Laryngoscope Article

8/12/2019 Laryngoscope Article

1/4

The LaryngoscopeVC 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 inthe 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 thesize 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

INTRODUCTIONThe term lipomatosis refers to a disorder character-

ized by multiple, nonencapsulated lipomas affectingvarious areas. 1 The majority of tumors of the tongue aremalignant. 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 60years old. Of the benign tumors, lipomas comprise avery small proportion, between 1% and 4.4% of allbenign oral lesions, and lipomatosis of the tongueis extremely rare. 3

A case of large multiple lipomatosis of the tonguewith intramuscular invasion is presented, the current lit-erature is reviewed, and the surgical treatment withpossible complications is discussed. To the best of ourknowledge, this is the ninth case of symmetrical lipoma-tosis of the tongue presenting as macroglossia. Afterextensive review of the literature, we believe that it is thebulkiest 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 last5 months. There was also mild dyspnea for the last 6months, 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. Theremainder of his medical and social history was noncon-tributory. The patient was not obese and did not abusealcohol, but he had chronic obstructive pulmonarydisease.

On clinical examination, the tongue was diffuselyand remarkably enlarged, with its borders protrudingsymmetrically 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, andthe mucosa was smooth with normal surface, and nolacerations or erosions were present. A moderateimpairment in the tongues movement was observedbecause of the increased size of the tongue. No tumormasses could be identified on the head, neck, trunk, orextremities.

Computed tomography scan demonstrated a lesionwith reduced signal that involved both sides of thetongue symmetrically. The lesion extended from the tipto the root of the tongue and epiglottis, causing moder-ate narrowing of the upper airway (Fig. 2). Magneticresonance imaging (T1 weighted) showed that the entiremuscular mass of the tongue had been replaced by adi-pose tissue (Fig. 3).

Bilateral partial glossectomy and tongue debulkingwere performed under general anesthesia. A spindle-shaped incision was made in both lateral edges of thetongue, extending from the tip of the tongue to the levelof the circumvallate papillae. Intraoperatively, adiposetissue 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..), andDepartment of Pathology ( P.I.), Venizeleio-Pananeio General Hospital,Heraklion, Greece.

Editors Note: This Manuscript was accepted for publication Augus t 15, 2012.

The authors have no funding, financial relationships, or conflictsof interest to disclose.

Send correspondence to Ioannis Vasileiadis, MD, Department of Otolaryngology, Head and Neck Surgery, Venizeleio-Pananeio GeneralHospital, Heraklion, Greece., 30, Alexandroupolis, Evros, Greece.E-mail: [email protected]

DOI: 10.1002/lary.23724

Laryngoscope 123: February 2013 Vasileiadis et al.: Symmetrical Lipomatosis of the Tongue422


2/4

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 takinginto consideration the postoperative shape and size of thetongue and avoiding injury to the lingual artery and lin-gual nerve. Following careful dissection, the lingualartery 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 sidesof the tongue symmetrically. The lesion was extended from the tipto the root of the tongue, causing moderate narrowing of theupper airway with reduction of the transversal diameter of thepharynx air column.

Fig. 3. Coronal magnetic resonance imaging view (T1 weighted)showed that the greater part of lingual muscles have beenreplaced by adipose tissue.

Fig. 4. Intraoperative view showing adipose tissue that invadeddeeply in the lingual muscles. No capsule formation was detected.[Color figure can be viewed in the online issue, which is availableat wileyonlinelibrary.com.]

Fig. 1. Bilateral enlargement of the tongue with its borders protrud-ing symmetrically (Mallampati class IV). [Color figure can be viewedin the online issue, which is available at wileyonlinelibrary.com.]



3/4

We placed four mattress sutures pressing the cephalic tothe caudal lingual surface. Consequently, we reduced thedead space and minimized the risk for hemorrhage.

Postoperatively, the volume of the tongue wasreduced and the load was also decreased on the existingmuscle fibers. The result was improvement of obstruc-tive sleep apnea syndrome symptoms, which was ourprimary objective.

Histopathological findings revealed lobules andsheets of mature adipocytes infiltrating betweenatrophic striated muscle fibers, confirming the diagnosisof symmetrical lipomatosis of the tongue (Fig. 5).

In the clinical follow-up, after 18 months thepatient had a minimal loss of articulation function,whereas the swallow function is normal. No obviousdeviation of the tongue to one side or atrophy wasobserved, and no regrowth of tumors was noticed 18months after surgery.

DISCUSSIONBenign tumors of the tongue are uncommon com-

pared with malignant tumors. 2 Lymphangioma,cavernous hemangioma, and neurofibromatosis are themost 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 asingle, pedunculated, superficial lesion. Enzinger andWeiss classified lipomas into five categories: 1) lipoma;2) variants of lipoma; 3) heterotopic lipoma; 4) infiltrat-ing or diffuse, neoplastic, or non-neoplastic proliferationof mature fat; and 5) hibernoma. 5

Although lipomas are considered to be benigntumors, 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 uncommonpathologic condition characterized by symmetric diffusegrowth of mature adipose tissue that commonly affectsthe neck and superior part of the trunk. 7 This condition,also known as Madelungs disease or Lanois-Bensaudesyndrome, has unknown etiology, but it is stronglyrelated to alcohol abuse. This uncommon disease pre-dominantly affects males between the ages of 30 and 60years 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 criteriaand thus was considered to be lipomatosis.

Katou et al. suggested that symmetric lipomatosisof the tongue and benign symmetric lipomatosis are dif-ferent entities, whereas other authors believed that theyare the same entity based on the similar histologic pat-terns seen in both conditions. 10 The most significantdifferences between the two conditions are indicated inTable I. Because the site of involvement and the age of appearance differ between lingual lipomatosis and be-nign symmetric lipomatosis, it still remainscontroversial whether they should be categorized as thesame entity.

The histological examination in cases of symmetriclingual lipomatosis reveals diffusely proliferated adiposetissue interspersed between the muscle fibers. It leads togradual 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 andcan be found in a variety of conditions, including congen-ital syndromes (Down syndrome, Beckwith-Wiedermannsyndrome), trauma (lingual hematoma), benign neo-plasms (lymphangioma, hemangioma), malignantneoplasms (squamous cell carcinoma), metabolic diseases(amyloidosis, glycogen storage disorders), and angioneur-otic edema. 10,11

Diagnosis of symmetrical lipomatosis of the tongueis established with clinical data, and computed tomogra-phy or magnetic resonance imaging may be useful.

Fig. 5. Photomicrograph showing lobules and sheets of matureadipocytes, diffusely infiltrating between few atrophic striatedmuscle 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 relationshipLingual involvement Rare AlwaysFat deposition in neck

and upper trunkTypical Never

Origin of patients Mediterranean Orientals



4/4

Computed tomography demonstrates a multifocal lesionwith density of adipose tissue invading the lingualmuscles bilaterally. Magnetic resonance imaging showsslightly ill-defined tissue of attenuation levels suggestiveof fat, invading bilaterally the lingual musculature, andreveals that the greater part of the lingual muscles havebeen replaced by adipose tissue. 12

The definitive diagnosis of lipomatosis is estab-lished after histopathological examination of thespecimen obtained at biopsy or glossectomy. Histologicalexamination reveals sheets and lobules of mature adipo-cytes infiltrating between muscle fibers. The musclefibers in areas show varying degrees of atrophy. Thenuclei of the fat cells appear bland, and the overlyingsquamous epithelium is intact and normal. 9

Because of the benign infiltrative nature of theunencapsulated fatty deposits, conservative surgicaltreatment is indicated to relieve functional impairment.Lipomatosis penetrates deep into the lingual tissue, andtherefore complete resection of the lesion is almostimpossible. Partial glossectomy is the surgical treatmentof choice because it reduces the size of the tongue andalso confirms histologically the deep lingual infiltrationof lipomatous tissue. 9,11 The volume of resection shouldbe carefully decided by taking into consideration thepostoperative shape and size of the tongue. After thesurgical removal of the masses, there is a significantimprovement in symptoms caused by the bulky tongue.

Regarding the management of the airway inpatients who undergo partial glossectomy, there is noabsolute indication to perform a tracheotomy in everypatient. Some authors suggest that temporary tracheot-omy should be done in patients who undergo partialglossectomy, when resection involves the floor of themouth and submandibular region. Certainly, the patient

should be informed preoperatively for the possibility of atracheotomy 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 lingualhematoma, and according to these parameters determinewhether a tracheotomy is necessary.

CONCLUSIONIntramuscular lipomatosis of the tongue is an

extremely rare phenomenon that can account for macro-glossia. Partial glossectomy is the treatment of choicebecause of the distinct possibility of a well differentiatedlipoma-like liposarcoma and the improvement of symptomsafter the tongues size reduction. Surgeons should be awareof the complication of lingual hematoma, especially when alarge portion of lingual tissue is resected bilaterally.

BIBLIOGRAPHY 1. Ogawa A, Nakamura H, Takahashi H. Benign symmetric lipomatosis of

the tongue: report of a case. J Oral Maxillofac Surg 1988;46:502504.2. Williams NS, Bulstrode CJK, OConnell PR. Bailey and Loves Short Prac-

tice of Surgery . 25th ed. London, UK: Hodder Arnold; 2008:741.3. Furlong MA, Fanburg-Smith JC, Childers EL. Lipoma of the oral and

maxillofacial region: site and subclassification of 125 cases. Oral Surg

Oral Med Oral Pathol Oral Radiol Endod 2004;98:441450.4. Bancroft LW, Kransdorf MJ, Peterson JJ, OConnor MI. Benign fattytumors: classification, clinical course, imaging appearance and treat-ment. Skeletal Radiol 2006;35:719733.

5. Enzinger FM, Weiss SW. Lipomas. In: Gay SM, Gery L, eds. Soft TissueTumors . 3rd ed. St. Louis. MO: Mosby-Year Book, Inc.; 1995:381431.

6. Kasper HU, Freigang B, Buhtz P, Roessner A. Lipoma-like liposarcoma of the tongue [in German]. Laryngorhinootologie 2000;79:5052.

7. Josephson GD, Sciafani AP, Stern J. Benign symmetric lipomatosis (Made-lungs disease) Otolaryngol Head Neck Surg 1996;115:170171.

8. Ghislain PD, Garzitto A, Legout L, Alcaraz I, Creusy C, Modiano P. Sym-metrical benign lipomatosis of the tongue and Launois-Bensaude lipo-matosis. Ann Dermatol Venereol 1999;126:147149.

9. Calvo-Garcia N, Prieto-Prado M, Alonso-Orcajo N, Junquera-Gutierrez LM.Symmetric lipomatosis of tongue: report of a case and review of the litera-ture. Oral Surg Oral Med Oral Pathol Oral RadiolEndod 1999;87:610612.

10. Katou F, Shirai N, Katsutoshi M, Satoh R, Satoh S. Symmetrical lipomato-sis of the tongue presenting as macroglossia. J Craniomaxillofac Surg1993;21:298301.

11. Lopez-Ceres A, Aguilar-Lizarralde Y, Villalobos Sanchez A, Prieto Sanchez

E, Valiente Alvarez A. Benign symmetric lipomatosis of the tongue inMadelungs disease. J Craniomaxillofac Surg 2006;34:489493.12. Norris DA, Doku C. Orofacial lipomas diagnosed by CT and MRI. J Am

Dent Assoc 1993;124:110115.


Documents

Laryngoscope Article