A case of symmetrical lipomatosis of the tongue presenting as macroglossia

Published online (EP) 18 June 2016 - Ann. Ital. Chir. 1

Ann. Ital. Chir.Published online (EP) 18 June 2016

pii: S2239253X16025627www.annitalchir.com

Pervenuto in Redazione Marzo 2016. Accettato per la pubblicazioneMaggio 2016.Correspondence to: Maria Giulia Cristoforo, Maxillo Facial Surgery,University “Magna Graecia”, Viale Europa loc. Germaneto, 88100Catanzaro Italy (e-mail: cristofaro@unicz.it)

Maria Giulia Cristofaro, Walter Colangeli, Umberto Riccelli, Mario Giudice

Department of Oral and Maxillo Facial Surgery (Chairmen: Prof. M. Giudice) University Magna Graecia of Catanzaro, Italy

A case of symmetrical lipomatosis of the tongue presenting as macroglossia.

Lipomas are the most common benign soft tissue mesenchymal tumours composed of mature adipose tissue. They areuncommon in the oral and maxillofacial regions, with 15-20 % of cases involving the head and neck region and lessthan 5% of all benign oral lesions. Multiple symmetric lipomatosis is rare and characterized by diffuse growth andnonencapsulated lipomas. It is usually found in the posterior neck and upper trunk and they are relatively infrequenton the oral and maxillofacial regions like Madelung disease. In the report, we describe a rare case of symmetrical lipo-matosis of tongue with OSAS and Dysartria. This lesions were resected under general anesthesia. Intraoperative findingsrevealed only adipose tissues with replacement of lingual muscles and no capsulation. The lesion was finally diagnosedas symmetric lipomatosis of the tongue based on clinical radiological and histologic examination. SLT (Symmetrical lipo-matosis of the tongue) is an extremely rare case that appears like a macroglossia. Partial glossectomy is the treatment ofchoice because of the improvement of symptoms and the low rate of recurrence.

KEY WORDS: Macroglossia, Oral lipoma, Tongue lipomatosis

floor, buccal sulcus and vestibule, palate, lip and gingi-va. Multiple involments are rare. A few cases of bilater-al symmetrical lipomatosis (BLS) have been reported inliterature and this desease usually affects the neck andupper trunk with only rare involvement of the tongue.Symmetrical lipomatosis of the tongue (SLT) is a sym-metrical and diffuse swelling of the bilateral tongue bor-der and some investigation have suggested that SLT maybe a subtype of BLS 2,3.We report a rare case of SLT infiltrating grown of non-encapsulated mature adipose tissue, presenting asmacroglossia with obstructive sleep apnea syndrome(OSAS) and dysarthria.

Case Report

A 68-year-old Italian male was referred to our depart-ment with gradually progressive painless slowly growingand worsening swelling on both lateral borders of thetongue that had been present for over 5 years.

Introduction

Lipomas are the most common benign soft tissue mes-enchymal tumours composed of mature adipose tissue.They are uncommon in the oral and maxillofacial regions,with 15-20% of cases involving the head and neck regionand less than 5% of all benign oral lesions 1. Lipoma oforal cavity may occur in any site but the cheek is thecommonest site of occurence followed by tongue, mouth

READ-ONLY

COPY

PRINTIN

G PROHIB

ITED

The patient had tongue dysmotility and dysarthria, upperairway obstruction with OSAS, dysfagia and psycosocialimpairment for the lost 8 months; he denied other neu-rosensory diseases, including dysgeusia, motor or auto-nomic neuropathy. The patient was not obese and didnot abuse alcohol; there was no family history of illness. Clinical examination showed symmetric bilateral tonguemasses: the size of the tongue was 15 cm x 9 cm x 8cm with a soft and elastic consistency (Figg. 1-2). Theoverlying mucosa was normal in color and texture withno ulceration or inflammation. On palpation the lesionswere rubbery and no fluctuant. There were no cervicalmasses and no other swellings anywhere in the body. Magnetic resonance imaging (MRI) was performed fordifferential diagnosis and preoperative evaluation of thelesion. T1-weighted images revealed a widespread high

signal intensity of the tongue, while fat-saturation T1-weighted images revealed low signal intensity in the sameregion (Fig. 3). Polysomnography revealed AHI index >15 >30 and oxy-gen saturation was > 80%.Polysomnography were we detect: air flow, blood oxy-gen levels, body position, brain waves (EEG), breathingeffort and rate electrical activity of muscles, eye move-ment, heart rate. Other investigation included complete blood tests exam-ination revealed that the full blood count, electrolytes,liver function test and blood glucose were all normal,only the cholesterol was elevated.An incision biopsy showed lobules of mature adipose tis-sue in the tongue submucosa suggested lipomatosis. The lesion was removed under general anesthesia and weperformed a glossoplastic reduction by variant ofDingman e Grabb glossectomy a peripheral excision cat-egory and suffer from the drawback of reducing thebulk of the tongue at the periphery while leaving thecentre and base bulky. The operative technique used inthis case is based on the submucosal vascular plexus,which allows excision of an intramuscular wedge toreduce the bulk of the tongue, along with the longi-tudinal wedge excision to reduce the width, and thetransverse wedge to reduce the length. This helps ingiving a uniform reduction of the macroglossia in allthree dimensions. While reducing the bulk of thetongue it spares the papillae at the dorsum which areresponsible for salty taste, the papillae at the sides whichare responsible for sour taste, and the papillae at thebase for bitter taste.

PATHOLOGICAL FINDING

Surgical specimen was constituted of two excision respec-tively of cm 10x4x6 and cm 7x3x2 (Fig. 4). The cutsurface showed multiple yellowish areas, with soft con-sistence.

M. G. Cristofaro, et al.

2 Ann. Ital. Chir. - Published online (EP) 18 June 2016

Fig. 1 and 2: A clinical view.

Fig. 3: Magnetic Resonance imaging (T1 weighted).

READ-ONLY

COPY

PRINTIN

G PROHIB

ITED

Histological examination showed a normal squamousepithelium overlying a mature adipose tissue lesion, thatreplaces the normal smooth muscle tissue. No pleomor-fism or atypia was observed. Albeit lipomatous cellsshowed a vague variety of size, no evidence of nuclearatypia or mitotic figures was detected. No multivacuo-lated lipoblasts were seen (Fig. 5).An immunohistochemical analysis was performed againstS-100 (Novocastra clone S1/61/69 BOND ready to use)Vimentin (Dako Anti-Vimentin clone V9, IS630, readyto use), Actin (Novocastra clone HHF35 BOND readyto use), and Desmin (Novocastra clone DE-R-11,BOND ready to use), to define the cells population.Negative controls were achieved by substitution of theprimary antibodies with normal rabbit serum. Blood ves-sels, normal tissue macrophages and muscle cells wereused as positive controls. The fat tissue was positive forS-100 and Vimentin, while the residual muscular com-ponent interspersed into the adipose tissue, was positivefor Desmin, Actin and Vimentin. The morphological and immunohistochemical analysiswere consistent with a diagnosis of multiple lipomas ofthe tongue / lipomatosis of the tongue.The patient’s postoperative course was uneventful andwithout functional deficits. The mobility of the tongueis not affected as the tongue base and frenulum are nottouched (Fig. 6).In order to assess the patient’s dysarthria, OSAS and dys-phagia, we evaluated his speech function on two weeksafter surgery using a listening test in which he was askedto speak Italian words. The listening test indicated thatthe patient’s dysarthria had resolved. Regarding dyspha-gia and OSAS, his tongue was now accommodated com-fortably within the oral cavity with significant improve-ment of deglutitionPost-operative Polysomnography revealed, also, normalsaturation and no more apnea events. He was well withno local recurrence and regrowth after 36-month follow-up period.

Discussion

Lipomas is a benign tumour composed of adipose tis-sue, and is the most common form of soft tissue tumour.Presentation as multiple tumours is infrequent, some areassociated with inherited disorders, for instance as inGardner’s or Bournville Syndrome, multiple lipomatosisor macroglossia 4,5. Enzinger and Weiss classified benign lipomatous lesionsinto 5 categories: lipoma, variants of lipoma, heterotopiclipoma, infiltrating or diffuse neoplastic or non-neoplas-tic proliferation of mature fat, hibernoma; the fourthgroup includes also BSL, diffuse lipomatosis and pelviclipomatosis 6.Lipoma of the tongue is rare and often presents as a sin-gle, superficial, peduncolated or sessil lesion, which accounts

Published online (EP) 18 June 2016 - Ann. Ital. Chir. 3

A case of symmetrical lipomatosis of the tongue presenting as macroglossia.

Fig. 5: H&E – 50X magnification. A panoramic view of the lesion,please note the lipomatous tissue substituting the normal muscularcomponent, that appears scanty and atrophic. Overlying squamousepithelium appear normal.

Fig. 6: Postoperative view.

Fig. 4: Surgical specimen constituted of two excision respectively ofcm 10x4x6 and cm 7x3x2.

READ-ONLY

COPY

PRINTIN

G PROHIB

ITED

for 0.3% of tongue neoplasms, is a benign condition andtypically described as well- circumscribed, submucosal, withless than 1 cm swelling, and located on the lateral edge ofthe anterior two-thirds of the tongue surface. Lipomatosis is characterized by multiple sides of involve-ment, include mature lipocytes, infiltration into the mus-cle and absence of encapsulation of the adipose tissue.Benign symmetrical lipomatosis (BSL) can affect theneck, cheeks, breast, upper arm and axilla, but oralinvolvement is extremely rare. BSL predominant occursin people with Mediterranean ethnicity and is associat-ed with alcoholism and obesity. Instead Simmetrical lipo-matosis of the tongue (SLT), first reported by Desmondin 1944, involves only the tongue and was reported pre-dominantly in Asian people in absence of obesity andsometimes also without a history of alcoholism 7.Although SLT is been previously described as a variantof BSL, this condition is likely separate dese 8,9.Our case of SLT is particular because the patient wasnot an Asian man and had not an history of alcoholismand obesity. Only the clinical and histopathologic fea-tures are common with other reported cases of SLT inliterature. Microscopically, it is composed of matureadipocytes; however, in 20% of cases, it demonstrateshistologic variants that includes spindle cell lipoma, pleo-morphic lipoma, angiolipoma, fibrolipoma, myxoid lipo-ma, and atypical lipoma.

Preoperative magnetic resonance imaging is useful forthe management plan. The strikingly high intensity sig-nal on both T1- and T2-weighted images are suggestiveof lipoma and it further delineates the extent of tumorinvolvement. Lipomatosis of the tongue had an ill defined marginbecause of infiltration of muscles 10. A lipoma differsfrom normal body fat in that its lipid is not availablefor metabolism and it is surrounded by a capsule. Inaccording with others Authors (Chikui) in our case weobserved high signal intensity masses detected bilateral-ly in the tongue and curved lines of low signal intensi-ty running bilaterally from the lingual septum (the rem-nants of the internal lingual muscles) 11,12.The treatment for the SLT is surgical resection whenassociated with functional disorder (obstructive sleepapnea, dysarthria, etc.), with the aim to restore function.Lipomatosis penetrates deep into the lingual tissue there-fore complete resection of the lesion is almost impossi-ble so partial glossectomy is the surgical treatment ofchoice 13.The local recurrence of intramuscular lipoma has beenreported to range from 19 to 62,5% 5 due to the dif-ficulty of radical surgical excision. No recurrence case inthe tongue have been reported in literature, regardlesspost-operative follow-up is raccomanded.Surgical excision provides more tissue for examinationthan fine needle aspiration and sometimes the only wayto make certain diagnosis and to exclude other pathol-ogy for differential diagnosis of macroglossia: Congenital

(Down’s syndrome, Cretinism, Beckwith-Wiedemannsyndrome or exomphalos, Macroglossia or gigantism syn-drome); Inflammatory (Lymphoedema); Trauma (Lingualhaematoma); Neoplasm (Benign like neurofibroma, hae-mangioma, lymphangioma or malignant like squamouscell carcinoma); Metabolic disorders (Glycogen storagedisorders, Amyloidosis); Endocrine (Acromegaly,Myxoedema); Miscellaneous (Angioneuroticoedema,Madelung Disease) 7-10.

Conclusion

SLT is an extremely rare case that appears like amacroglossia. Partial glossectomy is the treatment ofchoice because of the improvement of symptoms and thelow rate of recurrence described in the literature. Ourcase was clinically and histopathologically typical and thetreatment performed was effective in restoring normaltongue function and speech.

Riassunto

I lipomi sono i più comuni tumori mesenchimali deitessuti molli, composti da tessuto adiposo maturo. Sonorari nell’ambito orale e nella regione maxillo-facciale, conil 15-20% dei casi riguardanti il capo e la regione delcollo, e rappresentano meno del 5% di tutte le lesionibenigne del cavo orale.La lipomatosi multipla e simmetrica è rara, ed è carat-terizzata da lipomi non capsulati ed a crescita diffusa. Silocalizzano abitualmente posteriormente nel collo e neltronco prossimale, e sono relativamente rari a livello ora-le e maxillo-facciale come nel caso della malattia diMadelung.In questa presentazione si descrive un raro caso di lipo-matosi simmetrica della lingua con OSAS (ObstructionSleep Apnea Syndrome) e disartria. La lesione è stataasportata sotto anestesia generale. Il reperto intraopera-torio ha dimostrato soltanto tessuti adiposi senza capsu-la e sostituzione del muscoli linguali. La diagnosi finaleè stata di lipomatosi della lingua in base ai reperti cli-nici, radiologici ed istologici.La LSL (Lipomatosi simmetrica della lingua) rappresen-ta un caso estremo che si presenta clinicamente comemacroglossia. Il trattamento di scelta è una glossettomiaparziale che determina un miglioramento della sintoma-tologia ed una bassa incidenza di recidiva.References

1. Kaur RP, Kler S, Bhullar A: Intraoral lipoma. Dent Res J(Isfahan), 2011; 8(1):48-51.

2. Ishikava Y, Katada Y, Enomoto m, Kawada K, Okamoto Y,Ozawa M: A case of symmetrical lipomatosis of the tongue presentingas macroglossia. Oral Science International, 2006; 90-93.

3. Fregnani ER, Pires FR, Falzoni R, Lopes MA, Vargas PA:

M. G. Cristofaro, et al.

4 Ann. Ital. Chir. - Published online (EP) 18 June 2016

READ-ONLY

COPY

PRINTIN

G PROHIB

ITED

Lipomas of the oral cavity: Clinical findings, histological classificationand proliferative activity of 46 cases. Int J Oral Maxillofac Surg,2003; 32:49-53.

4. Cristofaro MG, Giudice A, Amantea M, Riccelli U, GiudiceM: Gardner’s syndrome: A clinical and genetic study of a family.Oral Surg Oral Med Oral Pathol Oral Radiol, 2013; 115(3).

5. Naruse T, Yanamoto S, Kawano T, Yoshitomi I, Yamada S-I,Kawasaki G, Fujita S, Ikeda T, Umeda M: Intramuscolar lipoma ofthe tongue: Report of a case complicated with diffuse lipomatosis.Journal of Oral and Maxillofacial Surgery, Medicine and patholo-gy, 2012; 237-40.

6. Enzinger FM, Weiss SW: Lipomas. In Gay SM, Gery L, edi-tors. Soft tissue tumours. III ed St. Loius: Mosby-year Book, Inc,1995; 381-431.

7. Desmond AD: A case of lipomatosis of the tongue.Br J Surg, 1947; 35:210.

8. Calvo-García N, Prieto-Prado M, Alonso-Orcajo N, Junquera-Gutierrez LM: Symmetric lipomatosis of the tongue: report of a caseand review of the literature. Oral Surg Oral Med Oral Pathol OralRadiol Endod, 1999; 87(5):610-12.

9. Lee SH, Yoon HJ: Bilateral asymmetric tongue classic lipomas.Oral Surg Oral Med Oral Pathol Oral Radiol, 2012; 114(1):e15-8.

10. Chikui T, Yonetsu K, Yoshiura K, Miwa K, Kanda S, OzekiS, Shinohara M: Imaging findings of lipomas in the orofacial regionwith CT, US, and MRI. Oral Surg Oral Med Oral Pathol OralRadiol Endod, 1997; 84(1):88-95.

11. Keskin G, Ustundag E, Ercin C: Multiple infiltrating lipomas ofthe tongue. J Laryngol Otol, 2002;116:395-97.

12. Vasileiadis I, Mastorakis G, Ieromonachou P, Logothetis I:Symmetrical lipomatosis of the tongue-a rare cause of macroglossia:Diagnosis, surgical treatment, and literature review. Laryngoscope,2013; 123(2):422-25.

13. Katou F, Shirai N, Motegi K, Satoh R, Satoh S: Symmetricallipomatosis of the tongue presenting as macroglossia. Report of two cas-es. J Craniomaxillofac Surg, 1993; 21(7):298-301.

Published online (EP) 18 June 2016 - Ann. Ital. Chir. 5

A case of symmetrical lipomatosis of the tongue presenting as macroglossia.

READ-ONLY

COPY

PRINTIN

G PROHIB

ITED