The manifestations of multiple myeloma are protean and related to bony osteolytic lesions, and to medullar and renal insufficiency. We report a patient who presented with otalgia as the inaugural symptom of multiple myeloma. Local irradiation combined with systemic chemotherapy led to the disappearance of the temporal bone mass and the accompanying symptoms. To date, 24 months after the diagnosis, the patient is still in remission. The literature on otological involvement in multiple myeloma is reviewed. Symptoms are non-specific and include hearing loss, tinnitus, dizziness, facial paralysis, and otalgia. The diagnosis of multiple myeloma should be considered in the presence of a temporal bone mas

Abele, R.

Allal, A. S.

Dulguerov, P.

Kurt, A.-M

Montandon, P.

Quinodoz, D.

Ruefenacht, D.

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The Journal of Laryngology and OtologyMay 1998, Vol. 112, pp. 469-471Clinical RecordsMultiple myeloma presenting with external ear canal massD. QUINODOZ, M.D.*, P. DULGUEROV, M.D.*, A.-M. KURT, M.D.t, D. RUEFENACHT, M.D4, R. ABELE, M.D.**A. S. ALLAL, M.D4 P. MONTANDON, M.D.*AbstractThe manifestations of multiple myeloma are protean and related to bony osteolytic lesions, and to medullar andrenal insufficiency. We report a patient who presented with otalgia as the inaugural symptom of multiplemyeloma. Local irradiation combined with systemic chemotherapy led to the disappearance of the temporalbone mass and the accompanying symptoms. To date, 24 months after the diagnosis, the patient is still inremission.The literature on otological involvement in multiple myeloma is reviewed. Symptoms are non-specific andinclude hearing loss, tinnitus, dizziness, facial paralysis, and otalgia. The diagnosis of multiple myeloma shouldbe considered in the presence of a temporal bone mass.Key words: Ear; Multiple myeloma; Pain; Drug therapy; RadiotherapyIntroductionMultiple myeloma (MM) accounts for 10 per cent ofhaematological malignancies and one per cent of allcancer-related deaths (Alexian and Dimopulos, 1994;Bataille and Harousseau, 1997). MM is a neoplasticproliferation of monoclonal plasma cells within thehaematopoietic bone marrow. Symptoms are related tothese osteolytic lesions (skeletal pain, pathological frac-tures, hypercalcaemia), to the replacement of bonemarrow by the neoplastic process (immunosuppressionresulting in increased bacterial infections, anaemia, bleed-ing), and to renal insufficiency.The diagnosis of MM requires at least two of thefollowing criteria (Barlogie, 1995; Selby and Gore, 1995):1) serum or urinary monoclonal immunoglobulin whichcan be composed of the entire immunoglobulin protein, ofcomplete light chains, or various fragments; 2) bone-marrow infiltration by large and immature plasma cells;3) osteolytic lesions. Several staging systems have beendescribed (Barlogie, 1995; Selby and Gore, 1995).Since MM is a systemic illness, involvement of thetemporal bone is possible. Nevertheless, otological symp-toms in MM have rarely been described. A case is reportedin which the presenting signs of MM were otological. Theliterature on temporal bone involvement in MM isreviewed.Case reportA 70-year-old male, without previous medical history,consulted because of progressively worsening right earacheof six months duration. He did not complain of hearingloss, tinnitus, or dizziness.Clinical examination revealed a right external ear canalpartially obstructed by a subcutaneous, smooth mass,located on the posterior bony-cartiligenous junction. Thetympanic membrane was intact. Tuning fork testing wasnormal and symmetrical. A right peripheral facial paresiswas present.A temporal bone computed tomography (CT) scan(Figure la) showed an osteolytic lesion, 2.5 cm in diameter,enhanced by contrast material, and replacing the rightmastoid. The mass eroded the posterior canal wall andprotruded in the posterior fossa. A second osteolytic lesionwas found at the level of the right occipital condyle.These lesions exhibited homogeneous enhancement withgadolinium on magnetic resonance imaging (MRI)(Figure lb).A biopsy of the external auditory canal mass wasperformed under local anaesthesia. The pathology showeda proliferation of atypical plasma cells compatible with amultiple myeloma (Figure 2) and immunohistochemicalstaining was positive for the Kappa light chains andnegative for the Lambda light chains (not shown).Further investigations, including a skull and skeletalX-rays, a protein electrophoresis, an immunoelectro-phoresis and a bone marrow biopsy, permitted thediagnosis of IgG-Kappa multiple myeloma, without renalfailure, anaemia, or hypercalcaemia. The disease was thusstaged as Ilia.The patient was treated with Alkeran and Prednisone,and local radiation therapy delivered through two lateralweighted fields for a total dose of 30 Gy (6 MeV photons;10 fractions of 3 Gy).Three months after this treatment, the patient wasgreatly improved. The otalgia and facial paralysis resolved.The external auditory canal lesion regressed completely.From the Departments of Otolaryngology - Head and Neck Surgery*, Pathologyt, Radiology ,^ and Medicine**, University CantonHospital, 24 Rue Micheli-du-Crest, 1211 Geneya 14, Switzerland.Accepted for publication: 4 March 1998.469https:/www.cambridge.org/core/terms. https://doi.org/10.1017/S0022215100140800Downloaded from https:/www.cambridge.org/core. University of Basel Library, on 11 Jul 2017 at 08:24:28, subject to the Cambridge Core terms of use, available at470 D. QUINODOZ, P. DULGUEROV, A-M. KURT, D. RUEFENACHT, R. ABELE, A. S. ALLAL, P. MONTANDON(a) (b)FIG. 1Initial axial CT and MRI scans. The CT scan, after i.v. application of contrast material, is filmed with soft tissue windows (Figure la).The homogenous enhancing temporal bone mass presents as an osteolytic lesion of the right mastoid and sino-dural angle platewithout signs of periosteal reaction or invasion of the sigmoid sinus. A second lesion with similar radiological characteristics ispresent at the base of skull near the foramen magnum. The Tl-weighted MRI image with gadolinium-DPTA (Figure lb) alsoexhibits a pattern of homogeneous enhancement after contrast application.To date, 24 months after the diagnosis, the patient does notpresent any local or general complaints and has resumedhis normal activities.DiscussionA variety of tumours, either primary or secondary, caninvolve the temporal bone (Hyams, 1987; Abemayor et al.,1988; Kemink et al., 1993). Symptoms are not specific andtend to vary with the location of the disease process andthe invaded anatomical structures (Schuknecht, 1993).While physical examination, audiological and vestibularinvestigations are warranted, they are rarely diagnostic andradiological studies such as thin section CT or MRI scansFIG. 2The lesion is composed of a dense cellular infiltrate, mainlycomposed of plasma cells arranged in cords, separated by afibro-vascular stroma. (H & E; X 400).are required to determine the involved structures and helpin selecting the surgical approach for excision or biopsy(Jackler and Parker, 1992).Involvement of the temporal bone has been reported tooccur in the terminal stage of MM. However, at this stage,otological symptoms are overshadowed by the manifesta-tions of diffuse skeletal disease (Schuknecht, 1993).In our case, the presenting signs of MM were related toan involvement of the temporal bone. The mastoid massresulted in otalgia and a facial palsy without any otherotological or systemic symptoms.In the literature, only a few individual case reportsdescribe a symptomatic MM temporal bone involvement(Table I). In 1979, Lavine et al. presented a patient alreadydiagnosed with MM, who complained of dizziness, hearingloss and tinnitus. Three other cases with otologicalsymptoms in patients with diffuse signs of MM havebeen published (Shone, 1985; Keay, 1988; Chevalier et al.,1992).Marks and Brookes in 1985 were the first to describe apatient with left deafness, tinnitus, facial palsy, andintermittent vertigo who had an isolated mastoid lesion.Biopsy of the external auditory canal lesion led to thediagnosis of MM. Funakubo and Kikuchi (1994) describe asimilar case. None of these studies included histopatholo-gical sections of the temporal bone.Li et al. (1994) studied the temporal bone histopathologyin eight patients who had died from advanced, dissemi-nated MM. Among these eight patients only two hadotological symptoms, while 14 out of 16 temporal bones(87 per cent) exhibited MM lesions in various locations.The structures involved were exclusively bony, withsparing of mucosa, skin and other key structures such asthe facial nerve or the carotid artery.https:/www.cambridge.org/core/terms. https://doi.org/10.1017/S0022215100140800Downloaded from https:/www.cambridge.org/core. University of Basel Library, on 11 Jul 2017 at 08:24:28, subject to the Cambridge Core terms of use, available atCLINICAL RECORDS 471TABLE ICASES OF TEMPORAL BONE INVOLVEMENT IN MULTIPLE MYELOMA REPORTED IN THE LITERATUREHearing FacialAge Sex loss Deafness Tinnitus Vertigo palsySystemicOtalgia symptomsLa vine (1979)Marks and Brookes (1985)Shone (1985)Keay (1985)Chevalier et al. (1992)Funakubo and Kikuchi (1994)Present case55715558627170FMFFFMMThe temporal bone is, therefore, often involved inadvanced cases of MM. The clinical expression of thisinvolvement is, however, unusual (Table I) and onlyexceptionally does MM present with otological symptoms,as in our patient.ReferencesAbemayor, E., Canalis, R. F., Greenberg, P., Wortham, D. G.,Rowland, J. P., Sun, N. C. J. (1988) Plasma cell tumors ofthe head and neck. Journal of Otolaryngology 17: 376-381.Alexian, R., Dimopulos, M. (1994) The treatment of multiplemyeloma. New England Journal of Medicine 330: 484-489.Barlogie, B. (1995) Plasma cell myeloma. In WilliamsHematology. 5th Edition. (Beutler, E., Lichtman, M. A.,Coller, B. S., Kipps, T. J., eds.), McGraw-Hill, New York,pp 1109-1123.Bataille, R., Harousseau, J. L. (1997) Multiple myeloma. NewEngland Journal of Medicine 336: 1657-1664.Chevalier, X., Wrona, N. N., Larget-Piet, B. (1992) Vestibularsyndrome in multiple myeloma: role of magnetic resonanceimaging. Clinical Rheumatology 11: 4O5 t^O7.Funakubo, T., Kikuchi, A. (1994) A case of myeloma withfacial palsy. Ada Otolaryngologica (Suppl 511): 200-203.Hyams, V. F. (1987) Pathology of tumors of the ear. InComprehensive Management of Head and Neck Tumor.(Thawley, S. E., Panje, W. R., eds.), W. B. Saunders,Philadelphia, pp 168-193.Jackler, R. K., Parker, D. A. (1992) Radiographic differentialdiagnosis of petrous apex lesions. American Journal ofOtology 13: 561-574.Keay, D. (1988) Total bilateral hearing loss as a complicationof myeloma. Journal of Laryngology and Otology 102:357-358.Kemink, J. L., Graham, M. D., Gebarski, S. S. (1993) Surgeryof the temporal bone neoplasms. In Otolaryngology - Headand Neck Surgery. 2nd Edition. (Cummings, C. W.,Fredrickson, J. M., Harker, L. A., Krause, C. J., Schuller,D. E., eds.), Mosby Year Book, Inc., St Louis,pp 3353-3370.Lavine, R. F., Mattucci, K. F., Merton, C. W. (1979) Multiplemyeloma of the temporal bone. Ear Nose and ThroatJournal 58: 210-213.Li, W., Schachern, P. A., Morizono, T., Paparella, M. M.(1994) The temporal bone in multiple myeloma. Laryngo-scope 104: 675-680.Marks, P. V., Brookes, G. B. (1985) Myelomatosis presentingas an isolated lesion in the mastoid. Journal of Laryngologyand Otology 99: 903-906.Schuknecht, H. F. (1993) Pathology of the Ear. 2nd Edition.Harvard University Press, Cambridge, p 498.Selby, P., Gore, M. (1995) Myeloma and other plasma cellmalignancies. In Oxford Textbook of Oncology. (Peckham,M., Pinedo, H. M., Veronesi, U., eds.), Oxford UniversityPress, Oxford, pp 1852-1878.Shone, G. R. (1985) Facial palsy due to myeloma of thetemporal bone. Journal of Laryngology and Otology 99:907-908.Address for correspondence:Pavel Dulguerov, M.D.,Department of Otolaryngology, Head and Neck Surgery,University Canton Hospital,24 Rue Micheli-du-Crest,1211 Geneva 14,Switzerland.Fax: (4122) 372 82 40e-mail: Pavel.Dulguerov@hcuge.chhttps:/www.cambridge.org/core/terms. https://doi.org/10.1017/S0022215100140800Downloaded from https:/www.cambridge.org/core. 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Multiple myeloma presenting with external ear canal mass

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