Journal of Neuro-Ophthalmology, March 1984, Volume 4, Issue 1

Orbital involvement in multiple myeloma. A new angiographic presentation.

A 49-year-old black female presented with proptosis, orbital pain, decreased visual acuity, and a left frontal bone mass. This lesion resembled a meningioma by computerized tomography and carotid angiography. Following craniotomy, a tissue diagnosis was made of plasmacytoma, with systemic myeloma found by bone marrow biopsy. This case was unusual in its radiographic appearance as plasmacytomas are usually avascular. This case illustrates that orbital plasmacytomas may radiographically mimic other orbital tumors such as meningiomas, melanomas, and orbital carcinomas, and that definitive diagnosis must often depend on histopathologic study.

J. elin. Neuro-ophthalmol. 4: 25-29, 1984. Orbital Involvement in Multiple Myeloma A New Angiographic Presentation HARRY A. HAMBURGER, M.D. Abstract A 49-year-old black female presented with propto­sis, orbital pain, decreased visual acuity, and a left frontal bone mass. This lesion resembled a m.enin­gioma by computerized tomography and carotid angiography. Following craniotomy, a tissue diag­nosis was made of plasmacytoma, with systemic myeloma found by bone marrow biopsy. This case was unusual in its radiographic appearance as plas­macytomas are usually avascular. This case illus­trates that orbital plasmacytomas may radiographi­cally mimic other orbital tumors such as menin­giomas, melanomas, and orbital carcinomas, and thai definitive diagnosis must often depend on his­topathologic study. A 49-year-old black female was first seen in February 1983, with progressive swelling of her left frontal bone, proptosis, and decreased visual acu'·y in the left eye. She first noticed painless SWI lmg of her left forehead 2 months earlier. In January 1983, a rapid increase in the swelling of ?~r f~rehead, marked proptosis, and conjunctival Injection of the left eye occurred. At the time of our initial examination, she denied any prior his­t~ ry of headache, dizziness, loss of balance, or diplopia, but did complain of increasing lethargy and.a tendency to bruise easily. . ~lSu~1 acuity in the right eye was 20/20. The VISIon In the left eye was reduced to 20/200. Visual field examination revealed a central sco­toma in the left eye. External examination showed a large, soft, nonpulsatile mass eroding through the left frontal bone (Fig. 1). There was marked p~optosis.and .lateral displacement of the left eye WIth conjunctival chemosis and injection. Move­me~ t of the left eye was significantly limited in all fields of gaze. A 2+ Marcus Gunn pupil was found on the left. Slip lamp examination was normal in both eyes. Fundoscopic examination showed slight temporal optic atrophy in the left eye. Medical examination showed a moderately From the Department of Ophthalmology, Sinai Hospital of Detroit, Michigan; and the Department of euro-ophthal· mology, Bascom Palmer Eye Institute, Miami, Florida. March 1984 obese female with' bipedal edema. Her neck was supple with no lymphadenopathy or thyroid en­largement. The heart and lungs were normal, and there were no breast masses found on palpation. The abdomen was soft with no masses or organ­omegaly. There were multiple small ecchymoses present on the left arm and right leg. eurological examination showed no other abnormalities than the pupillary and motility disturbances of the left eye. Admission laboratory studies showed a mild anemia with a hemoglobin of 11.7 g/dl The sedimentation rate was 62 mm/hour. The serum protein was increased with a monoclonal gamma globulin spike on electrophoresis. A test for Bence-Jone's protein in the urine was negative. The serum viscosity was increased to 2.25 relative to water. A chest x-ray showed multiple bilateral areas of basal discoid atelectasis suggestive of multiple pulmonary emboli. A bone survey revealed lu­cencies of the cranium, mandible, left scapula, proximal femur, and thoracic spine. A skull series showed a large 7.5 x 7.0 cm area of erosion involving the left anterior and lateral portions of the frontal bone. There was lysis of a portion of the left superior orbital fissure, lesser and greater wings of the sphenoid, and frontal process (Fig. 2). A bone marrow biOpsy showed infiltration with immature dysplastic plasma cells. There were adequate megakaryocytes, granulocytes, and precursors of red blood cells present. Computerized tomography confirmed destruc­tion of the left orbit, frontal, and parietal bones. There was a large tissue mass compressing the left lateral ventricle, extending from the fronto­parietal region in the left orbit, which showed marked contrast enhancement (Fig. 3). A left common carotid and left external carotid angiogram showed a vascular, extra-axial mass in the left frontal region of the brain with extracran­ial and intraorbital extension. Due to its extreme vascularity, the mass was initially felt to be a meningioma (Fig. 4). Craniotomy on February 14, 1983, showed a gray, friable mass with bony destruction in the left frontal region. Pathologic sections revealed 25 Orbital Involvement in Multiple Myeloma Figure 1. Initial presentation of a 49-year-old black female with decreased vision, pain, and proptosis of the left eye. Note the marked left exotropia, proptosis, and left frontal swelling. Figure 2. Skull x-ray showing a large 7.5 X 7.0 cm area of erosision involving the left frontal bone. Note the multiple lytic lesions throughout the skull ranging from 1 to 6 mm in diamete.r. numerous plasmacytoid and atypical plasma cells .with hyperchromic, enlarged nuclei. Both the brain and bone marrow biopsies were felt to be diagnostic of malignant multiple myeloma. The patient was begun on radiotherapy and chemotherapy with Alkeran and prednisone. Over the next 2 weeks, there was return of vision in the left eye, decreased proptosis, and marked shrinking of the left frontal mass. A repeat computerized tomogram showed a dramatic decrease in the size of the mass with diminution of the ventricular shift. Over the next month following treatment, the motility of the left eye improved and the proptosis disappeared (Fig. 5). Discussion Multiple myeloma is an uncontrolled prolifer­ation of plasma cells with the overproduction of specific proteins in the immunoglobulin class. I In Journal of Clinical Neuro-ophthalmology Hamburger Figure 3. Pastcontrast computerized tomography of the brain. Note the large left frontal mass that has extended extracranially. There is evidence of mass e((ect, with shifting of the ventricular system to the right. Figure 4. Left common carotid and left external carotid angiogram. A lateral view shows a large vascular mass in the left frontal region of the brain with marked dye uptake. The staining appeared in the midarterial phase, and persisted into the midvenous phase. Note the presence of a smaller vascular lesion in the superior region of the skull. the majority of cases the immunoglobulin is the IgG fraction. Patients present with bone pain, pathologic fractures, anemia, hlpercalcemia, fa­tigue, and increased infection.2 - The presentation is usually in the fourth to seventh decade of life, March 1984 although the disease has been described in chil­dren. Other associated systemic findings are an increased sedimentation rate, hyperproteinemia, skin purpura, petechiae, and Bence-Jone's pro­teinuria. Ocular findings are relatively common 27 Orbitallnvolvement in Multiple Myeloma Figure 5. Appearance of the patient following a course of systemic chemo­therapy with Alkeran and prednisone. and irradiation to the brain and left orbit. ote the marked decrease in the amount of proptosis of the left eye with no strabismus. Also note the moderate amount of left upper lid swelling and erythema which are characteristic postirradiation skin changes. with corneal crystals, pars plana cysts, retinal hemorrhages, conjunctival crystals, lacrimal swelling, and papilledema reported.q - D Although orbital involvement is rare, Boniuk in a review of the literature in 1981, found 35 cases of myeloma affecting this region.N - 27 He found that patients commonly presented with proptosis, decreased visual acuity, orbital pain, and diplopia 2 .29 It is felt by many that the orbital manifestations of multiple myeloma are due to local accumulation of malignant plasma cells, and or the secondary effects of dysproteinemia.3o Rodman described three cases of orbital involvement in which ma­lignant plasma cells infiltrated the lateral rectus in one, and diffuse~ filled the entire muscle cone in the other two. 2 There have also been cases reported of isolated orbital plasmacytomas with­out systemic multiple myeloma.3 ) In such cases of multiple myeloma involving the orbit without systemic spread, the prognosis is markedly im­proved.) 1 Our case was unusual in that the patient pre­sented with massive bowing and erosion of the left frontal bone and proptosis. The amount of bony destruction of the orbit and skull Seen on x-ray has not been described previously in mul­tiple myeloma. Due to the enhancement on computerized to­mography and the vascularity on cerebral angio­graphy, the orbital mass in our patient was ini­tially diagnosed as a left frontal meningioma with orbital extension. Rosenbaum et al. described the angiographic appearence of three cases of orbital plasmacytomas.32 The tumors were seen to be relatively avascular and semisolid in appear­ance. 32 Reports by others of the angiographic appearance of orbital plasmacytomas are sparse in the literature due to the risk of sudden death from contrast media injection in patients with dysproteinemia.33 Others have described plas­macytomas angiographically as having a normal vascular supply, being faintly visible, or showing a faint vascular blush after subtraction studies.]~ In none of the previously studied cases of orbital plasmacytoma has an angiographic or radiologic picture similar to our case been described. The mass was intensely vascular throughout the ar­terial and venous phases of the angiogram, with large intralesional Channels, and compression of the adjacent cerebral vasculature. Without biopsy, our patient's lesion could eas­ily have been misdiagnosed. Many other masses can show a vascular patter on angiography such as melanoma, hemangioma, schwannomas, men­ingiomas, and primary orbital carcinoma.]5-37 If the initial diagnosis of meningioma had been accepted in our patient, a significant delay in the initiation of proper therapy could have occurred. Whereas the treatment of orbital meningioma is surgical, radiation therapy, or observation, that of orbital plasmacytoma is chemotherapy and irradiation. Diagnosis histologically of plasmacytomas may be difficult. At times it may be impossible to differentiate a reactive proliferation of plasma cells seen in orbital pseudotumor or Grave's dis- Journal of Clinical Neuro-ophthalmology ease from an isolated plasmacytoma.27 Multiple myeloma involving the orbit has been histologi­cally confused with undifferentiated carcinoma, reticulum cell sarcoma, and amelanotic mela­noma. J8 Although Rosenbaum has described plasma­cytomas of the orbit as being avascular, this an­giographic classification may have to be modified in light of our findings. Following proper therapy for multiple myeloma, our patient has shown no recurrence of her orbital plasmacytoma, and her hematologic parameters are stable. It is suggested by this case that computerized tomography and angiography are not always diagnostic of intra­cranial and orbital masses, and that in some cases biopsy is the best means of differentiation. References 1. Orellana, J., and Friedman, A: Ocular manifesta­tions of multiple myeloma, Waldenstroms macro­globulinemia, and benign monoclonal gammopa­thy. Surv. Ophthalmol. 26: 157-169, 1981. 2. Azar, H.A, and Potter, M.: Multiple Myeloma and Related Disease, Vol. 1. Hagerstown, Harper and Row, Hagerstown, Maryland, 1973, pp. 1-152. 3. Farhargi, M., and Osserman, E.F.: The treatment of multiple myeloma. Semin. Hematol. 10: 149-161, 1973. 4. Kyle, R.A: Multiple myeloma: Review of 869 cases. Mayo Clin. Proc. 50: 29-40, 1975. 5. Levin, W. c.: Multiple myeloma. Arch. Intern Med. 135: 27-32, 1975. 6. Osserman, E.F., and Takatsuki, K.: Plasma cell myeloma gammaglobin synthesis and structure. Medicine 42: 357-384, 1963. 7. Rosen, B.J.: Multiple myeloma. A clinical review. Med. Clin. North Am. 59: 375-386, 1975. 8. Azzena, D., Costa, U., Ghigliotta, G., and Astengo, F.: Serum hyperviscosity in multiple myeloma and its clinical and neuropsychiatric implications. Con­fin. Neural. 30: 65-75, 1968. 9. Burki, E.: Uber Hornhautveranderung bei einem Fall von Multiplem Myelom (Plasmacytom). Ophthalmologica 135: 565-572, 1958. 10. Markoff, N.: Uber Kristallbildung in Hornhaut bei Uretharantherapie des Myeloma. Schweiz Med. Wochenschr. 78: 987-988, 1948. 11. Pinkerton, R.M.H., and Robertson, D.M.: Corneal and conjunctival changes in dysproteinemia. In­vest. Ophthalmol. 8: 357-364, 1969. 12. Ashton, N.: Ocular changes in multiple myeloma­tosis. Arch. Ophthalmol. 73: 487-494, 1965. 13. Sanders, T.E., and Podos, S.M.: Pars plana cysts in multiple myeloma. Trans. Am. Acad. Ophthalmol. Otolaryngol. 70: 951-958, 1966. 14. Sanders, T.E., Podos, S.M., and Rosenbaum, L.J.: Intraocular manifestations of multiple myeloma. Arch. Ophthalmol. 77: 789-794, 1967. 15. Siansky, H.H., Bronstein, M. and Gartner, S.: Cil­iary body cysts in multiple myeloma. Arch. Ophtha/mol. 76: 686-689, 1966. 16. Reimann, H.A: Hyperproteinemia as a cause of March 1984 Hamburger autohemagglutination.]AMA 99: 1411-1414, 1932. 17. Spalter, H.F.: Abnormal serum proteins and retinal vein thrombosis. Arch. Ophthalmol. 62: 868-881, 1959. 18. Berneaud-Kotz, G., and Janke, K.: A case of dys­proteinemia with unusual fundus changes. Klin. Monatsbl. Augenheikd. 125: 160-171, 1954. 19. Cucco, G., and Pende, G.: Fundus changes in plasmacytoma. Ann. Otolaryngol. 81: 477, 1955. 20. Danis, P., Brauman, S. and Coppez, P.: Lesions of the fundus of the eye found in certain hyperpro­teinemias, particularly those of myelomatous ori­gin. Acta. Ophthalmol. 33: 33-52, 1955. 21. Aronson, S.B., and Shaw, R: Corneal crystals in multiple myeloma. Arch. Ophthalmol. 61: 541-546, 1959. 22. Parker, S.T.: Plasmacytoma of the lacrimal gland. Proc. Roy. Soc. Med. 31: 137-139, 1937. 23. Clark, E.: Plasma cell myeloma of the orbit. Br. ,. Ophthalmol. 37: 543-554, 1953. 24. Forrest, AW.: Intraorbital tumors. Arch. Ophthal­mol. 41: 198-232, 1949. 25. Benjamin, I., Taylor, H., and Spindler, J.: Orbital and conjunctival involvement in multiple mye­loma. Report of a case. Am. ]. Clin. Pathol. 63: 811­817,1975. 26. Levin, S.R., Spaulding, AG., and Wirman, J.A.: Multiple myeloma. Orbital involvement in a youth. Arch. Ophthalmol. 95: 642-644, 1977. 27. Rodman, H.I., and Font, RL.: Orbital involvement in multiple myeloma. Review of the literature and report of three cases. Arch. Ophthalmol. 87: 30-35, 1972. 28. McFadzean, RM.: Orbital plasma cell myeloma. Br.]. Ophthalmol. 59: 164-165, 1975. 29. Pasmantier, M.W., and Azar, H.A: Extraskeletal spread in multiple plasma cell myeloma: A review of 57 autopsied cases. Cancer 23: 167-174, 1969. 30. Giarelli, Luigi, Melato, Mauro, Campos, Emilio, Falconieri, Giovanni: Eye involvement in multiple myeloma. Hematologica 66: 57-67, 1981. 31. Dolin, S., and Dewar, J.P.: Extramedullary plas­macytoma. Am.'. Pathol. 32: 83-103, 1956. 32. Rosenbaum, A., Zingesser, L., and Schechter, M.: Angiographic Observations in Myeloma With Result­ant Exophthalmos. Neuro-Ophthalmology, Vol. 5, J. Lawton Smith, Ed. Huffman Publishing Co., Hal­landale, Florida, 1970, chap. 22, pp. 333-345. 33. Mucchi, L., and Columella, F.: Arteriography in diseases of bone. J. Fae. Radial. 3: 135, 1951. 34. Jim, V.K.S.: Plasmacytoma of orbit. Am.'. Ophthal­mol. 39: 43, 1955. 35. Grino, A, and Billet, E.: Diagnosis of orbital tumors by angiography. Am. j. 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