A Case of Symmetrical Lipomatosis of the Tongue Presenting As Macroglossia

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A Case of Symmetrical Lipomatosis of the Tongue Presenting as Macroglossia

Yoshimi Ishikawa, Yu Katada, Masahiro Enomoto, Kensuke Kawada, Yoshiyuki Okamoto and Mikio Ozawa

Department of Oral Surgery, Fujisawa City Hospital (Chief: Dr. Yoshimi Ishikawa)

Abstract: Symmetrical lipomatosis is characterized by symmetrical infi ltration of adipose tissue into the head, neck, or upper trunk. By contrast, oral involvement is extremely rare. The present report describes a case of symmetrical lipomatosis of the tongue (SLT) presenting as macroglossia. The patient was a 63-year- old Japanese male who developed progressive dysarthria secondary to enlargement of the tongue. The patient's medical history was notable for alcoholic hepatitis and hyperlipidemia. Marginal glossectomy was performed on both sides of the tongue to achieve volume reduction and restore normal speech. Histopathologic examination of the resected tissue revealed diffuse infi ltration with adipose tissue lacking a fi brous capsule. The patient's postoperative course was uneventful, and he remained free of recurrence or regrowth and functional defi cits at the 6-year follow-up time point.

Key words: Symmetrical lipomatosis, Macroglossia, Dysarthria, Alcoholic hepatitis, Hyperlipidemia

Introduction thria since developing painless swelling of the tongue Benign symmetrical lipomatosis (BSL) is character- border in 1993 was referred to our clinic on August 4, ized by symmetrical infi ltrating growth of non-encapsu- 1996. The patient was 165 cm tall and weighed 53 kg lated mature adipose tissue. The disease usually affects with no apparent nutritional problems. He had no fam- the neck and upper trunk, and tongue involvement is ily history of signifi cant disease, but had been treated comparatively rare. Symmetrical lipomatosis of the for alcoholic hepatitis and hyperlipidemia since 1988. tongue (SLT) was fi rst described by Desmond1 in 1944 Clinical examination revealed bilateral swelling of as a symmetrical and diffuse swelling of the bilateral the tongue and a smooth, yellowish, and lobulated mu- tongue border. Some investigators have suggested that cosa. Numerous soft and painless small masses were SLT may be a subtype of BSL, as both conditions share found along the tongue border. The dorsal mucosa was common pathological features2,3. The present report de- normal in color, and the papillae showed no sign of atro- scribes a case of SLT presenting as macroglossia. phy (Fig. 1). No tumor masses were identifi ed on the neck or at other sites of the body. Case report Computerized tomography (CT) scanning demonstrat- A 63-year-old Japanese male with progressive dysar- ed multilobular low-density areas with diffuse infi ltration into the tongue (Fig. 2). Magnetic resonance imaging (MRI) also showed high intensity nodules and Received 1/11/05; revised 4/25/06; accepted 10/2/06. Requests for reprints: Yoshimi Ishikawa, Department of Oral poorly defi ned adipose tissue infi ltrating into the tongue Surgery, Fujisawa City Hospital, 2-6-1 Fujisawa, Fujisawa-shi, muscle bilaterally (Fig. 3). Kanagawa 251-0052, Japan, Phone: ＋0466–25–3258, Fax: ＋0466–25–3258, E-mail: [email protected] Preoperative laboratory examination revealed high November, 2006 Symmetrical Lipomatosis of the Tongue 91

A B

Fig. 1 Preoperative photograph Fig. 4 Operative photographs show bilateral marginal glossectomy. shows bilateral swelling A: Preoperative view of the tongue. B: Postoperative view

levels of alanine aminotransferase (73 U/L), aspartate aminotransferase (65 U/L), alkaline phosphatase (353 U/L), γ-glutamyl transpeptidase (365 U/L), triglyceride (142 mg/dl), and β-lipoprotein (575 mg/dl). Biopsies of the right tongue border showed yellow adipose tissue in the tongue submucosa, and histopatho- logically suggested lipomatosis. The fi nal diagnosis was thus SLT presenting as macroglossia. To reduce the tongue volume, a marginal glossectomy was performed under general anesthesia on December 8, 1996. At the center of the tongue the width was 90 Fig. 2 CT scan shows a multilobular low-density area. mm. The tongue was excised at bilateral borders (each 20 mm in width) and decreased in width to 50 mm (Fig. 4A, 4B). Yellow adipose tissue was present beneath the submucosal layer with indistinct boundaries and without fi brous encapsulation. The weight of the specimens excised was 52 g in total. Histopathologic examination revealed diffusely proliferating adipose tissue consisting of mature lipocytes that were interspersed from the submucosa to the deep muscle layer. The adipose tissue had infi ltrated the muscle bundles. There was no capsule, and lipoblasts were absent (Fig. 5). In order to assess the patient's dysarthria, we evaluated his speech function one week after the operation using a listening test in which he was asked to speak Fig. 3 MR imaging shows high-intensity nodules Japanese words. The results of the listening test indi- and adipose tissue invading into the lingual muscle. cated that the patient's dysarthria had resolved. The patient's postoperative course was uneventful, and he 92 Oral Science International Vol. 3, No. 2 remained free of recurrence, regrowth and functional ous reports, BSL can affect the neck, cheeks, breast, up- defi cits at the 6-year follow-up time point. per arm, and axilla, but oral involvement is extremely rare. In terms of etiology, the disease is more common Discussion in middle-aged males with a history of excessive alcohol Benign lipomatous lesions can be classifi ed into fi ve use or liver disease5. The histopathologic features of different categories4 : (1) lipoma, (2) variants of lipoma, symmetrical lipomatosis are diverse, including mature (3) heterotopic lipoma, (4) infi ltrating or diffuse neoplas- lipocytes, infi ltration into the muscle and lack of a fi - tic or non-neoplastic proliferation of mature fat, or (5) brous capsule. Indeed, these features were present in hibernoma. The fourth group includes BSL, diffuse li- the current patient, supporting a diagnosis of SLT. Al- pomatosis, and pelvic lipomatosis. According to previ- though SLT was similar to diffuse lipomatosis and in- tramuscular lipoma of the tongue in terms of histopathological diagnosis, a differential diagnosis may be made based on clinical fi ndings. Calvo-Garcia6 reported that BSL is common in Medi- terranean people, while SLT is more frequent in the Japanese population. Of the 11 cases of SLT reported in the Japanese population2,3,7–12, all affected individuals were middle-aged, non-obese males, and 9 of 11 patients had a history of alcoholic hepatitis or alcoholism. Fur- ther, 10 of 11 cases had macroglossia, and 5 of 11 patients developed dysarthria secondary to enlargement of the tongue. All 11 patients had tongue involvement without lesions on other body areas (Table 1), despite the potential similarities between SLT and BLT. In- deed, recent reports11,12 cited the different sites of involvement as evidence that SLT and BSL are distinct Fig. 5 Histopathological examination entities. Thus, although SLT has been previously de- shows diffusely proliferated 2,3,8,9 adipose tissue consisting of scribed as a variant of BSL , this condition is likely a mature lipocytes (HE ×50). separate disease, particularly in the Japanese popula-

Table 1 Summary of reported cases of SLT

Case No. Sex Age Obesity Alcoholism Macroglossia Dysarthria Body lesion

14 M67 －＋＋＋－ 2 5 M71 －＋＋＋－ 3 6 M61 －－＋＋－ 4 6 M71 －±＋－－ 5 7 M66 －＋＋＋－ 6 7 M78 －＋－－－ 7 8 M74 －－＋－－ 8 9 M63 －＋ + －－ 9 10 M62 －＋±－－ 10 11 M67 － ++－－ 11 M63 － +++－

(＋: positive －: negative ±: vague) No. 11 is the present case. November, 2006 Symmetrical Lipomatosis of the Tongue 93 tion. Oral Maxillofac Surg 46: 502–504, 1988. Surgical resection is the sole mode of treatment for 3. Yoshimura Y., Miyakawa A., Kotani M., Yoshimura H., Od- macroglossia. For cases with severe complications or no ajima T., Yamamoto E., and Kohama G.: A case of macro- functional disorder, only biopsies were performed for di- glossia caused by benign symmetrical lipomatosis. Jpn J Oral Maxillofac Surg 34: 1061–1065, 1988. agnosis and such patients received only follow-up treat- 4. Enginger F.M. and Weiss S.W.: Soft tissue tumors. Mosby ment. Thus, marginal glossectomy should be performed Co, St Louis, 1983, pp.199–241. with particular attention to either maintain or restore 5. Shuller F.A., and Graham J.K.: Benign symmetrical lipo- function. Because the average tongue width of Japa- matosis (Madelung's disease). Plast Reconst Surg 57 : 13 nese adult males is 49 mm , the patient in the present 662–665 1976. report underwent resection of 40 mm of tissue to reduce 6. Calvo-Garcia N., Pietro-Pardo M., Alonso-Oracajo N., and tongue size from 90 mm to 50 mm. This resulted in res- Junquera-Guiterrez L.M.: Symmetric Lipomatosis of the olution of dysarthria, which we evaluated one week af- tongue, report of a case and review of the literature. Oral ter the operation by listening to his pronunciation of Surg Oral Med Oral Pathol 87: 610–612, 1999. some Japanese words. 7. Katou F., Shirai N., Motegi K., Satoh R., and Satoh S.: The indistinct margin between the adipose tissue and Symmetrical lipomatosis of the tongue presenting as mac- the tongue in patients with SLT makes complete resec- roglossia. Report of two cases. J Cranio Maxill Surg 21: 298–301, 1993 tion problematic. Although this suggests that patients 8. Honda Y., Ozeki S., Oobu K., Goto K., Yoshiura K., and undergoing surgical management of SLT may have a Ohishi M.: Two cases of benign symmetrical lipomatosis of relatively high rate of recurrence or regrowth, there had the tongue. Jpn J Oral Maxillofac Surg 44: 342–344, been no reports of a recurrence described in the litera- 1998. 11 ture. Tohya reported a slow-growing case after a biop- 9. Inoda H., Yamamoto G., and Yoshitake K.: A case of benign 14 sy, and Tizian reported one case of BSL that trans- symmetric lipomatosis of the tongue. Jpn Oral Diag/Oral formed to liposarcoma. Regardless, postoperative Med 13: 206–209, 2000. follow-up is important to assure that recurrence or re- 10. Kurokawa H., Kimura K., Miura K., and Takeda S.: Lipo- growth does not occur and that tongue function and matosis of the tongue presenting as a macroglossia: Re- speech has normalized. port of a case. Jpn Oral Diag/Oral Med 13: 451–454, 2000. 11. Tohya T., Kawahara K., Hotta F., Shirozu T., and Sano D.: Conclusion Bilateral lipomatosis of the tongue: Report of a case. J Ai- We reported a rare case of SLT presenting as macro- indai Shi 39: 519–523, 2001. glossia. The present case was clinically and histopatho- 12. Sato Y., Takahashi E., Takahashi Y., Sakamoto I., Omura logically typical. Marginal glossectomy was effective in K., and Okada N.: A case of symmetric lipomatosis of the restoring normal tongue function and speech. tongue. Jpn J Oral Maxillofac Surg 50: 297–300, 2004. 13. Kaneko U.: Nippon Jintai Kaibougaku Vol 2. Nanzando, References Tokyo, 1970, pp.35–44. 1. Desmond A.D.: A case of lipomatosis of the tongue. Br J 14. Tizian C., Berger A., and Vyukoupil K.F.: Malignant de- Surg 35: 210, 1947. generation in Madelung's disease (benign lipomatosis of 2. Ogawa A., Nakamura H., and Takahashi H.: Benign sym- the neck). Br J Plast Surg 36: 187–189, 1983. metrical lipomatosis of the tongue: Report of a case. J