A C T A O P H T H A L M O L O G I C A VOL. 5 4 1976
Stanford University Medical Center, Division of Ophthalmology (Head: A . Ralph Rosenthal), Department of Pathology (Head: David Korn) and Department of Radiology (Head: Malcolm A . Bagslzaw), Stanford, California, U S A
ORBITAL INVOLVEMENT BY PLASMACYTOMA Report of Two Cases BY
EEVA NIKOSKELAINEN, ANGELOS DELLAPORTA, THOMAS RICE, BARBARA EGBERT and BARTON LANE
T w o patients with orbital involvement of plasma cell myeloma are presented. T h e first patient presented a n isolated plasmacytoma in the orbit; the second patient had generalized plasma cell myeloma. In both cases X-rays and computed tomographic scanning gave valuable information and biopsy confirmed the diagnosis.
K e y words: orbita - plasmacytoma - tomography.
Plasma cell myeloma usually arises from the bone marrow, occasionally from extraosseous tissues. Sometimes myeloma presents itself as an apparently single skeletal lesion termed plasmacytoma. I n most cases plasmacytomas ultimately develop disseminated disease (Clarke 1953; Blodi 1975; Busse et al. 1975; McFadzean 1975; Rodman bk Font 1972). Clarke divides orbital myelomas into two categories - those originating in the orbit and those which involve the orbit secondarily (Clarke 1953). In this paper the clinical features of two cases with orbital plasma cell myeloma are described. The first patient had a solitary plasmacytoma in the orbit, the second presented secondary involvement of the orbit by generalized plasma cell myeloma. Rereived July 23, 1 S i G
7 55
Eeva Nikoskelainen. Angelos Dellaporta, Thomas Rice, Barbara Egbert and Barton Lane
Case Reports Case I . A 69-year-old white woman presented in August, 1975, with slowly progressive drooping of the right upper lid of two months duration. She had increased tearing and sensation of “ a film over the right eye”. There were no systemic symptoms. She had a G mm proptosis of the right eye but no diplopia was elicited and there was no restriction of the eye movements. A 35 mm diameter defect of the frontal bone
A
B
Fig. 1. Computerized tomographic scanning of the orbits. The thickness of the sections is 5 mm each. The plane of section of the right orbit is slightly superior to the corresponding section of the left due to the rotation of the patient’s head.
A: Scan at the level of the horizontal meridian of the right globe. The right optic nerve and lateral rectus muscle are displaced medially by soft tissue mass in the lateral wall of the orbit.
B: 5 mm above the level of scan A the soft tissue tumour has completely destroyed the lateral wall of the orbit (small arrowheads). Proptosis of the right globe is evident.
756
Orbital Inuoluernent by Plasrnacytoma was
palpable near the temporal-superior margin of the orbit. T h e patient reported pain
on pressure below the middle of the right eyebrow. Slit lamp examination showed
mild conjunctival chemosis and some irregularity of the epithelium of the upper third cornea of the right eye. T h e refractive media were clear and the fundus normal. T h e applanation tension was 19 in each eye. T h e corrected visual acuity was R. E.: 20140, J6, and L. E.: 20/20, J I . The pupils were of equal size and both direct a n d consensual reaction to light was normal. T h e visual fields on the Goldmann perimeter were normal. The left eye was normal. Radiographic studies including skull and sinus series demonstrated an extensive destructive process involving the right supra-orbital ridge, adjacent frontal bone, the lateral margin of the right frontal sinus and probably the supra-orbital ethmoid sinus in this region. T h e roof of the orbit and the zygomatic process of the frontal bone as well as the lateral extremity of the lesser wing ol the sphenoid also showed patchy destruction. T h e optic foramen and superior orbital fissure were normal. Computed tcrnographic scanning of the orbit (Fig. 1 A, 1 B) confirmed the proptosis of the right eye. T h c optic nerve was displaced medially by a soft-tissue mass in the right posterolateral orbit, which became denser following intravenous contrast material. T h e lateral wall of the orbit was destroyed by the tumour. There was no extension of the tumour into the brain. Brain scan showed increased radioactivity in the flow and static views over the area of the right orbit. Orbitotomy revealed a large bony defect of the lateral two-thirds of the orbital roof and the upper one-third of the lateral orbital wall by a grey tumour. Biopsy specimen showed sheets of plasma cells with characteristic eccentric nuclei, nuclear chromatin pattern, and juxtanuclear zones. Some of the cells were binucleated and others had prominent nucleoli. M G P stains showed uniform pyroniniophilia (Fig. 2). T h e histological diagnosis was plasmacytoma. There were no other bone involvements.
Fig. 2. Microscopic section of orbital biopsy in high power view reveals characteristic morphology of plasma cells with cartwheel chromatin, eccentric nuclei, binucleation and juxtanuclear clear zones. (Haematoxylin and Eosin, x 480).
757 Acta ophthal. 54, A
49
Eeua Nikoskelainen, Angelos Dellaporta, Thomas Rice, Barbara Egbert and Barton Lane
A
B
1
Fig. 3. Computerized tomographic scanning of the orbits and maxillary antra. A: Scanning at the level of the maxillary sinuses demonstrates a soft tissue mass filling the right antrum with extension medially into the nasopharynx (between arrows).
B: 5 mm above scan A the tumour extends into the floor of the orbit posteriorly (arrow).
Chest X-ray was normal. Serum and urine protein studies were negative for paraproteins. Bone marrow biopsy from the iliac crest was normal. The impression was that the patient had isolated plasmacytoma i n the right orbit with no evidence of systemic involvement. Five thousand rads of radiotherapy were given to the right orbit and the sinuses which subsequently led to regression of the proptosis.
Case 2. A 48-year-old white male presented in December, 1972, with a mass on his forehead, which had an underlying lytic lesion. Biopsy showed plasma cell myeloma. It was treated successfully with radiation. The patient was asymptomatic until September, 1973, when he presented with partially collapsed vertebra T-7. He was treated with bedrest, melphalen and prednisone with symptomatic improvement. In April, 1975, he manifested pancytopenia. H e suffered further compression of vertebra T-7 and
758
Orbital Involvement by Plasmacytoma developed neurologic signs of motor weakness in the left lower extremity. At this time he was treated with pelvic traction, prednisone, melphalen, blood transfusions and radiation therapy; 500 rads were administered to the thoracic vertebrae and 1000 rads to the mass on his sternum with good results. In July, 1975, the patient was referred to the eye clinic because of increased tearing and blurred vision in the right eye of one month duration, and diplopia of three days duration. His face was asymmetric with increase in the right nasal labial fold. He had a 2 mm proptosis of the right eye. There was moderate restriction of eye movements upwards and to the right, both associated with diplopia. No tumour or bony defects were palpable. Slit lamp examination showed slight conjunctival chemosis in the right eye. The refractive media were clear. The pupils were of equal size and both direct and consensual reaction to light was normal. Fundus examination showed choroidal folds extending from the right optic disc to the macula. Numerous drusen were around the macula in both eyes. Otherwise the fundi were normal. The visual acuity was 200120, J1 in both eyes. The visual fields with the Goldmann perimeter were normal. Radiographic studies of skull, orbits and facial bones showed extensive permeative lucencies involving the entire cranial vault as well as the right maxilla and mandible. There was marked demineralization of the dorsum sellae. The right maxillary antrum was opaque and the medial, lateral and posterior walls were destroyed. T h e roof of the antrum was markedly elevated by the underlying tumour. There were soft tissue densities in the anterior ethmoids and posterior sphenoid sinuses. Chest X-rays showed multiple destructive rib lesions, with associated extrapleural densities, consistent with the diagnosis of plasma cell myeloma. Bone survey for metastases showed diffuse involvement of all of the visualized bones. Computed tomographic scanning demonstrated a soft-tissue mass in the right maxillary antrum, extending superiorly into the posterior orbit (Fig. 3 A, 3 B). A second soft-tissue mass in the right orbit was seen more superiorly behind the globe (Fig. 4).
Fig. 4 . Computerized tomographic scanning at the level of the upper third of the orbits shows a second mass seen behind the right globe (arrowhead). No bony destruction or intracranial tumour is identified.
759 49’
Eezm Nikoskelainen, Angelos Dellaporta, Thomas Rice, Barbara Egbert and Barton Lane
Fig. 5. Microscopic section of maxillary sinus biopsy. High power details show some cells with characteristic features of plasma cells, others with large atypical nuclei. (Haematoxylin and Eosin, x 480).
In July, 1975, the patient had a biopsy of the tumour extending into the right nasal cavity as well as biopsy of the right maxillary tumour. T h e biopsies showed multiple fragments of atypical plasma cells with large nuclei with prominent nucleoli and abundant pyroniniophilic cytoplasm (Fig. 5). T h e features were similar to those of a previous bone marrow biopsy consistent with plasma cell myeloma. 'The right cranial sinuses were irradiated with 4000 rads. One month after radiotherapy there was marked resolution of the proptosis. I n November, 1975, there was no proptosis and no restriction of the movements of the right eye.
Discussion According to Rodman & Font (Rodman & Font 1972), the incidence of orbital involvement in plasma cell myeloma is rare. I t has to be differentiated from benign plasma cell accumulations which are produced by chronic inflammation (Reese 1964). In the present paper, two patients with orbital plasma cell myeloma were presented. Blurred vision, increased tearing, diplopia and drooping of the upper eye lid and proptosis were the presented symptoms. Computed tomographic scanning of the orbit is a new method to investigate orbital lesions (Gawler et al. 1974; Momose et al. 1975). I n both cases, this method gave valuable information of the extent and localization of the tumour. Biopsy confirmed the diagnosis in both patients. 760
Orbital Involvement by Plasmacytoma T h e first patient h a d solitary orbital plasmacytoma without evidence of generalized disease. Extended follow-up and detailed postmortem studies h a v e proven that solitary extramedullary plasmacytoma presents the first stage i n generalized disease. Later, patients develop other signs of plasma cell myeloma (Clarke 1953; Rubenzik & Tenzil 1975; Williams et al. 1972). Therefore, patients with orbital plasmacytomas h a v e to b e followed f o r generalized disease. T h e second patient developed orbital involvement as a complication of generalized plasma cell myeloma. 'The prognosis of this disease has improved during the last few years (Williams et al. 1972). W i t h the present therapeutic regimens significant degrees of improvement or remission can be achieved in a t least 70 to 80 per cent of all patients with plasma cell myeloma (Williams et al. 1972). I n both presented cases radiotherapy led to marked improvement of orbital involvement.
References Blodi F. C. (1976) Pathology of orbital bones. Amer. /. Ophthal. 81, 1-26. Busse H.. Miiller K. M. & Osmers F. (1975) Plasmozytom der Orbita. Klin. Mbl. Augenhrilk. 167, 818-823.
Clarke E. (1953) Plasma cell myeloma of the orbit. Brit. /. Ophthal. 37, 543-553. Gawler J., Sanders M. D., Bull J. W. D., duBoulay G. & Marshall J. (1974) Computer assisted tomography in orbital disease. Brit. /. Ophthal. 58, 571-587. MsFadzean R. M. (1975) Orbital plasma cell myeloma. Brit. /. Ophthal. 59, 164-165. Momose K. New P. F. J., Grove A. S. Jr. & Scott W. R. (1975) The use of computed tomography in ophthalmology. Radiology 115, 361-368. Reese A. B. (1964) Tumors of the eye, 2nd Ed. Hoeber, New York, p. 475476. Rodman H. I. 8: Font R. L. (1972) Orbital involvement in multiple myeloma; Review of the literature and report of three cases. Arch. Ophthal. 87, 30-35. Kubenzik R. k Tenzil R. (1975) Multiple myeloma involving the lacrimal gland. Annals of O/jhthnl. 7, 1077-1078. Williams W. J., Beutler E., Erslev A. J. & Rundles R. V. (1972) Hcmatology, McGrawHill Book Company, Inc., pp. 958-967. J.?
Aictlimr's riddress:
E. Nikoskelainen, M. D., Stanford University Medical Center, Division of Ophthalmology, Stanford, California 94305, USA.
761
Stanford University Medical Center, Division of Ophthalmology (Head: A . Ralph Rosenthal), Department of Pathology (Head: David Korn) and Department of Radiology (Head: Malcolm A . Bagslzaw), Stanford, California, U S A
ORBITAL INVOLVEMENT BY PLASMACYTOMA Report of Two Cases BY
EEVA NIKOSKELAINEN, ANGELOS DELLAPORTA, THOMAS RICE, BARBARA EGBERT and BARTON LANE
T w o patients with orbital involvement of plasma cell myeloma are presented. T h e first patient presented a n isolated plasmacytoma in the orbit; the second patient had generalized plasma cell myeloma. In both cases X-rays and computed tomographic scanning gave valuable information and biopsy confirmed the diagnosis.
K e y words: orbita - plasmacytoma - tomography.
Plasma cell myeloma usually arises from the bone marrow, occasionally from extraosseous tissues. Sometimes myeloma presents itself as an apparently single skeletal lesion termed plasmacytoma. I n most cases plasmacytomas ultimately develop disseminated disease (Clarke 1953; Blodi 1975; Busse et al. 1975; McFadzean 1975; Rodman bk Font 1972). Clarke divides orbital myelomas into two categories - those originating in the orbit and those which involve the orbit secondarily (Clarke 1953). In this paper the clinical features of two cases with orbital plasma cell myeloma are described. The first patient had a solitary plasmacytoma in the orbit, the second presented secondary involvement of the orbit by generalized plasma cell myeloma. Rereived July 23, 1 S i G
7 55
Eeva Nikoskelainen. Angelos Dellaporta, Thomas Rice, Barbara Egbert and Barton Lane
Case Reports Case I . A 69-year-old white woman presented in August, 1975, with slowly progressive drooping of the right upper lid of two months duration. She had increased tearing and sensation of “ a film over the right eye”. There were no systemic symptoms. She had a G mm proptosis of the right eye but no diplopia was elicited and there was no restriction of the eye movements. A 35 mm diameter defect of the frontal bone
A
B
Fig. 1. Computerized tomographic scanning of the orbits. The thickness of the sections is 5 mm each. The plane of section of the right orbit is slightly superior to the corresponding section of the left due to the rotation of the patient’s head.
A: Scan at the level of the horizontal meridian of the right globe. The right optic nerve and lateral rectus muscle are displaced medially by soft tissue mass in the lateral wall of the orbit.
B: 5 mm above the level of scan A the soft tissue tumour has completely destroyed the lateral wall of the orbit (small arrowheads). Proptosis of the right globe is evident.
756
Orbital Inuoluernent by Plasrnacytoma was
palpable near the temporal-superior margin of the orbit. T h e patient reported pain
on pressure below the middle of the right eyebrow. Slit lamp examination showed
mild conjunctival chemosis and some irregularity of the epithelium of the upper third cornea of the right eye. T h e refractive media were clear and the fundus normal. T h e applanation tension was 19 in each eye. T h e corrected visual acuity was R. E.: 20140, J6, and L. E.: 20/20, J I . The pupils were of equal size and both direct a n d consensual reaction to light was normal. T h e visual fields on the Goldmann perimeter were normal. The left eye was normal. Radiographic studies including skull and sinus series demonstrated an extensive destructive process involving the right supra-orbital ridge, adjacent frontal bone, the lateral margin of the right frontal sinus and probably the supra-orbital ethmoid sinus in this region. T h e roof of the orbit and the zygomatic process of the frontal bone as well as the lateral extremity of the lesser wing ol the sphenoid also showed patchy destruction. T h e optic foramen and superior orbital fissure were normal. Computed tcrnographic scanning of the orbit (Fig. 1 A, 1 B) confirmed the proptosis of the right eye. T h c optic nerve was displaced medially by a soft-tissue mass in the right posterolateral orbit, which became denser following intravenous contrast material. T h e lateral wall of the orbit was destroyed by the tumour. There was no extension of the tumour into the brain. Brain scan showed increased radioactivity in the flow and static views over the area of the right orbit. Orbitotomy revealed a large bony defect of the lateral two-thirds of the orbital roof and the upper one-third of the lateral orbital wall by a grey tumour. Biopsy specimen showed sheets of plasma cells with characteristic eccentric nuclei, nuclear chromatin pattern, and juxtanuclear zones. Some of the cells were binucleated and others had prominent nucleoli. M G P stains showed uniform pyroniniophilia (Fig. 2). T h e histological diagnosis was plasmacytoma. There were no other bone involvements.
Fig. 2. Microscopic section of orbital biopsy in high power view reveals characteristic morphology of plasma cells with cartwheel chromatin, eccentric nuclei, binucleation and juxtanuclear clear zones. (Haematoxylin and Eosin, x 480).
757 Acta ophthal. 54, A
49
Eeua Nikoskelainen, Angelos Dellaporta, Thomas Rice, Barbara Egbert and Barton Lane
A
B
1
Fig. 3. Computerized tomographic scanning of the orbits and maxillary antra. A: Scanning at the level of the maxillary sinuses demonstrates a soft tissue mass filling the right antrum with extension medially into the nasopharynx (between arrows).
B: 5 mm above scan A the tumour extends into the floor of the orbit posteriorly (arrow).
Chest X-ray was normal. Serum and urine protein studies were negative for paraproteins. Bone marrow biopsy from the iliac crest was normal. The impression was that the patient had isolated plasmacytoma i n the right orbit with no evidence of systemic involvement. Five thousand rads of radiotherapy were given to the right orbit and the sinuses which subsequently led to regression of the proptosis.
Case 2. A 48-year-old white male presented in December, 1972, with a mass on his forehead, which had an underlying lytic lesion. Biopsy showed plasma cell myeloma. It was treated successfully with radiation. The patient was asymptomatic until September, 1973, when he presented with partially collapsed vertebra T-7. He was treated with bedrest, melphalen and prednisone with symptomatic improvement. In April, 1975, he manifested pancytopenia. H e suffered further compression of vertebra T-7 and
758
Orbital Involvement by Plasmacytoma developed neurologic signs of motor weakness in the left lower extremity. At this time he was treated with pelvic traction, prednisone, melphalen, blood transfusions and radiation therapy; 500 rads were administered to the thoracic vertebrae and 1000 rads to the mass on his sternum with good results. In July, 1975, the patient was referred to the eye clinic because of increased tearing and blurred vision in the right eye of one month duration, and diplopia of three days duration. His face was asymmetric with increase in the right nasal labial fold. He had a 2 mm proptosis of the right eye. There was moderate restriction of eye movements upwards and to the right, both associated with diplopia. No tumour or bony defects were palpable. Slit lamp examination showed slight conjunctival chemosis in the right eye. The refractive media were clear. The pupils were of equal size and both direct and consensual reaction to light was normal. Fundus examination showed choroidal folds extending from the right optic disc to the macula. Numerous drusen were around the macula in both eyes. Otherwise the fundi were normal. The visual acuity was 200120, J1 in both eyes. The visual fields with the Goldmann perimeter were normal. Radiographic studies of skull, orbits and facial bones showed extensive permeative lucencies involving the entire cranial vault as well as the right maxilla and mandible. There was marked demineralization of the dorsum sellae. The right maxillary antrum was opaque and the medial, lateral and posterior walls were destroyed. T h e roof of the antrum was markedly elevated by the underlying tumour. There were soft tissue densities in the anterior ethmoids and posterior sphenoid sinuses. Chest X-rays showed multiple destructive rib lesions, with associated extrapleural densities, consistent with the diagnosis of plasma cell myeloma. Bone survey for metastases showed diffuse involvement of all of the visualized bones. Computed tomographic scanning demonstrated a soft-tissue mass in the right maxillary antrum, extending superiorly into the posterior orbit (Fig. 3 A, 3 B). A second soft-tissue mass in the right orbit was seen more superiorly behind the globe (Fig. 4).
Fig. 4 . Computerized tomographic scanning at the level of the upper third of the orbits shows a second mass seen behind the right globe (arrowhead). No bony destruction or intracranial tumour is identified.
759 49’
Eezm Nikoskelainen, Angelos Dellaporta, Thomas Rice, Barbara Egbert and Barton Lane
Fig. 5. Microscopic section of maxillary sinus biopsy. High power details show some cells with characteristic features of plasma cells, others with large atypical nuclei. (Haematoxylin and Eosin, x 480).
In July, 1975, the patient had a biopsy of the tumour extending into the right nasal cavity as well as biopsy of the right maxillary tumour. T h e biopsies showed multiple fragments of atypical plasma cells with large nuclei with prominent nucleoli and abundant pyroniniophilic cytoplasm (Fig. 5). T h e features were similar to those of a previous bone marrow biopsy consistent with plasma cell myeloma. 'The right cranial sinuses were irradiated with 4000 rads. One month after radiotherapy there was marked resolution of the proptosis. I n November, 1975, there was no proptosis and no restriction of the movements of the right eye.
Discussion According to Rodman & Font (Rodman & Font 1972), the incidence of orbital involvement in plasma cell myeloma is rare. I t has to be differentiated from benign plasma cell accumulations which are produced by chronic inflammation (Reese 1964). In the present paper, two patients with orbital plasma cell myeloma were presented. Blurred vision, increased tearing, diplopia and drooping of the upper eye lid and proptosis were the presented symptoms. Computed tomographic scanning of the orbit is a new method to investigate orbital lesions (Gawler et al. 1974; Momose et al. 1975). I n both cases, this method gave valuable information of the extent and localization of the tumour. Biopsy confirmed the diagnosis in both patients. 760
Orbital Involvement by Plasmacytoma T h e first patient h a d solitary orbital plasmacytoma without evidence of generalized disease. Extended follow-up and detailed postmortem studies h a v e proven that solitary extramedullary plasmacytoma presents the first stage i n generalized disease. Later, patients develop other signs of plasma cell myeloma (Clarke 1953; Rubenzik & Tenzil 1975; Williams et al. 1972). Therefore, patients with orbital plasmacytomas h a v e to b e followed f o r generalized disease. T h e second patient developed orbital involvement as a complication of generalized plasma cell myeloma. 'The prognosis of this disease has improved during the last few years (Williams et al. 1972). W i t h the present therapeutic regimens significant degrees of improvement or remission can be achieved in a t least 70 to 80 per cent of all patients with plasma cell myeloma (Williams et al. 1972). I n both presented cases radiotherapy led to marked improvement of orbital involvement.
References Blodi F. C. (1976) Pathology of orbital bones. Amer. /. Ophthal. 81, 1-26. Busse H.. Miiller K. M. & Osmers F. (1975) Plasmozytom der Orbita. Klin. Mbl. Augenhrilk. 167, 818-823.
Clarke E. (1953) Plasma cell myeloma of the orbit. Brit. /. Ophthal. 37, 543-553. Gawler J., Sanders M. D., Bull J. W. D., duBoulay G. & Marshall J. (1974) Computer assisted tomography in orbital disease. Brit. /. Ophthal. 58, 571-587. MsFadzean R. M. (1975) Orbital plasma cell myeloma. Brit. /. Ophthal. 59, 164-165. Momose K. New P. F. J., Grove A. S. Jr. & Scott W. R. (1975) The use of computed tomography in ophthalmology. Radiology 115, 361-368. Reese A. B. (1964) Tumors of the eye, 2nd Ed. Hoeber, New York, p. 475476. Rodman H. I. 8: Font R. L. (1972) Orbital involvement in multiple myeloma; Review of the literature and report of three cases. Arch. Ophthal. 87, 30-35. Kubenzik R. k Tenzil R. (1975) Multiple myeloma involving the lacrimal gland. Annals of O/jhthnl. 7, 1077-1078. Williams W. J., Beutler E., Erslev A. J. & Rundles R. V. (1972) Hcmatology, McGrawHill Book Company, Inc., pp. 958-967. J.?
Aictlimr's riddress:
E. Nikoskelainen, M. D., Stanford University Medical Center, Division of Ophthalmology, Stanford, California 94305, USA.
761
