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Unusual osseous sclerosis in multiple myeloma

the immunoglobulin-(; type. biii normal total protoin: r:levnl.r(l crythroc:yte sediineiitalion rate. 4 2 inm/h (nornial < 20 mrn/h), but iiornial haernoglobin. whito cell aiid differential Count. and alkaline phosphatascs. There was no sign of Bencc-Joncs prnteiii in tho urine.

Key words ---P-

Miiltiple m y e l o m a - O s t e o s c ~ e r o s i s

Schlüsselwörter

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Discussion

Multiples Myelome - Osteosklerose

In this paticnt tlia dingiiosis of niultiple rnyeloma seems establishcd. but sonir of 1 . 1 1 ~Iindings are not typical fnr the discasc. arens ol'octeosclerosis. in thc facisl boiios with rnarked spiciilal.ions on the surface. and involveineiit ofihe orbit (4. 5).

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riiiiltiple rnyeloma bone lesions arc? usunlly purely osteolytic. but osteosclerotic bonc changes iiiay occur (1 - 3). Wc prcscnt a casc with spiculated sclerotic hone changes in the iacinl boiies coinbined with a bone destructing plasmacjl.nmn iii the upper respiratory tract and orbit. Iii

Cüse report

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The cliissic osseous manifostntions of inultiple myeloiria are well known. l ' h c consist o i iiiiiltiple .,punched-out" areas of destruction of vnrious sizes without marginal sclorosis, oitcn associated wil.11 gerieralised ostcoporosis ( 6 ) . Osteoclerotic changes a r e mostly rclatcd to I'rnctures and diagnostic or therapeutic proccdures ( 7 . 8 ) . but primary osteosclcrotic lesions in multiple myeloma have been describcd (1 - 3 , 7). snd estirnated to show up in 3 % of the patients (9).They appear either a s solitary tir

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A 51-vear-old vreviouslv healthv man ~ r e s e n t c d with a iour-iiioritli hisiory of sielling i i the rcgion o l the right Zygoma accornpanied by gradual obstruction ofthe right nasal cavity. Exarnination reveaied the swelling 10 be a bony protuberancc witli slight irillanirnation ofthe superjacent skin. locatcd below nnd lateral to the right cyc. Rhinoscopy revenled a soft tissiie rnass in thc right nasal cavity. A hard inobile lymph node was palpablc in the leit. sii brnaiidibular region.

Conventional radiography of the paranasal siiiuses (Fig. 1) sliowed osseous sclerosis in thc lateral wnll of' the right maxillary sinus. which by CT (Fig. 2) was found to be located mainly a t thc? zygornntic bone. on which spiculation occurrcd. A large soft tissue turnour cornponent was secn. which almost occluded the maxillary sinus and extended into tlie lateral part of thc right orbit. A soit. tissiie turnoiir extending from thc right valatiiie process along- the nasal Septum into the sphenoidal siiius was also Seen. Skclctal suwey was perfor~nedand revealed a ,.punched-out" lesion ori ttie rigtit side of the skull. and sclcrotic changes in the right iliurn and in the 1lththoracicvertebra (Fig. 3). Scintigraphy showed increased activity on tlie riglit side o i t h e skull a i d face. in thc lowcr thoracal spiiie, arid the right ilium. Biopsy from thc nasal tumour revealed tissue pronounccd infiltrated with atypical plasnia cells without amyloid deposition. Iminiiiiohistological staining revcalcd cytoplasniatic rnonoclonal irnrnunoglobulin-G of the kappn type coiripatible with myeloma. Marrow biopsies taken rroiri the sternurn and iliac crest were normal. Laboratory exarninations rcvcalcd paraproteiri of

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Fortschr. Röntgenstr. 155.5(1991)472 -473 O Georg'l'hicrncVcrlng Stuttgart. New York

Fig. 1 Frontal radiographof the facial bonesshowingsclerosisofthe right zygomatic boneand a sofi tissue mass in the maxillar sinus.

Fig. 2 CT scans through the maxillar sinuses and the orbits, deinonstratingspiculated new bone formation on the zygomatic bone, and a soft tissue massextendrng into the right orbit. nasal. maxillar, and sphenoida1cavity.

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ßy J . AlOr-echtsen and A. C. Jurik I)epirr~irieiitsoil)iiigiiosLic: Kadiology. 'l'he County arid thc Miinicipal Ilosl)it;rl. llnivcrsity ol'.Aarhus. Doiiniai.k

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References .-.-P-

Fig. 3 Frontal and lateral radiographs of the thoracic spine showingcondensation of the 11"'verlebra.

multiple localised lesions or. rarely. as a diffuse process. mostcommonly involving the axial skeleton (G). 'rhe finding o i spiciilated sclerotic lesions as Seen in the present patient has previously only been described in a fcw cases with rnyelorna (2.8. 10).The locatioii of facial lesions is linewise unusual. lnvolvement of the orbita a s psrt ofmultiple myeloma is rarc (4. 5). Sclerotic bone lesions may occur as part of the Poerus syridrome (1). The prescnt patient. however, had no neuropathy. organomegaly. endocrinopathy, or skin disease a s Seen in patients with this Syndrome (1).

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Sartoris, D. $1..D. Pale, P. Haghigizi. C . Creenu~ay,L). Hcsnick: Plasma cell sclerosis oi bonc: a spectrurn of discasc. .I. Caii. Assoc. Radiol. 37 ( 1986) 25- 34 Krninin. I'., C. .I. ß'nngio. A. Smelin: Multiple iriyeloma with new bone forinatioii. Arch. Intcrn. Med. 84 (I 949) 976 -982 ßrandon. C., W. ,Martel, L. Weatherbee. I'. Capck: Case report 572. Skeletal Hadiol. 1 8 (1989) 542 -546 7un.q. G..P. T. Finger, I. Klein, C). Chess. Al. Y. ~Llanhassel:Plasmacyloiria of the orbit. Arch. Ophthalrnol. 106 (1988) 1622 Rotiman, H . I , , R. L. Fonl: Orbital involveiiieiii in rnultiplc mycloma. Arcli. Ophthalmol. 87 ( 1972) 30-35 Resrlirk. D.,G'. Niwayarna: Diagnosis of bonc and joint disordsrs with ciiiphasis on articular abnornialities. Saunders. Philadelphia (1981) 1872-1880. 191 4-1933. 27 12- 21 13. 3007-3012 Hirnrneuarb. B , J. Sebs. J. Rabinowits: Uiiiisual roentgenographic presentations of multipln rnyelonia. J. Bonc Joint Surg. 56-A (1 974) 1723 -1728 Odelberg-Johnsor2,0.:Osteosclerotic changes i i i iriyelomatosis. Acta Radiol. 52 (1959) 139.- 144 Euison, G.. K. 7: Euanst Bone sclerosis in rnulti~le Br. . mvelorna. . J. Hadiol. 40 (1967) 81 -89 Yentis. 1.: Radiological aspects of myeloiiiatosis. Clin. Radiol. 12 (1961) 1-7

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Uuusual osscous sclerosis in mult~plernyelonia ---

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Unusual osseous sclerosis in multiple myeloma.

Schaukasten 472 - -- -. .- . - -- Unusual osseous sclerosis in multiple myeloma the immunoglobulin-(; type. biii normal total protoin: r:levnl.r...
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