CHOROIDAL MELANOMA MASQUERADING AS PANUVEITIS IN A PATIENT WITH MULTIPLE MYELOMA Vincent R. Vann, MD, PHD,* Gian P. Giuliari, MD,* Rodolfo M. Banda, MD,* Patricia Che´vez-Barrios, MD,†‡ Gregory N. Fuller, MD, PHD,‡ Dan S. Gombos, MD‡
Background: Choroidal melanoma can sometimes present with associated anterior uveitis or panuveitis that can mask the underlying intraocular neoplasm and confound the diagnosis. Methods: Retrospective chart review. Results: A 58-year-old woman presented with recurrent episodes of hypopyon, severe panuveitis, and a choroidal mass lesion in the right eye. Extensive systemic workup led to a diagnosis of multiple myeloma. Despite successful treatment of myeloma, the choroidal mass persisted and increased in size. Transscleral biopsy of the mass lesion revealed primary malignant choroidal melanoma. Conclusion: This case demonstrates that choroidal melanoma can present as masquerade syndrome with significant intraocular inflammation. Such cases pose a significant diagnostic dilemma to the clinician. Transscleral choroidal biopsy can provide critical information that is essential to establishing a definitive diagnosis and treatment plan. RETINAL CASES & BRIEF REPORTS 3:152–155, 2009
From *Valley Retina Institute, P.A., McAllen, Texas; †the Department of Pathology, The Methodist Hospital, Houston, Texas; and ‡The University of Texas M.D. Anderson Cancer Center, Houston, Texas.
lease cytokines and other inflammatory mediators that can produce associated episcleritis, anterior uveitis, or even panuveitis.3 We report a unique case of primary malignant choroidal melanoma that presented as masquerade syndrome involving recurrent episodes of severe panuveitis in association with multiple myeloma. The severity of the intraocular inflammation and the diagnosis of multiple myeloma were confounding factors that hindered the establishment of a definitive diagnosis in this rather rare and challenging case.
C
horoidal melanoma is the most common primary intraocular neoplasm. The diagnostic features that distinguish malignant choroidal melanomas from benign choroidal nevi have been the subject of much controversy. Fortunately, as a result of the Collaborative Ocular Melanoma Study and other studies, guidelines have been established to facilitate the diagnosis of this malignant disease.1,2 Early choroidal melanoma is typically asymptomatic and is first detected during routine ophthalmic examination. Symptomatic cases that present with blurred vision, visual field defects, or flashes and floaters are likely to be more advanced. In cases of advanced choroidal melanoma, necrotic tissue can re-
Case Report A 58-year-old woman presented in September 2005 with the chief complaint of decreased vision in the right eye over 24 hours. She also reported ongoing neck pain for 8 months that was treated with naproxen. At initial presentation, visual acuity was 20/200 in the right eye and 20/25 in the left eye. There were 4⫹ cells and a 1-mm hypopyon in the anterior chamber of the right eye (Fig. 1A). The view of the posterior pole was obscured due to severe vitritis, but a peripheral confluent area suspicious for full-thickness chorioretinitis was noted. B-Scan ultrasonography showed significant vitritis
The authors have no potential conflicts of interest to disclose. Reprint requests: Gian P. Giuliari, MD, Valley Retina Institute, P.A., 1309 East Ridge Road, Suite 1, McAllen, TX 78503; e-mail: [email protected]
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Fig. 1. A, Photograph of the right eye shows conjunctival injection, abundant cells, and a 1-mm hypopyon in the anterior chamber. B, Transverse B-scan ultrasonogram at 6:00 o’clock with high gain (92 dB) shows severe vitritis. C, Transverse B-scan ultrasonogram at 9:00 o’clock with low gain (61 dB) shows a domeshaped choroidal mass lesion just nasal to the optic disk with low internal reflectivity and dimensions of 3.04 mm in maximum apical thickness and 8.47 mm in basal diameter.
(Fig. 1B) and a dome-shaped choroidal mass lesion in the nasal quadrant with low internal reflectivity and dimensions of 3.0 mm in maximum apical thickness and 8.5 mm in maximum basal diameter (Fig. 1C). Results of ophthalmic examination of the left eye were unremarkable. Due to the acute presentation, an infectious etiology such as endogenous endophthalmitis was suspected. Diagnostic anterior chamber paracentesis was performed to obtain aqueous fluid for analysis. Results of bacterial and fungal cultures were negative, and results of polymerase chain reaction for herpes simplex virus, varicella-zoster virus, and Toxoplasma DNA were also negative. The patient was treated with intravitreal and systemic antibiotics as well as topical corticosteroids. Over several weeks, she had marked improvement in symptoms with an increase in visual acuity to 20/25 in the right eye. As vitritis cleared, an elevated, hyperpigmented, choroidal mass lesion was visualized nasal to the optic disk in the right eye (Fig. 2). There was no associated serous retinal detachment. Although the mass lesion was suspicious for uveal melanoma, the differential diagnosis included choroidal metastasis, infectious
Fig. 2. Fundus photograph of the nasal quadrant of the right eye shows an elevated, hyperpigmented, choroidal mass lesion just nasal to the optic disk.
choroidal abscess, inflammatory choroidal granuloma, and hemorrhagic choroidal detachment. Given the acute presentation with severe intraocular inflammation, the mass was initially thought to be a probable choroidal abscess related to endogenous endophthalmitis. However, results of a systemic workup for an infectious source of endogenous endophthalmitis were negative, including transesophageal echocardiography and ultrasonography of the liver and gallbladder. Over the next 3 months, the patient had two episodes of decreased vision due to severe recurrent panuveitis with hypopyon in the right eye. Each episode resolved with topical corticosteroid treatment and systemic antibiotic therapy. Serial B-scan ultrasonograms showed that the choroidal mass remained stable in size over time. In January 2006, she had a fourth episode of panuveitis with decreased vision in the right eye. Due to the unknown etiology of the recurrent episodes of intraocular inflammation, diagnostic vitrectomy with vitreous biopsy was performed at that time. Cytologic examination with immunohistochemical stains of vitreous fluid showed no signs of infection or malignancy. Only reactive inflammatory cells (histiocytes) and necrotic cells were detected. The patient was then referred to an ocular oncologist for further evaluation of the choroidal mass lesion. Complete oncologic workup was performed, but no primary systemic neoplasm was identified as a possible source of metastasis to the choroid. Abdominal computed tomography showed only nonspecific changes. Magnetic resonance imaging of the brain and orbit showed a 0.8 ⫻ 0.9 ⫻ 0.4-cm lesion in the medial aspect of the right globe 4 mm medial to the optic disk. The lesion was hyperintense with homogeneous enhancement on a T1-weighted image and no intensity on a T2-weighted image. During the oncologic workup, the patient continued to complain of persistent neck pain with new onset of right shoulder pain and calf pain. She was then referred to a rheumatologist for further evaluation. Extensive laboratory testing was performed to rule out any systemic immunologic or hematologic disorders. Testing revealed the following positive results: (elevated) serum IgG level, 4,460 mg/dL (normal range, 600 –1,600 mg/dL); serum M protein level, 4.1 g/dL (IgG ); and Bence Jones proteinuria (0.78 g/d). Bone survey demonstrated osteopenic lucencies of the skull and the lateral aspect of the right eighth rib with fracture and biconcave remodeling in the thoracic and lumbar vertebrae consistent with osteopenia. She underwent bone marrow biopsy that showed 42%
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plasmacytosis positive for CD38, CD138, and light chains. At this point, she was diagnosed with IgG multiple myeloma. Thus, it was presumed that the choroidal mass might represent intraocular involvement by multiple myeloma with infiltration of the choroid by malignant plasma cells. In March 2006, the patient was treated for multiple myeloma with standard-dose chemotherapy (bortezomib, thalidomide, and dexamethasone) with an excellent response. Subsequently, she underwent stem cell transplantation for consolidation. Over several months, both serum M protein and Bence Jones protein levels were reduced to 0, and myeloma went into remission. However, despite successful treatment of multiple myeloma, the choroidal mass persisted and did not resolve. In fact, serial A-scan and B-scan ultrasonograms demonstrated an increase in the size of the choroidal mass. By June 2006, the maximum apical thickness had increased to 5.7 mm, and the maximum basal diameter had increased to ⬇12.6 mm. Because multiple myeloma did not appear to account for the choroidal mass, a definitive tissue diagnosis of the mass lesion was required. Therefore, choroidal biopsy of the intraocular mass was performed via a transscleral approach in June 2006. Only a very small piece of tumor was obtained, but histopathologic analysis of the biopsy material showed that the tumor was composed of
Fig. 3. Histopathologic analysis of a specimen of the choroidal mass lesion in the right eye obtained by transscleral biopsy. A, Hematoxylin– eosin staining shows that the lesion is composed predominantly of spindle A cells with focal spindle B cells (arrows) around vascular loops. B, Immunohistochemical staining shows that the tumor cells stain darkly positive for S100 and pan-melanin.
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spindle A cells with focal clusters of atypical spindle B cells diagnostic for melanoma (Fig. 3A). Focal vascular loops were detected by periodic acid–Schiff staining. Positive immunohistochemical staining with S100 and pan-melanin and negative staining with CD68 and cytokeratin confirmed the diagnosis (Fig. 3B). In July 2006, choroidal melanoma was treated with local radioactive iodine 125 plaque brachytherapy for 5 days. Choroidal melanoma regressed after treatment, and there were no signs of recurrence over 14 months of posttreatment follow-up. At the last ophthalmic examination, visual acuity was counting fingers in the right eye with a 2⫹ posterior subcapsular and dense brunescent cataract. Interestingly, there were no further episodes of uveitis since brachytherapy was applied. Multiple myeloma also remained in complete remission as of the last follow-up visit in September 2007.
Discussion The term “masquerade syndrome” has been used to describe a group of disorders that mimic ocular inflammatory disease, most often due to an underlying malignant neoplasm.4 In many cases, the disease that masquerades is not only vision threatening but also potentially fatal. Hence, failure to recognize and properly diagnose a case of masquerade syndrome can result in significant morbidity and mortality. In the current case, a 58-year-old woman with primary malignant choroidal melanoma presented with recurrent episodes of hypopyon and panuveitis in association with multiple myeloma. To our knowledge, this is the first description of masquerade syndrome involving panuveitis in association with both primary choroidal melanoma and multiple myeloma presenting simultaneously in one patient. This represents an extremely rare and unusual case of masquerade syndrome. Our patient presented with severe intraocular inflammation involving both anterior and posterior segments. It is possible that a nonspecific intraocular inflammatory response related to multiple myeloma could have contributed to panuveitis seen in our patient. However, given the results of vitreous biopsy, inflammation in our case was most likely reactive inflammation due to underlying choroidal melanoma and not due to infiltration by neoplastic plasma cells related to multiple myeloma. Although such presentations are rare, isolated cases of choroidal melanoma in association with significant intraocular inflammation have been reported previously. Various forms of inflammation have been described including episcleritis, anterior uveitis, panuveitis, and panophthalmitis.3,5,6 Multiple myeloma is a plasma cell malignancy often associated with destructive skeletal lesions. Orbital and ocular involvement in multiple myeloma is uncommon. Cysts of the ciliary body and retinal vascular lesions represent the most common ocular findings.7 Intraocular invasion by neoplastic plasma cells is a rare ophthalmic manifestation of multiple myeloma; to
CHOROIDAL MELANOMA MASQUERADING AS PANUVEITIS IN MULTIPLE MYELOMA
our knowledge, involvement of the choroid has never been reported. Shakin et al8 reported a case of documented multiple myeloma that presented with infiltration of the iris simulating nongranulomatous uveitis. Tranos et al9 described a patient with multiple myeloma who had a well-formed solid infiltrate in the anterior chamber that resembled a hypopyon (pseudohypopyon). In contrast to our case, both of these prior investigations found that neoplastic plasma cells were present in the anterior segment as determined by cytologic examination of cells obtained by anterior chamber paracentesis. In summary, masquerade syndromes are entities that emulate inflammatory conditions but that are in fact due to a neoplastic process. Our case demonstrates that choroidal melanoma can present as masquerade syndrome with significant intraocular inflammation. It is important to remember that numerous conditions can give rise to an appearance mimicking an inflammatory disorder that can confound the presentation and lead to an inaccurate diagnosis. When faced with such a diagnostic dilemma, careful history and examination in concert with appropriate ancillary investigations are vital. In our case, performing transscleral choroidal biopsy of the mass lesion proved essential to establishing a definitive diagnosis and an appropriate treatment plan. An ophthalmic surgeon who is skilled and well versed in choroidal biopsy techniques and histopathologic evaluation of tissue specimens by pathologists who are familiar with ophthalmic diseases are required to make the correct diagnosis in these challenging cases.
155
Key words: choroidal melanoma, multiple myeloma, panuveitis, anterior uveitis, uveitis, hypopyon, masquerade syndrome, transscleral biopsy, choroid, uvea, uveal, ocular, intraocular, inflammation, malignancy, neoplasm, tumor. References 1.
2.
3.
4. 5.
6.
7. 8. 9.
The Collaborative Ocular Melanoma Study Group. Factors predictive of growth and treatment of small choroidal melanoma. COMS report no. 5. Arch Ophthalmol 1997;115:1537– 1544. Shields CL, Cater J, Shields JA, et al. Combination of clinical factors predictive of growth of small choroidal melanocytic tumors. Arch Ophthalmol 2000;118:360–364. Fraser DJ Jr, Font RL. Ocular inflammation and hemorrhage as initial manifestations of uveal malignant melanoma. Incidence and prognosis. Arch Ophthalmol 1979;97:1311–1314. Read RW, Zamir E, Rao NA. Neoplastic masquerade syndromes. Surv Ophthalmol 2002;47:81–124. Sood NN, Ratnaraj A. Malignant melanoma of choroid presenting as panophthalmitis. J All India Ophthalmol Soc 1968; 16:67–69. Nzegwu MA, Waziri-Erameh MJ, Ukponmwan CU, Igbe AP. Ocular melanoma presenting as a case of panophthalmitis in a 78-year-old Nigerian woman (masquerade syndrome): an uncommon finding in Benin City, Nigeria. Eur J Cancer Care 2007;16:451–452. Knapp AJ, Gartner S, Henkind P. Multiple myeloma and its ocular manifestations. Surv Ophthalmol 1987;31:343–351. Shakin EP, Augsburger JJ, Eagle RC Jr, et al. Multiple myeloma involving the iris. Arch Ophthalmol 1988;106:524–526. Tranos PG, Andreou PS, Wickremasinghe SS, Brazier JD. Pseudohypopyon as a feature of multiple myeloma. Arch Ophthalmol 2002;120:87–88.
Background: Choroidal melanoma can sometimes present with associated anterior uveitis or panuveitis that can mask the underlying intraocular neoplasm and confound the diagnosis. Methods: Retrospective chart review. Results: A 58-year-old woman presented with recurrent episodes of hypopyon, severe panuveitis, and a choroidal mass lesion in the right eye. Extensive systemic workup led to a diagnosis of multiple myeloma. Despite successful treatment of myeloma, the choroidal mass persisted and increased in size. Transscleral biopsy of the mass lesion revealed primary malignant choroidal melanoma. Conclusion: This case demonstrates that choroidal melanoma can present as masquerade syndrome with significant intraocular inflammation. Such cases pose a significant diagnostic dilemma to the clinician. Transscleral choroidal biopsy can provide critical information that is essential to establishing a definitive diagnosis and treatment plan. RETINAL CASES & BRIEF REPORTS 3:152–155, 2009
From *Valley Retina Institute, P.A., McAllen, Texas; †the Department of Pathology, The Methodist Hospital, Houston, Texas; and ‡The University of Texas M.D. Anderson Cancer Center, Houston, Texas.
lease cytokines and other inflammatory mediators that can produce associated episcleritis, anterior uveitis, or even panuveitis.3 We report a unique case of primary malignant choroidal melanoma that presented as masquerade syndrome involving recurrent episodes of severe panuveitis in association with multiple myeloma. The severity of the intraocular inflammation and the diagnosis of multiple myeloma were confounding factors that hindered the establishment of a definitive diagnosis in this rather rare and challenging case.
C
horoidal melanoma is the most common primary intraocular neoplasm. The diagnostic features that distinguish malignant choroidal melanomas from benign choroidal nevi have been the subject of much controversy. Fortunately, as a result of the Collaborative Ocular Melanoma Study and other studies, guidelines have been established to facilitate the diagnosis of this malignant disease.1,2 Early choroidal melanoma is typically asymptomatic and is first detected during routine ophthalmic examination. Symptomatic cases that present with blurred vision, visual field defects, or flashes and floaters are likely to be more advanced. In cases of advanced choroidal melanoma, necrotic tissue can re-
Case Report A 58-year-old woman presented in September 2005 with the chief complaint of decreased vision in the right eye over 24 hours. She also reported ongoing neck pain for 8 months that was treated with naproxen. At initial presentation, visual acuity was 20/200 in the right eye and 20/25 in the left eye. There were 4⫹ cells and a 1-mm hypopyon in the anterior chamber of the right eye (Fig. 1A). The view of the posterior pole was obscured due to severe vitritis, but a peripheral confluent area suspicious for full-thickness chorioretinitis was noted. B-Scan ultrasonography showed significant vitritis
The authors have no potential conflicts of interest to disclose. Reprint requests: Gian P. Giuliari, MD, Valley Retina Institute, P.A., 1309 East Ridge Road, Suite 1, McAllen, TX 78503; e-mail: [email protected]
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Fig. 1. A, Photograph of the right eye shows conjunctival injection, abundant cells, and a 1-mm hypopyon in the anterior chamber. B, Transverse B-scan ultrasonogram at 6:00 o’clock with high gain (92 dB) shows severe vitritis. C, Transverse B-scan ultrasonogram at 9:00 o’clock with low gain (61 dB) shows a domeshaped choroidal mass lesion just nasal to the optic disk with low internal reflectivity and dimensions of 3.04 mm in maximum apical thickness and 8.47 mm in basal diameter.
(Fig. 1B) and a dome-shaped choroidal mass lesion in the nasal quadrant with low internal reflectivity and dimensions of 3.0 mm in maximum apical thickness and 8.5 mm in maximum basal diameter (Fig. 1C). Results of ophthalmic examination of the left eye were unremarkable. Due to the acute presentation, an infectious etiology such as endogenous endophthalmitis was suspected. Diagnostic anterior chamber paracentesis was performed to obtain aqueous fluid for analysis. Results of bacterial and fungal cultures were negative, and results of polymerase chain reaction for herpes simplex virus, varicella-zoster virus, and Toxoplasma DNA were also negative. The patient was treated with intravitreal and systemic antibiotics as well as topical corticosteroids. Over several weeks, she had marked improvement in symptoms with an increase in visual acuity to 20/25 in the right eye. As vitritis cleared, an elevated, hyperpigmented, choroidal mass lesion was visualized nasal to the optic disk in the right eye (Fig. 2). There was no associated serous retinal detachment. Although the mass lesion was suspicious for uveal melanoma, the differential diagnosis included choroidal metastasis, infectious
Fig. 2. Fundus photograph of the nasal quadrant of the right eye shows an elevated, hyperpigmented, choroidal mass lesion just nasal to the optic disk.
choroidal abscess, inflammatory choroidal granuloma, and hemorrhagic choroidal detachment. Given the acute presentation with severe intraocular inflammation, the mass was initially thought to be a probable choroidal abscess related to endogenous endophthalmitis. However, results of a systemic workup for an infectious source of endogenous endophthalmitis were negative, including transesophageal echocardiography and ultrasonography of the liver and gallbladder. Over the next 3 months, the patient had two episodes of decreased vision due to severe recurrent panuveitis with hypopyon in the right eye. Each episode resolved with topical corticosteroid treatment and systemic antibiotic therapy. Serial B-scan ultrasonograms showed that the choroidal mass remained stable in size over time. In January 2006, she had a fourth episode of panuveitis with decreased vision in the right eye. Due to the unknown etiology of the recurrent episodes of intraocular inflammation, diagnostic vitrectomy with vitreous biopsy was performed at that time. Cytologic examination with immunohistochemical stains of vitreous fluid showed no signs of infection or malignancy. Only reactive inflammatory cells (histiocytes) and necrotic cells were detected. The patient was then referred to an ocular oncologist for further evaluation of the choroidal mass lesion. Complete oncologic workup was performed, but no primary systemic neoplasm was identified as a possible source of metastasis to the choroid. Abdominal computed tomography showed only nonspecific changes. Magnetic resonance imaging of the brain and orbit showed a 0.8 ⫻ 0.9 ⫻ 0.4-cm lesion in the medial aspect of the right globe 4 mm medial to the optic disk. The lesion was hyperintense with homogeneous enhancement on a T1-weighted image and no intensity on a T2-weighted image. During the oncologic workup, the patient continued to complain of persistent neck pain with new onset of right shoulder pain and calf pain. She was then referred to a rheumatologist for further evaluation. Extensive laboratory testing was performed to rule out any systemic immunologic or hematologic disorders. Testing revealed the following positive results: (elevated) serum IgG level, 4,460 mg/dL (normal range, 600 –1,600 mg/dL); serum M protein level, 4.1 g/dL (IgG ); and Bence Jones proteinuria (0.78 g/d). Bone survey demonstrated osteopenic lucencies of the skull and the lateral aspect of the right eighth rib with fracture and biconcave remodeling in the thoracic and lumbar vertebrae consistent with osteopenia. She underwent bone marrow biopsy that showed 42%
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plasmacytosis positive for CD38, CD138, and light chains. At this point, she was diagnosed with IgG multiple myeloma. Thus, it was presumed that the choroidal mass might represent intraocular involvement by multiple myeloma with infiltration of the choroid by malignant plasma cells. In March 2006, the patient was treated for multiple myeloma with standard-dose chemotherapy (bortezomib, thalidomide, and dexamethasone) with an excellent response. Subsequently, she underwent stem cell transplantation for consolidation. Over several months, both serum M protein and Bence Jones protein levels were reduced to 0, and myeloma went into remission. However, despite successful treatment of multiple myeloma, the choroidal mass persisted and did not resolve. In fact, serial A-scan and B-scan ultrasonograms demonstrated an increase in the size of the choroidal mass. By June 2006, the maximum apical thickness had increased to 5.7 mm, and the maximum basal diameter had increased to ⬇12.6 mm. Because multiple myeloma did not appear to account for the choroidal mass, a definitive tissue diagnosis of the mass lesion was required. Therefore, choroidal biopsy of the intraocular mass was performed via a transscleral approach in June 2006. Only a very small piece of tumor was obtained, but histopathologic analysis of the biopsy material showed that the tumor was composed of
Fig. 3. Histopathologic analysis of a specimen of the choroidal mass lesion in the right eye obtained by transscleral biopsy. A, Hematoxylin– eosin staining shows that the lesion is composed predominantly of spindle A cells with focal spindle B cells (arrows) around vascular loops. B, Immunohistochemical staining shows that the tumor cells stain darkly positive for S100 and pan-melanin.
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spindle A cells with focal clusters of atypical spindle B cells diagnostic for melanoma (Fig. 3A). Focal vascular loops were detected by periodic acid–Schiff staining. Positive immunohistochemical staining with S100 and pan-melanin and negative staining with CD68 and cytokeratin confirmed the diagnosis (Fig. 3B). In July 2006, choroidal melanoma was treated with local radioactive iodine 125 plaque brachytherapy for 5 days. Choroidal melanoma regressed after treatment, and there were no signs of recurrence over 14 months of posttreatment follow-up. At the last ophthalmic examination, visual acuity was counting fingers in the right eye with a 2⫹ posterior subcapsular and dense brunescent cataract. Interestingly, there were no further episodes of uveitis since brachytherapy was applied. Multiple myeloma also remained in complete remission as of the last follow-up visit in September 2007.
Discussion The term “masquerade syndrome” has been used to describe a group of disorders that mimic ocular inflammatory disease, most often due to an underlying malignant neoplasm.4 In many cases, the disease that masquerades is not only vision threatening but also potentially fatal. Hence, failure to recognize and properly diagnose a case of masquerade syndrome can result in significant morbidity and mortality. In the current case, a 58-year-old woman with primary malignant choroidal melanoma presented with recurrent episodes of hypopyon and panuveitis in association with multiple myeloma. To our knowledge, this is the first description of masquerade syndrome involving panuveitis in association with both primary choroidal melanoma and multiple myeloma presenting simultaneously in one patient. This represents an extremely rare and unusual case of masquerade syndrome. Our patient presented with severe intraocular inflammation involving both anterior and posterior segments. It is possible that a nonspecific intraocular inflammatory response related to multiple myeloma could have contributed to panuveitis seen in our patient. However, given the results of vitreous biopsy, inflammation in our case was most likely reactive inflammation due to underlying choroidal melanoma and not due to infiltration by neoplastic plasma cells related to multiple myeloma. Although such presentations are rare, isolated cases of choroidal melanoma in association with significant intraocular inflammation have been reported previously. Various forms of inflammation have been described including episcleritis, anterior uveitis, panuveitis, and panophthalmitis.3,5,6 Multiple myeloma is a plasma cell malignancy often associated with destructive skeletal lesions. Orbital and ocular involvement in multiple myeloma is uncommon. Cysts of the ciliary body and retinal vascular lesions represent the most common ocular findings.7 Intraocular invasion by neoplastic plasma cells is a rare ophthalmic manifestation of multiple myeloma; to
CHOROIDAL MELANOMA MASQUERADING AS PANUVEITIS IN MULTIPLE MYELOMA
our knowledge, involvement of the choroid has never been reported. Shakin et al8 reported a case of documented multiple myeloma that presented with infiltration of the iris simulating nongranulomatous uveitis. Tranos et al9 described a patient with multiple myeloma who had a well-formed solid infiltrate in the anterior chamber that resembled a hypopyon (pseudohypopyon). In contrast to our case, both of these prior investigations found that neoplastic plasma cells were present in the anterior segment as determined by cytologic examination of cells obtained by anterior chamber paracentesis. In summary, masquerade syndromes are entities that emulate inflammatory conditions but that are in fact due to a neoplastic process. Our case demonstrates that choroidal melanoma can present as masquerade syndrome with significant intraocular inflammation. It is important to remember that numerous conditions can give rise to an appearance mimicking an inflammatory disorder that can confound the presentation and lead to an inaccurate diagnosis. When faced with such a diagnostic dilemma, careful history and examination in concert with appropriate ancillary investigations are vital. In our case, performing transscleral choroidal biopsy of the mass lesion proved essential to establishing a definitive diagnosis and an appropriate treatment plan. An ophthalmic surgeon who is skilled and well versed in choroidal biopsy techniques and histopathologic evaluation of tissue specimens by pathologists who are familiar with ophthalmic diseases are required to make the correct diagnosis in these challenging cases.
155
Key words: choroidal melanoma, multiple myeloma, panuveitis, anterior uveitis, uveitis, hypopyon, masquerade syndrome, transscleral biopsy, choroid, uvea, uveal, ocular, intraocular, inflammation, malignancy, neoplasm, tumor. References 1.
2.
3.
4. 5.
6.
7. 8. 9.
The Collaborative Ocular Melanoma Study Group. Factors predictive of growth and treatment of small choroidal melanoma. COMS report no. 5. Arch Ophthalmol 1997;115:1537– 1544. Shields CL, Cater J, Shields JA, et al. Combination of clinical factors predictive of growth of small choroidal melanocytic tumors. Arch Ophthalmol 2000;118:360–364. Fraser DJ Jr, Font RL. Ocular inflammation and hemorrhage as initial manifestations of uveal malignant melanoma. Incidence and prognosis. Arch Ophthalmol 1979;97:1311–1314. Read RW, Zamir E, Rao NA. Neoplastic masquerade syndromes. Surv Ophthalmol 2002;47:81–124. Sood NN, Ratnaraj A. Malignant melanoma of choroid presenting as panophthalmitis. J All India Ophthalmol Soc 1968; 16:67–69. Nzegwu MA, Waziri-Erameh MJ, Ukponmwan CU, Igbe AP. Ocular melanoma presenting as a case of panophthalmitis in a 78-year-old Nigerian woman (masquerade syndrome): an uncommon finding in Benin City, Nigeria. Eur J Cancer Care 2007;16:451–452. Knapp AJ, Gartner S, Henkind P. Multiple myeloma and its ocular manifestations. Surv Ophthalmol 1987;31:343–351. Shakin EP, Augsburger JJ, Eagle RC Jr, et al. Multiple myeloma involving the iris. Arch Ophthalmol 1988;106:524–526. Tranos PG, Andreou PS, Wickremasinghe SS, Brazier JD. Pseudohypopyon as a feature of multiple myeloma. Arch Ophthalmol 2002;120:87–88.
