Orbital and Conjunctival Involvement in Multiple Myeloma Report of a Case IVY BENJAMIN, M.D.,

H E R B E R T T A Y L O R , M.D.,

AND JAMES SPINDLER,

M.D.

Department of Pathology, St. Louis University Hospitals, St. Louis, Missouri

ABSTRACT

A L T H O U G H T H E ORBITAL and conjunctival tissues, including the lacrimal gland, 15 are well known to have a predilection for development of inflammatory masses of plasma cells, neoplastic plasmacytomas and involvement in multiple myeloma are rare. Hellwig's review12 of extramedullary plasma-cell tumors showed conjunctiva as the second most frequent site of involvement, the first being the upper respiratory tract. None of the above-mentioned cases was associated with multiple myeloma. Pasmantier and Azar, 16 in their review of 56 autopsied cases of multiple myeloma, did not include the orbit or conjunctiva among the sites of extraskeletal spread. Rodman and Font 17 reported three cases and an additional case Received September 3, 1974; received revised manuscript November 1, 1974; accepted for publication November 1, 1974. Address reprint requests to Dr. Benjamin, Department of Pathology, St. Louis University School of Medicine, 1402 South Grand Boulevard, St. Louis, Missouri 63104. 811

(in an addendum) of orbital involvement in multiple myeloma. They also reviewed the world literature and found 27 other cases similar to their own, thereby totalling 31 cases during the period between 1953 and 1972. T h e purpose of this paper is to present another case of multiple myeloma with ocular changes consistent with orbital and conjunctival neoplastic plasmacytoma. Report of a Case A 54-year-old Negro man was first hospitalized on February 15, 1973, with a four-month history of progressive pain in the right arm and shoulder. Radiographic studies revealed lytic lesions of right humerus and right ischium, with a nondisplaced pathologic fracture of the humerus (Fig. 4). A biopsy taken at the time an intramedullary pin was placed in the humerus was interpreted as plasmacell myeloma. S e r u m p r o t e i n electrophoresis revealed a monoclonal peak

Downloaded from http://ajcp.oxfordjournals.org/ by guest on June 5, 2016

Benjamin, Ivy, Taylor, Herbert, and Spindler, James: Orbital and conjunctival involvement in multiple myeloma. Report of a case. Am J Clin Pathol 63: 811-817, 1975. Orbital and conjunctival involvement in multiple myeloma are rare. In most reported cases of multiple myeloma with orbital involvement proptosis has been the initial manifestation that has led to diagnosis of the disease. 5,7,8,10,17,18 Our case represents an example of terminal involvement of the orbit and conjunctiva, causing proptosis. T h e minimal conjunctival changes were the first indication of neoplastic proliferation of plasma cells in this location and, presumably, the orbit. (Key words: Plasmacytoma; Multiple myeloma; Conjunctiva; Orbit.)

812

BENJAMIN, TAYLOR AND SPINDLER

t

y < i

FIG. 1. T h e right eye, showing conjunctival erythema and swelling.

of gamma globulin (Fig. 7). Urinary Bence Jones protein was not detected. Serum inorganic phosphorus was minimally elevated ( 4 - 8 mg. per 100 ml.), but serum calcium and the remainder of laboratory findings were within normal limits. The patient was discharged and given radiotherapy to the humerus, as well as combined chemotherapy, on an outpatient basis. He was again hospitalized on March 4, 1974, for re-evaluation. At this time, lytic lesions were demonstrated in the clavicles and scapulas. The hematocrit had decreased from 35 to 22%, and progressive pulmonary dysfunction developed. T h e patient was also somewhat disoriented. Firm elevated lesions were palpated over the skull and paraspinous processes of

vertebrae. Marked conjunctival erythema of the right eye was noted. Laboratory findings on this admission included: total protein of 10.5 Gm. per 100 ml. (normal 6.0-8.5); albumin 2 - 6 . 0 Gm. per 100 ml. (normal 3.12-5.78); globulin, ^»-

. ' ^ \ . .; ; ^ * Downloaded from http://ajcp.oxfordjournals.org/ by guest on June 5, 2016

ifei



814

BENJAMIN, TAYLOR AND SPINDLER

A.J.C.P. — Vol. 63

plasmacytoid cells. Electrolytes were: sodium 126 mEq. per 1.; potassium 3.7 mEq. per 1.; chloride 92 mEq. per 1.; carbon dioxide-combining power was 20 mEq. per 1. Serum glucose was 190 mg. per 100 ml.; BUN 105 mg. per 100 ml., uric acid 15.0 mg. per 100 ml., serum calcium 13.0 mg. per 100 ml. Roentgenograms of the chest showed cardiac enlargement with diffuse interstitial infiltrates in the lungs and bilateral pleural effusion. T h e hospital course was one of progressive deterioration. Hypercalcemia was controlled with phosphosoda and saline solution. T h e conjunctival lesion enlarged to a size of 1.5 x 0.8 x 1.0 cm. Biopsy was not done, due to the patient's general

debility and pancytopenia. Terminally he had multisystem failure, and he died March, 15, 1974. Autopsy Findings. At autopsy, a few petechial hemorrhages were found on the trunk. T h e conjunctival lesion was 3 x 1 cm. and appeared to displace the globe upward and outward (Fig. 1). T h e internal chest wall and pleural surfaces of the ribs had multiple calcific nodules, and one on the eighth rib was contiguous with the parenchyma of the lung. T h e lungs weighed 1,800 Gm. (right lung) and 1,600 Gm. (left lung). T h e pleura showed occasional tumor nodules. T h e parenchyma was grossly hard and gritty. T h e heart weighed 260 Gm., and the myocardium was firm and beefy, with a gritty consis-

Downloaded from http://ajcp.oxfordjournals.org/ by guest on June 5, 2016

FIG. 4. Roentgenogram of the right humerus, showing the lytic lesion.

June 1975

1

OCULAR INVOLVEMENT IN MULTIPLE MYELOMA

815

*.•••

Downloaded from http://ajcp.oxfordjournals.org/ by guest on June 5, 2016

FIG. 5 (upper). Bone marrow, showing monotonous infiltration by plasma cells. Hematoxylin and eosin. x40. FIG. 6 (lower). Pulmonary parenchyma. Extensive calcification of alveolar septa. Hematoxylin and eosin. X 100.

BENJAMIN, TAYLOR AND SPINDLER

816

A.J.C.P. —Vol. 63

Normal range ( gm*l TOTAL PROTEIN

Globulins a\

02

» y

.50 1.00

.70 5.90

6.0 - 8 . 5 g m * 3.12 - 5.78

2.60

Albumin

1

10.8 gin*

A

0.14 -

0.45

0:40-

1.15

0.51 -

1.23

FIG. 7. Serum protein electrophoresis, showing monoclonal peak in gamma G region.

0.80 - 1.79

the pulmonary architecture was represented by a delicate lattice of calcification replacing alveolar septae (Fig. 6). There were scattered foci of leukocytes, which in several areas were composed almost totally of plasma cells. Other areas showed pulmonary h e m o r r h a g e and edema. Examination of the myocardium showed calcification of both small vessels and interstitial tissue. There were tumor infiltrates of the abdominal wall, diaphragm, mesentery, pancreas, kidneys, right adrenal, right testis, and thyroid. Calcification was also found in the kidneys, liver, and pancreas. Microscopic examination of the vertebrae revealed total replacement by plasma cells. Microscopically, the parathyroid was found to be atrophic. Discussion T h e usual clinical manifestation of orbital involvement in multiple myeloma is proptosis, and in many of the cases reported this has been the earliest manifestation of the disease, or among the earliest. 5,7,10,17 Other symptoms have varied from impairment of vision to total blindness, pain, or diplopia. 8,13,17 Various ocular complications in multiple myeloma

Downloaded from http://ajcp.oxfordjournals.org/ by guest on June 5, 2016

tency. Examination of the abdominal cavity showed several yellowish t u m o r nodules on the internal abdominal wall, diaphragm and mesentery. The liver was congested, with multiple flecks of black material on the cut surface. T h e tail of the pancreas was replaced by a firm pink tumor, which was diffuse. There was congestion of the spleen. The right adrenal contained a 1-cm. tumor nodule. T h e kidneys showed fleshy nodules in the perirenal capsule contiguous with the renal substance. A 2-cm. pink, fleshy tumor nodule involved the right testis. One parathyroid was identified. Light Microscopy. Examination of the conjuctiva showed unremarkable conjunctival mucosa with discrete infiltration of plasma cells of varying maturity, but predominantly immature forms (Figs. 2 and 3). Just beneath the mucosa the stroma was hyalinized. Congo red stains were negative for amyloid. The plasmacytic infiltrate was more dense and prominent in the deeper portions of the nodule. T h e nodules in the chest wall and pleural surfaces of ribs were masses of immature plasma cells. T h e lungs contained extensive calcification of pulmonary vessels and alveolar walls. In areas

June 1975

OCULAR INVOLVEMENT IN MULTIPLE MYELOMA

817

Summary A case of multiple myeloma involving the orbit and conjunctiva is presented. Skeletal and extraskeletal spread, 3 , 9 , n with hypercalcemia and marked metastatic cal-

in multiple plasma cell myeloma. A review of 57 autopsied cases. Cancer 23:167-174, 1969 17. Rodman H, Font R: Orbital involvement in multiple myeloma. Arch Ophthalmol 8 7 : 3 0 35, 1972 18. Taylor D, Taylor C: Multiple myelomatosis affecting the orbit. Br J Ophthalmol 4 1 : 4 3 8 439, 1957

Downloaded from http://ajcp.oxfordjournals.org/ by guest on June 5, 2016

have been r e p o r t e d , involving the cification in the viscera, was also present conjunctiva, 1 retina, 6,8,14 ciliary body, 2,4,14 in this case. and sclera, choroid, and iris.4 References The case presented here is different in that the orbital and conjunctival involve1. Arneson SB, Shaw R: Corneal crystals in multiple myeloma. Arch Ophthalmol 61:541-546, m e n t was t e r m i n a l , with minimal 1959 symptoms relating to the eye. T h e patient 2. Ashton N : O c u l a r c h a n g e s in m u l t i p l e was d i a g n o s e d as having multiple myelomatosis. Arch Ophthalmol 73:487-494, 1965 myeloma about a year prior to the ap3. Azar HA: Plasma cell myelomatosis and other pearance of ocular changes. X-ray studies monoclonal gammopatnies. Pathology Annual of the skull during the last admission did 1972. Edited by SC Sommers. New York, Appleton-Century-Crofts, 1972, pp 1-17 not reveal any osteolytic lesions. It would 4. Brownstein M: Ocular involvement in multiple appear, therefore, that orbital involvemyeloma. Arch Ophthalmol 55:188- 192, 1956 ment is primary, in the sense that it was 5. Clarke E: Plasma cell myeloma of the orbit. Br J Ophthalmol 37:543-553, 1953 not encroached upon secondarily from 6. Clarke E: Ophthalmological complications of cranial bones or paranasal sinuses. multiple myelomatosis. Br J Ophthalmol 39:233-236, 1955 The conjunctival involvement was ter- 7. Delong, P: Plasmacytoma of the orbit. Am J minal, and even at the time of the last Ophthalmol 32:1765-1748, 1949 admission to the hospital, the ocular signs 8. Donnelly EJ: Ocular complication of multiple myeloma. Am J Ophthalmol 47:211 - 2 1 4 , 1959 consisted mainly of erythema of the con- 9. Edwards GA: Extra osseous lesions in plasma cell myeloma. A report of six cases. Am J Med junctiva. It would seem, therefore, that 43:194-205, 1967 this terminal involvement was secondary 10. Handousa A: Multiple myelomatosis with propto a more extensive orbital tumor, as the tosis. Br J Ophthalmol 3 9 : 4 1 - 4 3 , 1955 autopsy revealed proptosis of the right 11. Hayes DW, Bennett WA, Heck FJ: Extramedull a r lesions in multiple myeloma. Review of eye. literature and pathological studies. Arch Pathol 53:262-266, 1952 Whether the orbital and conjunctival 12. Hellwig CA: E x t r a m e d u l l a r plasma cell tumors involvement in multiple myeloma is the as observed in various locations. Arch Pathol initial or terminal manifestation of the 36:95-111. 1943 disease, the importance of recognizing the 13. Jim VKS: Plasmacytoma of orbit. Am J Ophthalmol 39:43-45, 1955 ocular changes in making the diagnosis of 14. Johnson BL, Storey J D : Proteinaceous cysts of the ciliary epithelium. Their clear nature and neoplastic plasmacytoma in these locations immunoelectrophoretic analysis in a case of is obvious. Neoplastic plasmacytoma or multiple myeloma. Arch Pathol 84:166-170, involvement in multiple myeloma of the 1970 conjunctiva alone has not been recorded 15. Parker ST: Plasmacytoma of lacrimal gland. Proc R Soc Med 31:130-136, 1937-1938 in the literature. 16. Pasmantier MW, Azar H: Extraskeletal spread

Orbital and conjunctival involvement in multiple myeloma. Report of a case.

Benjamin, Ivy, Taylor, Herbert, and Spindler, James: Orbital and conjuctival involvement in multiple myeloma. Report of a case. Am J Clin Pathol 63: 8...
5MB Sizes 0 Downloads 0 Views