M. Shibata et al.

protein revealed a monoclonal
IgG with a κ chain. Chemotherapy with melphalan, cyclophosphamide, predonisolone andvincristine, and local radiotherapy for the mandibular mass
were administered. The chemotherapy was replaced with melphalan and predonisolone. The
patient was discharged following the disappearance of themass. But 8 months later pancytopenia was noted. The patient was then referred to a hematologist for chemotherapy
with a diagnosis of acute myelogenous leukemia. However, thepatient died of acute renal insufficiency several months later.

Fig. 4. Gingival mass located on the lower right 1st molar area (Patient 4).

tion, paresthesia of the lower lip, pain, bleeding andfracture of the jawbone, macroglossia and radiolucent lesions (Yaegaki et al., 1983). Osteolytic
lesions are more frequent in the mandible than in
the maxilla (Lee et al., 1996), especially in theposterior teeth region, ramus and condylar process,presumably because of greater hematopoietic
activity in these areas (Lambertenghi-deliliers et al.,Discussion
Although uncommon, an initial presentation ofmultiple myeloma may reveal oral or maxillofacialsymptoms. These include swelling, mass forma-Fig. 5. Radiograph of a skull showing multiple “punched-out” osteolytic lesions (Patient 4).

80

Oral multiple myeloma

1988; Lee et al., 1996). Oral lesions in patients withmultiple myeloma are not uncommon, but multiple
myeloma is often overlooked. Because the symptoms are various, it is very difficult to diagnose
multiple myeloma in the oral and maxillofacialregion. Amyloidosis as an additional complication
has been reported in 6% to 15% of patients withmultiple myeloma (Erich et al., 1986). Amyloid involvement of oral tissue is rather rare, and thetongue is the most encountered subsite (Rutger etal., 2002). Macroglossia, usually seen in primary
amyloidosis, occurs in approximately 20% ofpatients (Smith and Speculand, 1985; Yusa et al.,2001). It seems that almost all secondary amyloidosis originates from reactive systematic conditions(Mardinger et al., 1999). Oral amyloidosis often
comes along with various manifestations, such asmultiple soft nodules, hemorrhagic-type raised
lesions, rubbery swellings, with a normal overlying
mucosa, with or without multiple nodules on thelateral borders of the tongue or with prominent
crenation (Muto et al., 1991). In the present study,
submental swelling, tongue stiffness, macroglossia
and dysphagia were noted in Patient 1, and disturbed lingual mobility, induration of the tongue anddysphagia, in Patient 3. But there were no other abnormalities in the oral and maxillofacial region.
The tongue is the organ which is comparatively
easy to observe. Oral amyloidosis seems to be an
important key in leading to a diagnosis of multiple
myeloma. In Patient 1, we performed partial glossectomy to alleviate functional oral difficulty. The
survival time of patients with amyloidosis associated with multiple myeloma is shorter than those
without amyloidosis (Eddie et al., 1994). Patientswith primary amyloidosis should be treated palliatively because of their short survival periods.
Medical history and biochemical and hematologic
findings would be helpful in diagnosing multiple
myeloma in case of unexplained swelling and nodules in the oral region, even if radiography shows
no osteolytic lesions. Furthermore, when a diagnosis of tongue amyloidosis is confirmed, dentistsshould start searching for underlying disorders,such as multiple myeloma.

References
1 Keung Y, Yung C, Wong J, Shah F. Association oftemporal arteritis, retinal vasculitis and xanthomatosis with multiple myeloma: case report and literature review. Mayo Clin Proc 1998;73:657–60.
2 Lambertenghi-Deliliers G, Bruno E, Cortellezzi A,Fumagalli L, Morosini A. Incidence of jaw lesions
in 193 patients with multiple myeloma. Oral Surg
Oral Med Oral Pathol 1988;65:533–537.
3 Lee SH, Huang JJ, Pau WL, Chan CP. Gingival
mass as the primary manifestation of multiple myeloma. Oral Surg Oral Med Oral Pathol 1996;82:75–79.
4 Mardinger O, Rotenberg L, Chaushu G, Taicher S.
Surgical management of macroglossia due to primary amyloidosis. Int J Oral Maxillofac Surg 1999;
28:129–131.
5 Muto T, Sato K, Gregory J. Multiple nodules of thelip, cheeks, and tongue. J Oral Maxillofac Surg
1991;49:1003–1006.
6 Raubenheimer EJ, Dauth J, de Coning JP. Multiple
myeloma presenting with extensive oral and perioral
amyloidosis. Oral Surg Oral Med Oral Pathol 1986;
61:492–497.
7 Reinish EI, Raviv M, Srolovitz H, Gornitsky M.
Tongue, primary amyloisosis, and multiple myeloma. Oral Surg Oral Med Oral Pathol 1994;77:121–125.
8 Rutger I, Martijn R, Peter C, Theo M. Amyloidosisof the tongue as a paraneoplastic marker of plasma
cell dyscrasia. Oral Surg Oral Med Oral Pathol
2002;94:444–447.
9 Smith A, Speculand B. Amyloidosis with oral involvement. Br J Oral Maxillofac Surg 1985;23:435–444.
10 Yaegaki K, Kameyama T, Toyofuku S, Tanaka K,Yoshitake M, Kawashima R, et al. Clinicostatistical study of the multiple myeloma discoveredfrom oral manifestation. Nippon Koku Geka Gakkai
Zasshi 1983;29:2266–2272 (in Japanese).
11 Yusa H, Yoshida H, Kikuchi H, Onizawa K.
Dialysis-related amyloidosis of the tongue. J Oral
Maxillofac Surg 2001;59:947–950.

Received June 16, 2003; accepted July 31, 2003
Corresponding author: Masami Shibata81