Orbital solitary fibrous tumor: radiographic and histopathologic correlations.

OBJECTIVE: To correlate the clinicopathologic and radiographic features characteristic of orbital solitary fibrous tumor (SFT). METHODS: The diagnostic features and clinical outcome of seven adults with orbital SFT are retrospectively outlined. Orbital imaging was performed by ultrasonography, computed tomography, or magnetic resonance imaging. Some cases were imaged by multiple modalities. Histopathologic examination of each tumor specimen included standard light and immunohistochemical stains. RESULTS: Heterogeneous internal composition was better appreciated on magnetic resonance imaging than on computed tomography. All cases undergoing magnetic resonance imaging showed T1 isointensity and T2 hypointensity relative to gray matter. Strong, generalized immunohistochemical reactivity to vimentin and CD34 validated the diagnosis of SFT and differentiated the specimens from other spindle cell neoplasms. After complete tumor resection, our patients remain tumor free with postoperative intervals of 15 to 45 months. CONCLUSIONS: Solitary fibrous tumor has now been reported in 26 orbits. No physical finding is pathognomonic, but several imaging traits are highly characteristic. Intralesional image heterogeneity and a predominantly low T2 signal intensity are distinctive of SFT. Complete tumor resection and immunohistologic specimen evaluation are emphasized. Clinicians should consider the diagnosis of SFT when confronted with an adult patient having an orbital soft tissue mass demonstrating the distinctive magnetic resonance imaging findings.

Fine-needle aspiration for the diagnosis of primary epithelial tumors of the lacrimal gland and ocular adnexa.

Results of fine-needle aspiration (FNA) of solid-tissue neoplasms arising in the periocular glands are infrequently reported in the literature. To our knowledge, no previous series relating to this topic exist. Neoplastic processes that arise in the semiconfined area of the orbit behave as space-occupying lesions. Such lesions can exert significant pressure on the globe, be responsible for altered vision, and result in proptosis. When noninvasive techniques fail to confirm or rule out the suspicion of a neoplastic lacrimal or adnexal lesion, FNA may be of use in establishing a diagnosis in an efficient, reliable, timely, cost-effective, and safe manner. During the 14-yr interval from 1986-1999, 77 orbital/ocular needle aspiration biopsies were conducted by staff ophthalmologists at Allegheny General Hospital (Pittsburgh, PA). Review of the diagnoses for these specimens revealed seven primary solid-tissue lesions of the lacrimal gland and other adnexal glands, all arising in adult patients (age range, 45-92 yr; mean age, 74 yr). Primary lacrimal and adnexal gland neoplasms were found to represent approximately 9% of orbital fine-needle aspirations (7/79). The 7 cases included 3 lacrimal gland lesions diagnosed as benign mixed tumors, 3 lesions diagnosed as adenoid cystic carcinoma of the lacrimal gland, and 1 tumor diagnosed as sebaceous carcinoma of the meibomian holocrine glands. Cytologic diagnoses were rendered using standard criteria for salivary gland-type tumors. Tissue confirmation was available from surgical follow-up in 4 of the 7 cases, with 100% correlation. Although primary neoplasms of the lacrimal gland and glands of the eyelids are rare, accurate diagnoses of such lesions may be established with minimally invasive aspiration techniques. Preoperative aspiration biopsy diagnoses provide a great advantage to ophthalmic surgeons who routinely operate in a conservative fashion in an area of the body requiring great attention to cosmesis. Our experience indicates that FNA is a reliable and effective tool in the diagnosis and management of primary lacrimal and ocular adnexal tumors.

Surgery for orbital tumors. Part II: transorbital approaches.

Orbital tumors can be excised or biopsy samples obtained via transorbital approaches, especially those located in the anterior two thirds of the orbit. The indications and various surgical steps will be reviewed for the anterior, the anteromedial, and the lateral approaches. Some of these approaches can be combined or extended to accommodate large or deep-seated tumors.

Fine-needle aspiration for the diagnosis of orbital hematolymphoid lesions.

Ocular and periocular hematolymphoid diseases are a diverse group of lesions affecting various soft tissue structures within the orbital cavity. Lymphoid proliferations in particular are among the most commonly diagnosed entities in orbital pathology. When noninvasive techniques fail to confirm or rule out the suspicion of orbital neoplasia, fine-needle aspiration (FNA) may be of use in establishing a diagnosis in a reliable, timely, cost-effective and safe manner. From 1986 to 1999, 79 orbital/ocular needle aspiration biopsies were conducted by staff ophthalmologists at Allegheny General Hospital. Slides from these cases and corresponding reports were pulled from the cytology files and grouped into the two broad categories of hematolymphoid and other. Specimens came from patients ranging in age from 14 to 88 years (mean, 64 years) with eight patients having known histories of hematolymphoid disorders. Immunocytochemical (ICC) studies were performed in 33% of the cases (14/43). Review of the diagnoses for the 79 aspiration specimens revealed 30 cases diagnosed as lymphoma/atypical lymphocytic infiltrate, cases diagnosed as inflammation or abscess, three cases diagnosed as plasmacytoma, three cases called suboptimal with scant inflammatory cells, and one case of Langerhans' cell histiocytosis. Hematolymphoid diagnoses accounted for 54% (43/79) of all diagnoses. Histologic correlation was available in 33% (14/43) of the cases (nine cases diagnosed as cytologically atypical/malignant and five cases called cytologically benign/suboptimal) with 100% correlation. Hematolymphoid lesions of the orbit are readily diagnosed by FNA. Because many hematolymphoid malignancies are treated as systemic or multiorgan system diseases and because ocular lymphomas may also involve the central nervous system, nonsurgical attempts at diagnosis have the potential to spare the patient procedural morbidity which may be associated with open biopsy. Our experience indicates that the combination of FNA, judicious use of immunocytochemical studies, and correlation with pertinent clinical information and imaging studies allows for reliable and effective classification and diagnosis of orbital hematolymphoid lesions.

The clinical spectrum of schwannomas presenting with visual dysfunction: a clinicopathologic study of three cases.

Schwannomas (neurilemomas) are benign tumors that arise from Schwann cells in the peripheral nervous system. The most commonly involved nerves that cause neuro-ophthalmic manifestations are cranial nerves V and VIII. In this series of three women, schwannomas presented as intraconal masses that mimicked a cavernous hemangioma, a superior orbital mass transgressing the superior orbital fissure, and an expansive frontal lobe mass with clinical symptoms and signs of increased intracranial pressure. Although all three complained of visual blurring, none of our patients presented with Vth or VIIIth cranial nerve dysfunction. Histopathologic studies demonstrated well-circumscribed, encapsulated spindle-cell lesions with classic Antoni A and B patterns. Histopathologic examination is essential to confirm the diagnosis of a schwannoma that may be otherwise clinically confusing. Direct optic nerve compression, globe indentation with induced hyperopia, or increased intracranial pressure with optic nerve compromise may be responsible for visual symptoms. A multidisciplinary approach is often required because of the size and location of schwannomas.

Low-dose radiotherapy for lymphoid lesions of the orbit and ocular adnexa.

PURPOSE: There is no agreement within the radiation oncology and ophthalmic communities regarding the treatment of lymphoid lesions of the orbit and ocular adnexa. The authors report their experience with the use of low-dose radiation therapy for malignant and benign lymphoid masses of the orbital region in a series of 54 patients treated between 1985 and 1993. METHODS: All patients received 2 Gy per day for a total of 24 Gy, except when the lesion was extensive, in which case the therapy was 1.5 Gy per day for a total of 25.5 Gy. A diagnosis was established by incisional surgical biopsy in 26 patients and aspiration cytology in 28 patients. Those with a malignant or an indeterminate diagnosis were evaluated with a modified Ann Arbor staging system. RESULTS: Low-dose radiation therapy produced a complete response in 100% of the orbital lymphoid lesions. This local control was maintained in 52 patients (96%) for the first year and in 51 patients (95%) for 5 or more years with a mean follow-up of 7 years. One patient died of causes unrelated to the malignant lymphoma after the first year of observation. The mean age of the 54 patients was 67 years, and the range was 37 to 90 years. The mean ages of presentation for each location were: orbit, 67 years; conjunctiva, 68 years; lacrimal gland, 66 years; and eyelids, 72 years. The female-to-male ratio was 1.25:1 (34 women and 20 men). In this series, 9 patients had benign processes, 38 patients had non-Hodgkin's lymphoma, and 7 patients had abnormalities of indeterminate cause. All histologic subtypes of non-Hodgkin's lymphoma involving the orbit responded equally well to therapy. Forty-five patients had clinically staged disease as follows: stage I, 21 patients; stage II, 4 patients; stage III, 2 patients; and stage IV, 18 patients. Benign disease, diagnosed in 9 patients, was not staged. CONCLUSION: Low-dose radiation therapy proved effective in treating lymphoid lesions of the orbital area. No treatment-limiting complications occurred. The only early side effects were mild xerophthalmia and chemosis in 50% of patients, and the only chronic side effect was mild xerophthalmia in 33% of patients. Cataracts, corneal ulcerations, and retinal injury were not observed.

Significance of serum antibodies reactive with flavoprotein subunit of succinate dehydrogenase in thyroid associated orbitopathy.

AIMS: Thyroid associated orbitopathy (TAO) is an autoimmune disorder of extraocular muscles and orbital connective tissue. Identification of the principal target antigens would help the understanding of the pathogenesis of the disease and possibly lead to the development of specific therapies in the future. The purpose of this study was to measure serum antibodies against the flavoprotein subunit of succinate dehydrogenase in patients with TAO and correlate their presence with factors of TAO. METHODS: Sera of patients with active TAO of 6 months' duration or less were tested for antibodies against the flavoprotein subunit of succinate dehydrogenase. Clinical data were obtained by retrospective review of patients' charts. Enzyme linked immunosorbent assay was used to test sera for serum antibodies against purified succinate dehydrogenase. RESULTS: 38 patients with TAO and 32 healthy age and sex matched controls were included in the study. Anti-flavoprotein antibodies were detected in 24 out of 38 patients with TAO (63.16%) and in five out of 32 healthy controls (15.63%) (p<0.01). Neither age, sex, duration of thyroid disease, thyroid status, treatment of thyroid disease, smoking history, duration of orbitopathy, activity of orbitopathy, nor the presence of lid retraction were significantly associated with the presence of serum anti-flavoprotein antibodies (p>0.05). However, the total number of rectus muscles affected in both eyes of the patients was significantly correlated with the finding of a positive antibody test (p<0.05). CONCLUSIONS: Serum antibodies reactive with the flavoprotein subunit of succinate dehydrogenase are associated with extraocular muscle involvement in active TAO of recent onset.

Liposarcoma of the orbit: a management challenge.

A previously healthy 35-year-old man experiencing slowly progressive, painless proptosis of the right eye. Visual function was normal, but supraduction was limited. Computed tomography revealed a superior, extraconal orbital mass. Subtotal excision was performed, and a diagnosis of liposarcoma was rendered only with expert analysis. Despite subsequent orbital exenteration and postoperative radiation, a local recurrence developed 5 years later. The clinical features that predict recurrence, and management options that may promote longevity, are discussed.

The posterior inferior orbitotomy.

Four patients with orbital apex tumors between the optic nerve and inferior rectus underwent a posterior inferior orbitotomy through the maxillary sinus. Three tumors were removed successfully and the fourth was not located, but the visual function improved after surgery, presumably owing to decompression of the posterior orbital floor. The technique was carried out through a standard Caldwell-Luc approach through the maxillary sinus. The posterior inferior orbital wall was removed and the inferior rectus was retracted either laterally or medially to gain access to the tumor, which was removed microsurgically. The authors believe this approach provides a reasonably safe alternative to remove small, well-circumscribed, inferior posterior orbital apical tumors. It also avoids dissection through the orbit from other directions with the inherent risks of damaging overlying vital structures.

Eye muscle antibodies in patients with ocular myasthenia gravis: possible mechanism for eye muscle inflammation in acetylcholine-receptor antibody-negative patients.

Myasthenia gravis is an organ-specific autoimmune disorder generally thought to be caused by an antibody-mediated attack against the skeletal muscle nicotinic acetylcholine (Ach) receptor (AchR) at the neuromuscular junction. Extraocular muscle weakness and double vision are present in about 90% of patients with myasthenia gravis and are the predominant complaints in about 20% of patients, when the condition is called ocular myasthenia gravis (OMG). While serum antibodies against the AchR are detected in most patients with generalized myasthenia gravis (GMG), they are not found in about one-third of patients with the ocular variety, and epidemiological, clinical, and serological studies suggest that OMG and GMG are two separate diseases. Both forms of myasthenia gravis are sometimes associated with thyroid autoimmunity or thyroid-associated ophthalmopathy (TAO). We have therefore tested the sera of patients with GMG and OMG by Western blotting for antibodies against porcine eye muscle membrane proteins in general, and by enzyme-linked immunosorbent assays (ELISA) specifically for reaction with two skeletal muscle antigens which are prominent marker antigens for TAO, namely, the calcium-binding protein calsequestrin and the so-called "64-kDa protein." The 64-kDa protein has recently been identified as the flavoprotein subunit of mitochondrial succinate dehydrogenase. Patients with ophthalmopathy and myasthenia were excluded. Nine of the patients had associated Graves' hyperthyroidism without evident ophthalmopathy and one had Hashimoto's thyroiditis. Antibodies against porcine eye muscle membrane antigens of M(r) 15-110 kDa were detected in patients with GMG or OMG, one or more antibodies being detected in 100% of patients with GMG and in 88% of those with OMG. The most frequently found antibodies were those targeting eye muscle membrane proteins of 15, 67, and 110 kDa. Antibodies reactive with purified calsequestrin (63 kDa) were detected in 21% of patients with OMG but in no patient with GMG. Antibodies recognizing purified succinate dehydrogenase (67 kDa) were found in 42% of patients with OMG, in 100% (5 of 5) of patients with GMG, and in 48% of all patients with myasthenia gravis not associated with Graves' hyperthyroidism. There was no close correlation between any eye muscle-reactive antibody and antibodies against the AchR in either group of myasthenic patients. The findings support the notion that immunoreactivity against skeletal muscle proteins other than the AchR may play a role in the development of the muscle weakness in AchR antibody-negative patients with OMG and GMG, although it is unlikely that any of the antibodies demonstrated in this study are directly implicated. Similarly, while the demonstration of antibodies reactive with eye muscle antigens associated with TAO in patients with OMG raises the possibility that the link between the ocular lesions of myasthenia gravis and Graves' disease may be autoimmunity against a common antigen(s), it is more likely that both disorders are mediated by cytotoxic T cells recognizing another cell membrane antigen, such as the novel thyroid and eye muscle shared protein G2s, and that serum antibodies reactive with succinate dehydrogenase Fp subunit and calsequestrin are markers of an immune-mediated eye muscle reaction.

Reevaluation of the prevalences of serum autoantibodies reactive with "64-kd eye muscle proteins" in patients with thyroid-associated ophthalmopathy.

Serum autoantibodies reactive with eye muscle proteins of "64 kilodaltons (kd)" are frequently found in patients with Graves' hyperthyroidism and thyroid-associated ophthalmopathy (TAO). Earlier, we cloned a 64-kd protein that was identified as calsequestrin, a calcium-binding protein localized in the sarcoplasmic reticulum of striated muscle and extensively studied another cloned 64-kd protein, called 1D, which is expressed in thyroid and eye muscle, and some other tissues. Using a monoclonal antibody against calsequestrin, a polyclonal antibody against 1D and a TAO patient serum reactive with the "64-kd protein," as probes, we performed Western blots of porcine eye muscle membrane. We identified three different proteins in the 63 to 67 kd molecular weight range that were targeted by antibodies in sera from patients with TAO. It was not possible to differentiate antibodies reactive with calsequestrin and 1D because these two proteins have very similar molecular weights--63 to 64 kd--and band appearance in Western blotting. A 67-kd protein was most frequently recognized by TAO patients' sera. Serum antibodies reactive with the 67-kd protein were detected in 73% of patients with active TAO of 1 year duration or less, in 37% of patients with TAO of more than 3 years' duration, in 35% with Graves' hyperthyroidism without evident ophthalmopathy, in 30% of patients with Hashimoto's thyroiditis, and in 16% of normal subjects. Serum antibodies reactive with calsequestrin/1D were detected in 47% of patients with active TAO of less than 1 year, in 22% of patients with TAO longer than 3 years, 17% with Graves' hyperthyroidism without evident ophthalmopathy, in 10% of patients with Hashimoto's thyroiditis, and in 21% of normal subjects. The prevalence of anti-67-kd protein antibodies in TAO patients corresponded to those reactive with the so called "64-kd protein" that we have reported previously. In conclusion, we were able to improve the accuracy of the Western blots by comparing the molecular weight of positive bands using specific antibodies reactive with eye muscle antigens as probes. The previously recognized, and extensively studied, "64-kd protein" is now shown to have a molecular weight of 67 kd. The role of the various eye muscle antibodies in the diagnosis and management of the ophthalmopathy associated with Graves' hyperthyroidism needs to be addressed in prospective studies using purified or recombinant full-length proteins.

Solitary fibrous tumor of the orbit.

Solitary fibrous tumor (SFT) of the orbit is a very rare lesion that may be misdiagnosed as fibrous histiocytoma, hemangiopericytoma, or other orbital tumors. We present a 62-year-old man who presented with painless proptosis, 20 years following left eye enucleation for a presumed neurofibroma. On T2-weighted magnetic resonance imaging (MRI), a hypointense tumor almost filled his entire left orbit. There was no intracranial extension. The specimen obtained at orbital exenteration was consistent with the histologic, immunohistochemical, and electron microscopic findings of SFT. The tumor was positive for vimentin and CD34 staining but negative for S-100 protein and epithelial membrane antigen. Only nine other cases of SFT of the orbit have been documented in the literature. Recognition of SFT of the orbit as a distinct pathologic entity and further follow-up of published cases are needed to determine the prognosis of this rare lesion.

The evaluation and treatment of extraocular motility deficits.

Binocular diplopia, monocular diplopia and oscillopsia may be manifestations of skull base lesions or may result from skull base surgery. An ophthalmologic perspective on the diagnosis and treatment of these extraocular motility deficits is reviewed.

Antibodies against striated muscle, connective tissue and nuclear antigens in patients with thyroid-associated ophthalmopathy: should Graves' disease be considered a collagen disorder?

The identity and subcellular localization of the principal extraocular muscle (EOM) antigens and prevalences of the corresponding serum autoantibodies in thyroid-associated ophthalmopathy (TAO) need to be clarified. We have used porcine eye muscle tissue, which expresses all autoantigens identified in human tissue, as substrate in an indirect immunofluorescence assay. Several different patterns of antibody binding to EOM tissue antigens were observed with sera from patients with TAO namely, membrane, cytoplasmic, interstitial (endomysial) and nuclear. Overall, sera from 75% of patients with TAO contained one or more antibodies reactive with EOM, compared to 32% of patients with Graves' hyperthyroidism, 38% with Hashimoto's thyroiditis, and 16% of normals. All sera which reacted with EOM membrane or cytoplasmic antigens also reacted with the same antigen(s) in other skeletal muscle, but not in the other tissues tested. Sera from 31% of patients with TAO, but only 7% of those with Hashimoto's thyroiditis, and no patient with Graves' hyperthyroidism without evident ophthalmopathy, contained antinuclear antibodies (ANA). The most common nuclear fluorescence pattern was the finely speckled type typically associated with anti-Sm or anti-RNP antibodies. Significant positive correlations in patients with TAO were found between (i) EOM dysfunction and ANA (ii) eye disease of < 1 yr duration and EOM membrane-reactive antibodies and (iii) eye disease of < 1 yr duration and interstitial (endomysial) tissue-reactive antibodies. Although patients with Graves' disease do not usually exhibit other signs or immunologic features of a generalized collagen disorder, the finding of high prevalences of ANA and anti-striated muscle antibodies and, less often, anti-connective tissue antibodies in patients with ophthalmopathy, is consistent with it being a collagen-like disorder of the striated muscle, connective tissue and the thyroid. The reason why the inflammatory process is mainly limited to these tissues is unclear although cross reaction of ANA with tissue specific proteins or increased expression of muscle and connective tissue antigens in the orbit and skin, are possibilities.

The role of postoperative irradiation in the management of sphenoid wing meningiomas. A preliminary report.

PURPOSE: To determine whether postoperative radiation therapy decreases recurrence rates in subtotally excised and recurrent sphenoid wing meningiomas. METHODS: Patients with primary subtotally excised and recurrent sphenoid wing meningiomas who underwent surgery between 1981 and 1994 (n = 105) were prospectively followed for recurrence. Postoperative radiation was not recommended in patients who had complete excision; therefore, their recurrence rates were not evaluated in this study. Patients with malignant meningiomas also were excluded from analysis. Recurrence was defined as evidence of tumor growth on neuroimaging with or without clinical symptoms. RESULTS: Follow-up information was available for 86 patients; 69 had primary subtotally excised tumors and 17 had recurrent tumors. Follow-up information was unavailable in the remaining 19 patients. Tumor location and histopathology, type of surgery performed, and patient sex and age were similar in the irradiated and nonirradiated subgroups. Postoperative irradiation was delivered to 31 patients with primary tumors and 11 with recurrent tumors; none of these 42 patients had recurrence during a mean observation period of 4.2 and 3.5 years, respectively. The nonirradiated group consisted of 38 patients with primary tumors and 6 with recurrent tumors; 16 of 18 patients who had primary meningiomas had a recurrence and 5 of 6 who had recurrent tumors had another relapse (mean interval between resection and recurrence, 4.4 years and 14 months, respectively). CONCLUSIONS: Postoperative radiation appeared to delay recurrence in subtotally excised and recurrent sphenoid wing meningiomas during the time frame of this study.

Antibody-dependent cell-mediated cytotoxicity against orbital target cells in thyroid-associated ophthalmopathy and related disorders; close relationship between serum cytotoxic antibodies and parameters of eye muscle dysfunction.

We have carried out tests for antibody-dependent cell-mediated cytotoxicity (ADCC) against extra ocular muscle (EOM), Müller's muscle, orbital fibroblasts and skeletal muscle in patients with thyroid-associated ophthalmopathy (TAO) and related eye disorders. Cytotoxicity was measured as lactate dehydrogenase (LDH) release and results expressed as % cytotoxicity. Tests were positive, with EOM cells, in 65% of patients with TAO, 75% with ocular myopathy, a variant of TAO in which periorbital inflammation is minimal, 50% with euthyroid Graves' disease defined as ophthalmopathy associated with subclinical thyroiditis and in 50% of patients with stable lid lag and retraction but no other signs of progressive ophthalmopathy, but in only 13% of patients with Graves' hyperthyroidism without ophthalmopathy, 10% with Hashimoto's thyroiditis and 14% of patients with other thyroid disorders. Tests were positive, with Müller's muscle cells, in 40% of patients with TAO, 25% with ocular myopathy, 40% with euthyroid Graves' disease, 44% with lid lag, 19% with Graves'hyperthyroidism, 50% with Hashimoto's thyroiditis and in 37.5% of patients with other thyroid disorders. When skeletal muscle cells were used as target, tests were positive in 13% of patients with TAO, 31% with lid lag, 25% with Graves' hyperthyroidism and in 29% of patients with Hashimoto's thyroiditis, but in no patient with euthyroid Graves' disease or other thyroid disorders. Tests were negative in all patients and normals tested when EOM-derived fibroblasts were used as targets in ADCC. A significant positive correlation between % cytotoxicity against EOM cells and the severity of the eye muscle dysfunction expressed as an eye muscle index, was observed in patients with TAO. There was a significant negative correlation between the duration of eye disease and % cytotoxicity against EOM cells, suggesting higher titers of cytotoxic antibodies in the early stages of TAO. There was no correlation between % cytotoxicity and serum level of anti-TSH receptor antibodies, measured in a radioreceptor assay. These findings suggest that autoimmunity against Müller's muscle may play a role in the pathogenesis of persistent lid lag and retraction. The nature of the EOM and Müller's muscle autoantigens recognized by cytotoxic antibodies in the serum of patients with TAO and related eye disorders is unknown.

Extreme eyelid swelling as an unusual presentation of dysthyroid orbitopathy.

We describe seven cases of dysthyroid orbitopathy initially seen as severe periocular lid swelling. Imaging techniques typically demonstrate normal extraocular muscles, although moderate muscle enlargement may occur. Patients do not respond to steroids, radiation therapy, antihistamines, or diuretics. We believe this clinical picture represents a unique population of dysthyroid orbitopathy patients who are best managed by long-term observation followed by cosmetic surgery.

Fine needle aspiration diagnosis of orbital plasmacytoma with amyloidosis. A case report.

Fine needle aspiration (FNA) cytology of the orbit was used to diagnose an unusual case of multiple myeloma with secondary amyloid deposition in a 27-year-old man who presented with bilateral keratoconjunctivitis and left-sided ptosis. The FNA smears revealed immature plasma cells, extracellular eosinophilic material (amyloid) and numerous multinucleate giant cells. Subsequent histologic studies of excised tissue confirmed the cytologic diagnosis of plasmacytoma with secondary amyloidosis. Amyloid was further confirmed by electron microscopy. The patient was subsequently diagnosed as having multiple myeloma. The cytologic features of amyloidosis and plasmacytoma are presented. The differential diagnosis and the diagnostic utility of FNA cytology in the evaluation of these orbital lesions are also discussed. This apparently was the first case of orbital amyloidosis diagnosed by FNA cytology.