lipomatosis of the Tongue
422
No capsule formation was detected (Fig. 4). A large part of
nonencapsulated fat tissue was removed from each side,
reaching nearly to the root of the tongue. The volume of
resection in both sides was carefully decided by taking
into consideration the postoperative shape and size of the
tongue and avoiding injury to the lingual artery and lin-
gual nerve. Following careful dissection, the lingual
artery was identified and protected from possible injury. A
piece of lingual mucosa was removed to prevent folding.
Fig. 2. Axial contrast-enhanced computed tomography scan dem-
onstrated a mass with low signal density that involved both sides
of the tongue symmetrically. The lesion was extended from the tip
to the root of the tongue, causing moderate narrowing of the
upper airway with reduction of the transversal diameter of the
pharynx air column.
Fig. 3. Coronal magnetic resonance imaging view (T1 weighted)
showed that the greater part of lingual muscles have been
replaced by adipose tissue.
Fig. 4. Intraoperative view showing adipose tissue that invaded
deeply in the lingual muscles. No capsule formation was detected.
[Color figure can be viewed in the online issue, which is available
at wileyonlinelibrary.com.]
Fig. 1. Bilateral enlargement of the tongue with its borders protrud-
ing symmetrically (Mallampati class IV). [Color figure can be viewed
in the online issue, which is available at wileyonlinelibrary.com.]
Laryngoscope 123: February 2013 Vasileiadis et al.: Symmetrical Lipomatosis of the Tongue
423
We placed four mattress sutures pressing the cephalic to
the caudal lingual surface. Consequently, we reduced the
dead space and minimized the risk for hemorrhage.
Postoperatively, the volume of the tongue was
reduced and the load was also decreased on the existing
muscle fibers. The result was improvement of obstruc-
tive sleep apnea syndrome symptoms, which was our
primary objective.
Histopathological findings revealed lobules and
sheets of mature adipocytes infiltrating between
atrophic striated muscle fibers, confirming the diagnosis
of symmetrical lipomatosis of the tongue (Fig. 5).
In the clinical follow-up, after 18 months the
patient had a minimal loss of articulation function,
whereas the swallow function is normal. No obvious
deviation of the tongue to one side or atrophy was
observed, and no regrowth of tumors was noticed 18
months after surgery.
DISCUSSION
Benign tumors of the tongue are uncommon com-
pared with malignant tumors.
2
Lymphangioma,
cavernous hemangioma, and neurofibromatosis are the
most common benign tumors that cause macroglossia.
Other benign tumors of the tongue are papilloma, fibroe-
pithelial polyp, plexiform schwannoma, chondroma,
juvenile fibroma, chondroma, and lipoma.
1,4
Lipoma is a benign tumor composed of adipose tis-
sue, and is the most common form of soft-tissue tumor.
Lipoma of the tongue is rare and usually presents as a
single, pedunculated, superficial lesion. Enzinger and
Weiss classified lipomas into five categories: 1) lipoma;
2) variants of lipoma; 3) heterotopic lipoma; 4) infiltrat-
ing or diffuse, neoplastic, or non-neoplastic proliferation
of mature fat; and 5) hibernoma.
5
Although lipomas are considered to be benign
tumors, there is a small possibility of associated malig-
nancy. To exclude well-differentiated liposarcoma,
several cross-sections of the lesion should be examined.
Suspicious pathological characteristics include intramus-
cular invasion of the lesion and presence of lipoblasts.
6
Benign symmetric lipomatosis is an uncommon
pathologic condition characterized by symmetric diffuse
growth of mature adipose tissue that commonly affects
the neck and superior part of the trunk.
7
This condition,
also known as Madelungs disease or Lanois-Bensaude
syndrome, has unknown etiology, but it is strongly
related to alcohol abuse. This uncommon disease pre-
dominantly affects males between the ages of 30 and 60
years old and males living in the Mediterranean area.
8,9
Symmetric lipomatosis of the tongue is character-
ized by involvement exclusively of the tongue,
invasiveness, and absence of encapsulation of the adi-
pose tissue.
9
The present case satisfied all these criteria
and thus was considered to be lipomatosis.
Katou et al. suggested that symmetric lipomatosis
of the tongue and benign symmetric lipomatosis are dif-
ferent entities, whereas other authors believed that they
are the same entity based on the similar histologic pat-
terns seen in both conditions.
10
The most significant
differences between the two conditions are indicated in
Table I. Because the site of involvement and the age of
appearance differ between lingual lipomatosis and be-
nign symmetric lipomatosis, it still remains
controversial whether they should be categorized as the
same entity.
The histological examination in cases of symmetric
lingual lipomatosis reveals diffusely proliferated adipose
tissue interspersed between the muscle fibers. It leads to
gradual increase of tongue dimension, which causes
symptoms such as dysarthria, dysphagia, dyspnea, stri-
dor, sleep apnea, and dentofacial anomaly.
11
Macroglossia is the enlargement of the tongue and
can be found in a variety of conditions, including congen-
ital syndromes (Down syndrome, Beckwith-Wiedermann
syndrome), trauma (lingual hematoma), benign neo-
plasms (lymphangioma, hemangioma), malignant
neoplasms (squamous cell carcinoma), metabolic diseases
(amyloidosis, glycogen storage disorders), and angioneur-
otic edema.
10,11
Diagnosis of symmetrical lipomatosis of the tongue
is established with clinical data, and computed tomogra-
phy or magnetic resonance imaging may be useful.
Fig. 5. Photomicrograph showing lobules and sheets of mature
adipocytes, diffusely infiltrating between few atrophic striated
muscle fibers (hematoxylin-eosin stain, original magnification
100). [Color figure can be viewed in the online issue, which is
available at wileyonlinelibrary.com.]
TABLE 1.
Differences between benign symmetric lipomatosis (BSL) and
symmetric lipomatosis of tongue (SLT).
BSL SLT
Age of onset 30 to 60 Over 60
Alcohol abuse Strongly related No relationship
Lingual involvement Rare Always
Fat deposition in neck
and upper trunk
Typical Never
Origin of patients Mediterranean Orientals
Laryngoscope 123: February 2013 Vasileiadis et al.: Symmetrical Lipomatosis of the Tongue
424
Computed tomography demonstrates a multifocal lesion
with density of adipose tissue invading the lingual
muscles bilaterally. Magnetic resonance imaging shows
slightly ill-defined tissue of attenuation levels suggestive
of fat, invading bilaterally the lingual musculature, and
reveals that the greater part of the lingual muscles have
been replaced by adipose tissue.
12
The definitive diagnosis of lipomatosis is estab-
lished after histopathological examination of the
specimen obtained at biopsy or glossectomy. Histological
examination reveals sheets and lobules of mature adipo-
cytes infiltrating between muscle fibers. The muscle
fibers in areas show varying degrees of atrophy. The
nuclei of the fat cells appear bland, and the overlying
squamous epithelium is intact and normal.
9
Because of the benign infiltrative nature of the
unencapsulated fatty deposits, conservative surgical
treatment is indicated to relieve functional impairment.
Lipomatosis penetrates deep into the lingual tissue, and
therefore complete resection of the lesion is almost
impossible. Partial glossectomy is the surgical treatment
of choice because it reduces the size of the tongue and
also confirms histologically the deep lingual infiltration
of lipomatous tissue.
9,11
The volume of resection should
be carefully decided by taking into consideration the
postoperative shape and size of the tongue. After the
surgical removal of the masses, there is a significant
improvement in symptoms caused by the bulky tongue.
Regarding the management of the airway in
patients who undergo partial glossectomy, there is no
absolute indication to perform a tracheotomy in every
patient. Some authors suggest that temporary tracheot-
omy should be done in patients who undergo partial
glossectomy, when resection involves the floor of the
mouth and submandibular region. Certainly, the patient
should be informed preoperatively for the possibility of a
tracheotomy and give his consent to perform a tracheot-
omy. Surgeons should consider the extension of the
lesion, the narrowing of the upper airway, and the com-
plications of partial glossectomy, especially lingual
hematoma, and according to these parameters determine
whether a tracheotomy is necessary.
CONCLUSION
Intramuscular lipomatosis of the tongue is an
extremely rare phenomenon that can account for macro-
glossia. Partial glossectomy is the treatment of choice
because of the distinct possibility of a well differentiated
lipoma-like liposarcoma and the improvement of symptoms
after the tongues size reduction. Surgeons should be aware
of the complication of lingual hematoma, especially when a
large portion of lingual tissue is resected bilaterally.
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