Journal of Neuro-Ophthalmology, December 2003, Volume 23, Issue 4

Solitary Intracranial Extra-osseous Plasmacytoma Presenting with Ophthalmic Signs

Solitary plasmacytomas rarely develop in the skull, meninges, or brain. Ophthalmic signs as the initial manifestations of solitary intracranial plasmacytoma have rarely been described. We report the neuro-ophthalmologic, imaging, and pathologic findings for two patients. One patient presented with optic neuropathy, the second with bilateral sixth nerve palsies. Plasmacytoma is a treatable intracranial tumor that should be considered in the differential diagnosis of patients who present with optic neuropathy or sixth nerve palsy.

ORIGINAL CONTRIBUTION Solitary Intracranial Extra- osseous Plasmacytoma Presenting with Ophthalmic Signs Suzanne O. Brannan, FRCOphth, Bethan N. Matthews, FRCOphth, Vijay Savant, FRCSEd, Ray D. Brown, FRCOphth, Timothy D. Matthews, FRCOphth Abstract: Solitary plasmacytomas rarely develop in the skull, meninges, or brain. Ophthalmic signs as the ini­tial manifestations of solitary intracranial plasmacy­toma have rarely been described. We report the neuro-ophthalmologic, imaging, and pathologic findings for two patients. One patient presented with optic neuropathy, the second with bilateral sixth nerve palsies. Plasmacytoma is a treatable intracranial tumor that should be considered in the differential diagnosis of patients who present with optic neuropathy or sixth nerve palsy. ( JNeuro- Ophthalmol 2003; 23: 268- 271) CASE REPORTS Case 1 A 61- year- old woman with a history of Crohn's dis­ease and hemicolectomy presented with a 3- week history of painless deteriorating vision affecting the OS, and associ­ated with persistent left frontal headache of several weeks' duration. Best- corrected visual acuities were 20/ 20 OD and count fingers OS. There was a left afferent pupillary defect. Ocular motility examination was normal, as were slit- lamp biomicroscopy and funduscopy. Color vision, as assessed by Ishihara Pseudo- isochromatic Charts, was reduced in the OS. Constriction of the left nasal field was detected on Goldmann perimetry. The right visual field was full. Neu­rological examination elicited reduced sensation in the sec­ond division of the left fifth nerve. Cranial computed tomography ( CT) revealed a well-defined mass in the left cavernous sinus that extended an­teriorly through the optic foramen to abut the inferior aspect of the left optic nerve ( Fig. 1 A). Magnetic resonance imag­ing ( MRI) indicated a homogeneously enhancing mass at the apex of the orbit with possible involvement of the adja­cent bone, including the left clinoid process, and extending From the Birmingham & Midland Eye Center ( SOB, BNM, TDM) and the North Staffordshire Eye Department ( VS, RDB), Birmingham, United Kingdom. Address correspondence to Suzanne Brannan, Birmingham & Midland Eye Center, City Hospital NHS Trust, Dudley Road, Birmingham B18 7QU, UK; E- mail: suzanne@ brannan43. fsnet. co. uk into the orbit ( Fig. IB). These imaging features were inter­preted as indicating a left para- sellar meningioma. A left fronto- temporal craniotomy was performed for partial excision of the tumor. The portion encircling the left optic nerve was removed; the portion extending distally to the anterior limit of the cavernous was left behind. The pa­tient made a good postoperative recovery, but the visual acuity OS fell to no light perception. Light microscopy of the tumor showed atypical plasma cells ( Fig. 1C). Immuno-peroxidase staining for immunoglobulin light chains con­firmed kappa restriction, consistent with plasmacytoma ( Fig. ID). A myeloma screen included a negative marrow aspiration, skeletal survey, urine protein electrophoresis, and 24- hour urine for Bence- Jones protein. Serum protein electrophoresis demonstrated an abnormal monoclonal IgG kappa paraprotein band. The patient underwent radiotherapy to the residual tu­mor over a period of 4 weeks ( 50 Gray units). A 1- year follow- up MRI revealed complete resolution of the tumor ( Fig. IE). Visual function was unchanged. The patient's he­matological indices were within normal limits. Case 2 A 50- year- old man presented with sudden horizontal binocular diplopia. For the previous 8 weeks, he also had been complaining of constant headache that radiated from the left occiput to the fronto- parietal region. Apart from asthma, his past medical history was unremarkable. Best-corrected visual acuities were 20/ 20 OD, 20/ 30 OS. He could not abduct the OS beyond the midline. There was also a right abduction deficit, measuring approximately 50% of normal excursion. He had a left esotropia equal for near and distance that measured > 45 prism diopters. A diagnosis of bilateral sixth nerve palsy was made. No other abnormal neurologic signs were found, and his BP, blood sugar, and full blood counts were normal. MRI showed extensive tumor within the clivus ( Fig. 2A), bulging backwards and deforming the anterior aspect of the pre- pontine cistern ( Fig. 2B). The petrous apex on both sides was eroded, more on the left. The differential Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. 268 J Neuro- Ophthalmol, Vol. 23, No. 4, 2003 Intracranial Extra- osseus Plasmacytoma JNeuro- Ophthalmol, Vol. 23, No. 4, 2003 FIG. 1. Case 1. A: Axial CT shows a well- defined mass in the left cavernous sinus with extension through the optic foramen and erosion of the anterior clinoid process. B: Axial " T, enhanced MRI shows a homogeneously enhancing mass at the apex of the orbit with extension into the orbit. C: Hematoxylin- eosin stain of biopsy demonstrates atypical plasma cells. D: Immunperoxidase stain for light chains confirms kappa restriction ( dark-staining cells). E: Axial " T, enhanced MRI one year after surgery and chemotherapy shows resolution of the mass. diagnosis included metastasis, chordoma, plasmacytoma, or nasopharyngeal carcinoma. A transnasal biopsy of the mass showed atypical plasma cells, including a fewbinucleate forms. Immunohis-tochemical reactions indicated that this was a plasmacy­toma or an intracranial manifestation of multiple myeloma with kappa light chain monoclonality. A skeletal survey, bone marrow aspirate, serum electrophoresis, 24- hour urine collection for Bence- Jones protein, and all hematological parameters were normal. He was treated with dexamethasone 4mg/ d and sub­sequently underwent fractionated radiotherapy ( 45 Gray in Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. 269 JNeuro- Ophthalmol, Vol. 23, No. 4, 2003 Brannan et al. FIG. 2. Case 2. A: Axial T2 MRI shows an extensive homo­geneous mass in the clivus. B: Sagittal T1 MRI shows the mass extending into and deforming the anterior aspect of the pre- pontine cistern. 25 fractions), followed by CVAMP chemotherapy for 4 months. The sixth nerve palsies failed to resolve, so he un­derwent botulinum toxin injections into both medial recti, which allowed him to maintain binocular single vision with a slight head posture. Eight months later, he remained free of systemic symptoms. Follow- up neuroimaging has not been re­quested because of the known radiosensitivity of these tu­mors and the patient's stable clinical status. DISCUSSION Most cases of solitary extraosseous plasmacytoma occur in the nasopharynx, upper respiratory tract, lamina propria of the gastrointestinal tract, or other soft- tissue ar­eas ( 1). Intracranial plasmacytomas are rare ( 2). A review of the literature by Spaar ( 3) uncovered 32 cases up to 1980. In half of these cases, there was evidence of systemic dis­ease, whereas in the others the plasmacytoma was a soli­tary disorder. Patients with a solitary plasmacytoma often have a more favorable course than those with systemic myeloma. Therefore, determining whether a plasmacytoma is solitary is important prognostically. A study of 114 patients with solitary plasmacytoma ( 4) found that approximately 70% were alive after 10 years. In their case series, Bindal et al. ( 5) found that, in patients with intracranial plasmacytoma, multiple myeloma is unlikely to develop during the long term if it is not evident in the early postoperative period. The prognosis of solitary plasmacytoma is further altered by its location. Most patients with extraosseous plasmacy­toma, which commonly involves the head and neck region, may be curable because the tumor is radiosensitive ( 6). Included in the differential diagnosis of plasmacy­toma, based on morphologic criteria, is plasma cell granu­loma. The demonstration of immunoglobulin monoclonal-ity confirms a diagnosis of a neoplasm and excludes a diagnosis of granuloma, in which the immunofluorescent study would reveal a polyclonal population of plasma cells producing all three major classes of immunoglobulins. Clarke ( 7) classified cranial myelomas into three clinical groups: 1) Group 1, consisting of tumors that in­volve the skull base but that do not involve brain paren­chyma; they characteristically cause cranial nerve palsies; 2) Group 2, consisting of tumors that involve brain paren­chyma, with or without origin in the skull; and 3) Group 3, consisting of intra- orbital tumors. Our Case 1 belongs in Clarke's Group 2, demonstrat­ing a clinical picture of an intracranial tumor syndrome without bone or convexity dura mater involvement. The rar­est type of intracranial plasmacytoma, it presents with mul­tiple neurologic symptoms and signs, occasionally with those of a space- occupying lesion and raised intracranial pressure. An optic neuropathy usually would be associated with other neurologic signs ( 1). Isolated optic nerve com­pression from an intracranial plasmacytoma has been re­ported only rarely ( 8- 10). In two cases, an initial diagnosis of retrobulbar neuritis was made and the patients demon­strated transient alleviation of symptoms with systemic cor­ticosteroid therapy. To our knowledge, our Case 1 is the first reported example of a solitary intracranial extra- osseus plasmacytoma involving the cavernous sinus and the op­tic nerve. Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. 270 © 2003 Lippincott Williams & Wilkins Intracranial Extra- osseus Plasmacytoma JNeuro- Ophthalmol, Vol. 23, No. 4, 2003 Our Case 2 belongs in Clarke's Group 1, presenting with bilateral sixth nerve palsies associated with a lesion at the base of the skull. The sixth nerve is the most common cranial nerve affected by multiple myeloma, either in isola­tion or combination with other cranial neuropathies ( 7,11). Our Case 1 emphasizes the potential for plasmacy­toma to mimic memngioma on neuroimaging. Our Case 2 emphasizes how the elicitation of a subtle contralateral ab­duction deficit in a patient with an apparently unilateral ab­duction deficit changed the diagnostic focus. REFERENCES 1. Coppeto JR, Monteiro ML, Collias J, Uphoff D, Bear L. Foster- Kennedy syndrome caused by solitary intracranial plasmacytoma. Surg Neurol 1983; 19: 267- 72. 2. Mancardi GL, Mandybur TI. Solitary intracranial plasmacytoma. Cancer 1983; 51: 2226- 33. 3. Spaar FW. Paraproteinemias and multiple myeloma. In: Vinken PJ, Bruyn GW, eds. Handbook of Clinical Neurology, vol 39. Amster­dam: North Holland Publishing Co, 1980: 131- 80. 4. Jordan DR, Drouin J, Berry G, Watson AG. Intracranial plasmacy­toma associated with multiple myeloma. 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