International Ophthalmology 16: 39-44, 1992. 9 1992 Kluwer Academic Publishers. Printed in the Netherlands.
Unusual MRI findings in metastatic carcinoma to the choroid and optic nerve: a case report Patrick De Potter, 1 Jerry A. Shields, 1 Carol L. Shields, 1 Lawrence A. Yannuzzi, 2 Yale E. Fisher 2 & Vijay M. Rao 3
l Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, USA; 2Manhattan Eye, Ear and Throat Hospital, New York, New York, USA, and 3Department of Radiology, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, USA Accepted 30 July 1991
Key words: choroidal melanoma, choroidal metastasis, magnetic resonance imaging, metastatic adenocarcinoma Abstract A 51 year old man with biopsy proven pulmonary sarcoidosis and skin test positive for tuberculosis presented with features of an amelanotic flat choroidal mass suggestive of choroiditis. The mass enlarged despite corticosteroids and anti-tuberculous medications. A throrough systemic evaluation for possible primary tumor metastatic to the choroid was negative. Further clinical evaluation and magnetic resonance imaging suggested a diffuse primary choroidal malignant melanoma with optic nerve invasion. The eye was enucleated and the mass proved histopathologically to be a mucin secreting adenocarcinoma of unknown origin despite a repeat systemic work-up. The patient died three months after the onset of symptoms and three weeks after enucleation with diffuse metastases from an unknown primary cancer. Magnetic resonance imaging (MRI) is usually helpful in the differentiation of uveal melanoma from uveal metastasis. In this case, however, it suggested the diagnosis of a diffuse choroidal melanoma. The reason for the atypical MRI findings will be discussed.
Introduction It is believed that carcinoma metastatic to the uvea is more common than primary uveal melanoma [1-6]. The prevalence of metastases to the ocular region in patients with known metastatic cancer ranges from 0.07% (using routine ophthalmoscopy in patients with known systemic carcinoma) [7] to 12% (on post-mortem pathologic examination) [8]. The posterior choroid is the most common site of intraocular metastasis [1-4]. The ratio of isolated metastasis to the uvea compared with the orbit ranges from 6:1 to 9:1 in adults [3, 4, 9, 10]. Although most metastatic tumors have a rather typ-
ical appearance, occasionally they may be clinically confused with inflammatory diseases or other ocular tumors such as amelanotic uveal nevus, amelanotic uveal malignant melanoma and uveal lymphoma [4, 11]. We report the clinical, magnetic resonance imaging (MRI) and histopathologic findings in a patient with carcinoma metastatic to the choroid and the optic nerve that was originally diagnosed as sarcoidosis based on clinical findings and a positive lung biopsy of sarcoidosis.
40
P. D e Potter et al.
Fig. 1. Fundus photograph showing the bullous retinal detachment overlying a diffuse amelanotic choroidal mass with variable retinal pigment epithelium changes. Note the flame-shaped hemorrhages on the optic disc and tortuous retinal vessels.
Report of a case
A 51 year old white man, who had been a heavy cigarette smoker for 30 years, noted progressive visual decrease and pain in his left eye for three weeks. He was found to have a diffuse yellow tumefaction of the choroid in the posterior pole of his left eye with an overlying sensory retinal detachment, suggestive of choroiditis. Because of a history of sarcoidosis diagnosed by transbronchial biopsy five years earlier, a trial dose of corticosteroids was initiated for a presumed diagnosis of sarcoid choroiditis. This relieved the pain and redness but the choroidal mass continued to enlarge with expansion of the retinal detachment. He was subsequently found to test positive for tuberculosis so he was begun on anti-tuberculous triple therapy (Isoniazid, Myambutol, Rimactane) with a tapering dose of corticosteroids. There was no improvement. The possibility of metastatic disease was then considered. Diagnostic studies including complete blood count, serum glutamic oxalacetic transaminase (SGOT), serum glutamic pyruvic transaminase (SGPT), lactic deshydrogenase (LDH), alkaline phosphatase, carcinoembryonic antigen (ACE), glucose, computed tomography (CT) of the brain, chest and abdomen, colonoscopy, intravenous pyelogram, cystoscopy, dermat-
Fig. 2. B-scan ultrasonogram demonstrating the diffuse solid thickening of the juxtapapillary choroid and the overlying retinal detachment.
ologic evaluation revealed normal findings. The patient was referred to the Ocular Oncology Service, at Wills Eye Hospital for diagnosis and management. On our examination, two months after the onset of symptoms, the patient was lethargic and visual acuity was 6/6 (20/20) in the right eye (OD) and no light perception in the left eye (OS). The right eye was unremarkable. The left eye demonstrated epibulbar injection, trace flare and cells in the anterior chamber with no keratic precipitates. Ophthalmoscopy disclosed a subtotal bullous retinal detachment over and around a diffuse amelanotic choroidal mass with overlying retinal pigment epithelium changes. There were flame-shaped hemorrhages on the optic disc and tortuous retinal vessels (Fig. 1). Ultrasound revealed a diffuse solid thickening of the juxtapapillary choroid measuring 3.6 to 6.0 mm in thickness and showing medium internal reflectivity (Fig. 2). Magnetic resonance imaging (MRI) demonstrated a bright intensity signal located in the posterior wall of the left globe on Tl-weighted images with extension into the optic nerve, suspicious for tumor involvement. The exudative retinal detachment appeared less hyperintense with respect to the diffuse choroidal mass but more hyperintense with respect to the vitreous (Fig. 3). The T2-weighted images demonstrated a dark signal at the same location contrasting with the hyperintensity of the
Metastatic carcinoma
41
Fig. 3. Axial magnetic resonance imaging Tl-weighted image
Fig. 4. Axial magnetic resonance imaging T2-weighted image
(1.5 Tesla, with surface coil, T R = 600msec, TE = 20msec) showing a high intensity signal (hyperintense) located at the juxtapapillary choroidal level in the posterior pole and the proximal optic nerve of the left eye (V V) with respect to the overlying retinal detachment (c~> I~>) and the vitreous.
(1.5 Tesla, with surface coil, TR = 2400 msec, TE = 80 msec) showing a dark intensity signal (hypointensity) of the choroidal tumor (IV T ) with respect to the hyperintensity signal of the exudative retinal detachment ( ~ ~) and the vitreous. Due to the exudative nature of the retinal detachment, it appears more hyperintense than the vitreous.
overlying retinal detachment and the vitreous (Fig.
carcinoma metastatic to choroid, optic nerve, juxtapapillary retina. Within three weeks after enucleation, the patient rapidly declined, developed diffuse metastatic involvement of bones and liver and died. A primary site was not established despite repeat head, lung CT scans, biopsy of the stomach, lower endoscopy, testicular examination and urologic evaluation. Chest radiography demonstrated a right pleural effusion that possibly indicated a pulmonary primary site. Permission for autopsy was not granted.
4). In a view of a blind painful eye, a negative systemic evaluation for primary neoplasm, and the possibility of a diffuse amelanotic choroidal melanoma with secondary invasion of the optic nerve, it was elected to enucleate the eye. This was done without complications. Gross examination of the cut section of the globe revealed the choroid and the overlying sclera to be diffusely thickened by an infiltrative, yellow brown mass. A plaque of tumor encased in dense fibrous tissue adhered to the posterior surface of the globe. The neurosensory retina was detached (Fig. 5). Microscopically, the choroid was massively thickened by a partially necrotic infiltrate of mucin secreting adenocarcinoma (Fig. 6). The retinal pigmented epithelium showed disruption with pigment dispersion. Tumor infiltrated the optic nerve, the lamina cribrosa and pial septae posterior to the lamina but did not reach the line of surgical resection (Fig. 7). The alcian blue stain revealed pools of hyaluronidase-resistant extracellular mucin and multiple intracellular mucin vacuoles. Tumor cells also infiltrated swollen axonal fibers and emissarial channels in the sclera. The final diagnosis was mucin secreting adeno-
Comment
Intraocular metastases most commonly originate from primary sites of breast and lung in women and gastrointestinal tract in men [1-4]. Other less common primary sites include cutaneous malignant melanoma, pancreatic carcinoma, renal cell carcinoma and thyroid cancer [1, 3, 12]. In 18 to 25% of patients, the location of the primary tumor is never established [2, 3]. The choroid is the most common site of carcinoma metastatic to the eye. About 40% of the metastatic lesions to the choroid are partially located between the temporal retinal vascular arcades [2,
42
P. De Potter et al.
Fig. 6. High-power photomicrograph showing cords of mucin secreting adenocarcinoma cells infiltrating the choroid. Note pools of extracellular mucin. Hematoxylin and eosin, • 225.
Fig. 5. Horizontal cross section of the enucleated left eye showing diffuse thickening of the choroid and the sclera by a variably pigmented tumor. Note a plaque of tumor encased in dense fibrous tissue adherent to the juxtapapillary external sclera. The sensory retina is detached.
3]. Uveal metastases are typically yellow, placoid or dome-shaped lesions and are associated with a non-rhegmatogenous retinal detachment in 72% of the eyes [9]. Metastatic carcinoma to the optic disc is quite rare [3, 4]. In these cases, there is usually an extension of the juxtapapillary choroidal metastasis into the optic disc [4]. The clinical appearance of a blind painful red eye with external signs of inflammation and a solitary slightly elevated mass of the choroid initially suggested the diagnosis of choroiditis to the ophthalmologist who first evaluated the patient. The history of a positive pulmonary biopsy for sarcoidosis and a positive tuberculin test further suggested the diagnosis. When the lesion failed to respond to corticosteroids and anti-tuberculosis therapy, the diagnosis of metastatic carcinoma was considered but a thorough systemic evaluation revealed no primary tumor. Further clinical features of irregular diffuse choroidal mass surrounding the optic disc with associated subtotal exudative retinal detachment and pigment mottling suggested the diagnosis of primary diffuse choroidal malignant melanoma. Moreover, the MRI findings suggested the diagnosis of melanoma.
Choroidal malignant melanomas have characteristic MRI features due to the nature of melanin as a free-radical containing species that produces paramagnetic proton relaxation enhancement. T1 values for choroidal melanomas are then shorter than those of other intraocular tumors and appear hyperintense on Tl-weighted images and hypointense on T2-weighted images with respect to the vitreous and the optic nerve. Amelanotic tumors appear hyperintense with respect to the vitreous but isointense with respect to the optic nerve on Tl-weighted images [14]. In contrast, intraocular metastasic carcinoma
Fig. 7. High-power photomicrograph showing infiltration of the lamina cribrosa and pial septae anterior to the lamina by mucin secreting adenocarcinoma cells. Hematoxylin and eosin, • 225.
Metastatic carcinoma
generally shows varying T1 and T2 values which some authors speculated might be due to amount of associated hemorrhage or exudate [15]. In a report of a patient with choroidal metastasis from breast carcinoma, the lesion was seen as an ill defined area of hyperintensity on Tl-weighted images [16]. Unlike melanoma, the breast metastasis remained hyperintense in T2-weighted images. The presence of exudative retinal detachment appears also hyperintense on T1 and T2 scans making the identification of the tumor difficult [16]. In our case, we expected the amelanotic tumor to appear less hyperintense on Tl-weighted images due to the absence of the melanin free radicals. However, the lesion was hyperintense on T1weighted images with respect to the vitreous and the optic nerve and showed a hypointense signal in T2-weighted images suggesting the diagnosis of choroidal melanoma. These findings in our case of choroidal metastasis are unusual and may be explained by the extensive retinal pigment epithelium clumps noted clinically and histopathologically; the melanin within the retinal pigment epithelium cells could have contributed some paramagnetic effect to the proton relaxation, thus suggestive of melanoma. The absence of a choroidal hyperintense signal on T2 scans usually seen in metastatic lesions also suggested the clinical diagnosis of primary choroidal melanoma. Therapy for metastatic cancer to the eye and its adnexa is to palliative. Chemotherapy and/or radiotherapy in symptomatic patients appear to bring about regression of intraocular metastases [1, 2, 4]. In a symptomatic patient with metastatic cancer to the uvea or orbit unresponsive to chemotherapy, external beam radiation therapy is delivered in a tumor dose of 3000 to 4000 cGy over a 3- to 5-week period depending on the location and size of the lesion [4, 10, 17]. There is generally no particular reason to enucleate an eye known to contain a metastatic carcinoma unless there is intractable pain or the clinical presentation suggests the diagnosis of choroidal malignant melanoma. The long term prognosis of uveal metastases is poor. Various authors reported an overall median survival rate of 7.4 to 10.5 months from the time of ocular diagnosis in patients with metastatic carci-
43
noma to the eye and the orbit and this depends on the site of the primary malignancy [1-3, 13]. Our patient had an unusually rapid course and rapid death.
Acknowledgement Supported in part by a grant from the Macula Foundation, New York, New York, in part by the Robert Beckman Grant Endowment, Philadelphia, Pennsylvania and in part by the Eye Tumor Research Foundation Inc., Gladwyne, Pennsylvania. This paper was presented at the 1990 Theobald Society Meeting, Philadelphia, PA, May 11, 1990.
References 1. Shields JA, Shakin EP, Shields CL. Metastatic malignant tumors. In: Gold DH, Weingiest TA, editors. The eye in systemic diseases. Philadelphia: Lippincott, 1990; 299-303. 2. Shakin EP, Shields JA. The eye and ocular adnexa in systemic malignancy. In: Duana TD, editor. Duane's clinical ophthalmology. Philadelphia: Lippincott, 1989; 5: 34. 3. Ferry AP, Font RL. Carcinoma metastatic to the eye and orbit. A clinicopathologic study of 227 cases. Arch Ophthalmol 1974; 92: 276-86. 4. Shields JA, Shields CL. Intraocular tumors. A text and atlas. Philadelphia: WB Saunders co, 1992. In press. 5. Nelson CC, Hertzberg BS, Klintworth GK. A histopathologic study of 716 unselected eyes in patients with cancer at the time of death. Am J Ophthalmol 1983; 95: 728-33. 6. BullockJD, Yanes B. Ophthalmic manifestations ofmetastatic breast cancer. Ophthalmology 1980; 87: 961-72. 7. Godtfredsen E. On the frequency of secondary carcinoma in the choroid. Acta Ophthalmol 1944; 22: 394-400. 8. Bloch RS, Gartner S. The incidence of ocular metastatic carcinoma. Arch Ophthalmol 1971; 85: 673-75. 9. Stephens RF, Shields JA. Diagnosis and management of cancer metastatic to the uvea: a study of 70 cases. Ophthalmology 1979; 86: 1336-49. 10. Shields CL, Shields JA, Peggs M. Metastatic tumors to the orbit. Ophthal Reconstr Plast Surg 1988; 4: 73-80. 11. Font RL, Spaulding AG, Zimmerman LE. Diffuse malignant melanoma of the uveal tract: a clinicopathologic report of 54 cases. Trans Am Acad Ophthalmol and Otolaryngol 1968; 72: 87%95. 12. Kindermann WR, Shields JA, Eiferman RA et al. Metastatic renal cell carcinoma to the eye and adnexae. Ophthalmology 1981; 88: 1347-50. 13. Freedman MI, Folk JC. Metastatic tumors to the eye and
44
P. De Potter et al.
orbit: patient survival and clinical characteristics. Arch Ophthalmol 1987; 107: 1215-19. 14. Kolodny NH, Gragoudas ES, D'Amico DJ et al. Proton and sodium-23 magnetic resonance imaging of human ocular tissues: A model study. Arch Ophthalmol 1987; 105: 1532-36. 15. Haik BG, Saint Louis L, Smith ME et al. Magnetic resonance imaging in choroidal tumors. Ann Ophthalmo11987; 19: 218-38. 16. Peyman GA, Mafee MF. Uveal melanomas and simulating lesions: the role of magnetic resonance imaging and com-
puted tomography. Radiol Clin North Am 1987; 25: 47186. 17. Shields JA. Metastatic cancer to the orbit. In: Diagnosis and Management of Orbital Tumors. Philadelphia: WB Saunders, 1989: 291-315.
Address for offprints: Jerry A. Shields, Oncology Service, Wills Eye Hospital, Ninth and Walnut Streets, Philadelphia, PA 19107, USA
Unusual MRI findings in metastatic carcinoma to the choroid and optic nerve: a case report Patrick De Potter, 1 Jerry A. Shields, 1 Carol L. Shields, 1 Lawrence A. Yannuzzi, 2 Yale E. Fisher 2 & Vijay M. Rao 3
l Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, USA; 2Manhattan Eye, Ear and Throat Hospital, New York, New York, USA, and 3Department of Radiology, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, USA Accepted 30 July 1991
Key words: choroidal melanoma, choroidal metastasis, magnetic resonance imaging, metastatic adenocarcinoma Abstract A 51 year old man with biopsy proven pulmonary sarcoidosis and skin test positive for tuberculosis presented with features of an amelanotic flat choroidal mass suggestive of choroiditis. The mass enlarged despite corticosteroids and anti-tuberculous medications. A throrough systemic evaluation for possible primary tumor metastatic to the choroid was negative. Further clinical evaluation and magnetic resonance imaging suggested a diffuse primary choroidal malignant melanoma with optic nerve invasion. The eye was enucleated and the mass proved histopathologically to be a mucin secreting adenocarcinoma of unknown origin despite a repeat systemic work-up. The patient died three months after the onset of symptoms and three weeks after enucleation with diffuse metastases from an unknown primary cancer. Magnetic resonance imaging (MRI) is usually helpful in the differentiation of uveal melanoma from uveal metastasis. In this case, however, it suggested the diagnosis of a diffuse choroidal melanoma. The reason for the atypical MRI findings will be discussed.
Introduction It is believed that carcinoma metastatic to the uvea is more common than primary uveal melanoma [1-6]. The prevalence of metastases to the ocular region in patients with known metastatic cancer ranges from 0.07% (using routine ophthalmoscopy in patients with known systemic carcinoma) [7] to 12% (on post-mortem pathologic examination) [8]. The posterior choroid is the most common site of intraocular metastasis [1-4]. The ratio of isolated metastasis to the uvea compared with the orbit ranges from 6:1 to 9:1 in adults [3, 4, 9, 10]. Although most metastatic tumors have a rather typ-
ical appearance, occasionally they may be clinically confused with inflammatory diseases or other ocular tumors such as amelanotic uveal nevus, amelanotic uveal malignant melanoma and uveal lymphoma [4, 11]. We report the clinical, magnetic resonance imaging (MRI) and histopathologic findings in a patient with carcinoma metastatic to the choroid and the optic nerve that was originally diagnosed as sarcoidosis based on clinical findings and a positive lung biopsy of sarcoidosis.
40
P. D e Potter et al.
Fig. 1. Fundus photograph showing the bullous retinal detachment overlying a diffuse amelanotic choroidal mass with variable retinal pigment epithelium changes. Note the flame-shaped hemorrhages on the optic disc and tortuous retinal vessels.
Report of a case
A 51 year old white man, who had been a heavy cigarette smoker for 30 years, noted progressive visual decrease and pain in his left eye for three weeks. He was found to have a diffuse yellow tumefaction of the choroid in the posterior pole of his left eye with an overlying sensory retinal detachment, suggestive of choroiditis. Because of a history of sarcoidosis diagnosed by transbronchial biopsy five years earlier, a trial dose of corticosteroids was initiated for a presumed diagnosis of sarcoid choroiditis. This relieved the pain and redness but the choroidal mass continued to enlarge with expansion of the retinal detachment. He was subsequently found to test positive for tuberculosis so he was begun on anti-tuberculous triple therapy (Isoniazid, Myambutol, Rimactane) with a tapering dose of corticosteroids. There was no improvement. The possibility of metastatic disease was then considered. Diagnostic studies including complete blood count, serum glutamic oxalacetic transaminase (SGOT), serum glutamic pyruvic transaminase (SGPT), lactic deshydrogenase (LDH), alkaline phosphatase, carcinoembryonic antigen (ACE), glucose, computed tomography (CT) of the brain, chest and abdomen, colonoscopy, intravenous pyelogram, cystoscopy, dermat-
Fig. 2. B-scan ultrasonogram demonstrating the diffuse solid thickening of the juxtapapillary choroid and the overlying retinal detachment.
ologic evaluation revealed normal findings. The patient was referred to the Ocular Oncology Service, at Wills Eye Hospital for diagnosis and management. On our examination, two months after the onset of symptoms, the patient was lethargic and visual acuity was 6/6 (20/20) in the right eye (OD) and no light perception in the left eye (OS). The right eye was unremarkable. The left eye demonstrated epibulbar injection, trace flare and cells in the anterior chamber with no keratic precipitates. Ophthalmoscopy disclosed a subtotal bullous retinal detachment over and around a diffuse amelanotic choroidal mass with overlying retinal pigment epithelium changes. There were flame-shaped hemorrhages on the optic disc and tortuous retinal vessels (Fig. 1). Ultrasound revealed a diffuse solid thickening of the juxtapapillary choroid measuring 3.6 to 6.0 mm in thickness and showing medium internal reflectivity (Fig. 2). Magnetic resonance imaging (MRI) demonstrated a bright intensity signal located in the posterior wall of the left globe on Tl-weighted images with extension into the optic nerve, suspicious for tumor involvement. The exudative retinal detachment appeared less hyperintense with respect to the diffuse choroidal mass but more hyperintense with respect to the vitreous (Fig. 3). The T2-weighted images demonstrated a dark signal at the same location contrasting with the hyperintensity of the
Metastatic carcinoma
41
Fig. 3. Axial magnetic resonance imaging Tl-weighted image
Fig. 4. Axial magnetic resonance imaging T2-weighted image
(1.5 Tesla, with surface coil, T R = 600msec, TE = 20msec) showing a high intensity signal (hyperintense) located at the juxtapapillary choroidal level in the posterior pole and the proximal optic nerve of the left eye (V V) with respect to the overlying retinal detachment (c~> I~>) and the vitreous.
(1.5 Tesla, with surface coil, TR = 2400 msec, TE = 80 msec) showing a dark intensity signal (hypointensity) of the choroidal tumor (IV T ) with respect to the hyperintensity signal of the exudative retinal detachment ( ~ ~) and the vitreous. Due to the exudative nature of the retinal detachment, it appears more hyperintense than the vitreous.
overlying retinal detachment and the vitreous (Fig.
carcinoma metastatic to choroid, optic nerve, juxtapapillary retina. Within three weeks after enucleation, the patient rapidly declined, developed diffuse metastatic involvement of bones and liver and died. A primary site was not established despite repeat head, lung CT scans, biopsy of the stomach, lower endoscopy, testicular examination and urologic evaluation. Chest radiography demonstrated a right pleural effusion that possibly indicated a pulmonary primary site. Permission for autopsy was not granted.
4). In a view of a blind painful eye, a negative systemic evaluation for primary neoplasm, and the possibility of a diffuse amelanotic choroidal melanoma with secondary invasion of the optic nerve, it was elected to enucleate the eye. This was done without complications. Gross examination of the cut section of the globe revealed the choroid and the overlying sclera to be diffusely thickened by an infiltrative, yellow brown mass. A plaque of tumor encased in dense fibrous tissue adhered to the posterior surface of the globe. The neurosensory retina was detached (Fig. 5). Microscopically, the choroid was massively thickened by a partially necrotic infiltrate of mucin secreting adenocarcinoma (Fig. 6). The retinal pigmented epithelium showed disruption with pigment dispersion. Tumor infiltrated the optic nerve, the lamina cribrosa and pial septae posterior to the lamina but did not reach the line of surgical resection (Fig. 7). The alcian blue stain revealed pools of hyaluronidase-resistant extracellular mucin and multiple intracellular mucin vacuoles. Tumor cells also infiltrated swollen axonal fibers and emissarial channels in the sclera. The final diagnosis was mucin secreting adeno-
Comment
Intraocular metastases most commonly originate from primary sites of breast and lung in women and gastrointestinal tract in men [1-4]. Other less common primary sites include cutaneous malignant melanoma, pancreatic carcinoma, renal cell carcinoma and thyroid cancer [1, 3, 12]. In 18 to 25% of patients, the location of the primary tumor is never established [2, 3]. The choroid is the most common site of carcinoma metastatic to the eye. About 40% of the metastatic lesions to the choroid are partially located between the temporal retinal vascular arcades [2,
42
P. De Potter et al.
Fig. 6. High-power photomicrograph showing cords of mucin secreting adenocarcinoma cells infiltrating the choroid. Note pools of extracellular mucin. Hematoxylin and eosin, • 225.
Fig. 5. Horizontal cross section of the enucleated left eye showing diffuse thickening of the choroid and the sclera by a variably pigmented tumor. Note a plaque of tumor encased in dense fibrous tissue adherent to the juxtapapillary external sclera. The sensory retina is detached.
3]. Uveal metastases are typically yellow, placoid or dome-shaped lesions and are associated with a non-rhegmatogenous retinal detachment in 72% of the eyes [9]. Metastatic carcinoma to the optic disc is quite rare [3, 4]. In these cases, there is usually an extension of the juxtapapillary choroidal metastasis into the optic disc [4]. The clinical appearance of a blind painful red eye with external signs of inflammation and a solitary slightly elevated mass of the choroid initially suggested the diagnosis of choroiditis to the ophthalmologist who first evaluated the patient. The history of a positive pulmonary biopsy for sarcoidosis and a positive tuberculin test further suggested the diagnosis. When the lesion failed to respond to corticosteroids and anti-tuberculosis therapy, the diagnosis of metastatic carcinoma was considered but a thorough systemic evaluation revealed no primary tumor. Further clinical features of irregular diffuse choroidal mass surrounding the optic disc with associated subtotal exudative retinal detachment and pigment mottling suggested the diagnosis of primary diffuse choroidal malignant melanoma. Moreover, the MRI findings suggested the diagnosis of melanoma.
Choroidal malignant melanomas have characteristic MRI features due to the nature of melanin as a free-radical containing species that produces paramagnetic proton relaxation enhancement. T1 values for choroidal melanomas are then shorter than those of other intraocular tumors and appear hyperintense on Tl-weighted images and hypointense on T2-weighted images with respect to the vitreous and the optic nerve. Amelanotic tumors appear hyperintense with respect to the vitreous but isointense with respect to the optic nerve on Tl-weighted images [14]. In contrast, intraocular metastasic carcinoma
Fig. 7. High-power photomicrograph showing infiltration of the lamina cribrosa and pial septae anterior to the lamina by mucin secreting adenocarcinoma cells. Hematoxylin and eosin, • 225.
Metastatic carcinoma
generally shows varying T1 and T2 values which some authors speculated might be due to amount of associated hemorrhage or exudate [15]. In a report of a patient with choroidal metastasis from breast carcinoma, the lesion was seen as an ill defined area of hyperintensity on Tl-weighted images [16]. Unlike melanoma, the breast metastasis remained hyperintense in T2-weighted images. The presence of exudative retinal detachment appears also hyperintense on T1 and T2 scans making the identification of the tumor difficult [16]. In our case, we expected the amelanotic tumor to appear less hyperintense on Tl-weighted images due to the absence of the melanin free radicals. However, the lesion was hyperintense on T1weighted images with respect to the vitreous and the optic nerve and showed a hypointense signal in T2-weighted images suggesting the diagnosis of choroidal melanoma. These findings in our case of choroidal metastasis are unusual and may be explained by the extensive retinal pigment epithelium clumps noted clinically and histopathologically; the melanin within the retinal pigment epithelium cells could have contributed some paramagnetic effect to the proton relaxation, thus suggestive of melanoma. The absence of a choroidal hyperintense signal on T2 scans usually seen in metastatic lesions also suggested the clinical diagnosis of primary choroidal melanoma. Therapy for metastatic cancer to the eye and its adnexa is to palliative. Chemotherapy and/or radiotherapy in symptomatic patients appear to bring about regression of intraocular metastases [1, 2, 4]. In a symptomatic patient with metastatic cancer to the uvea or orbit unresponsive to chemotherapy, external beam radiation therapy is delivered in a tumor dose of 3000 to 4000 cGy over a 3- to 5-week period depending on the location and size of the lesion [4, 10, 17]. There is generally no particular reason to enucleate an eye known to contain a metastatic carcinoma unless there is intractable pain or the clinical presentation suggests the diagnosis of choroidal malignant melanoma. The long term prognosis of uveal metastases is poor. Various authors reported an overall median survival rate of 7.4 to 10.5 months from the time of ocular diagnosis in patients with metastatic carci-
43
noma to the eye and the orbit and this depends on the site of the primary malignancy [1-3, 13]. Our patient had an unusually rapid course and rapid death.
Acknowledgement Supported in part by a grant from the Macula Foundation, New York, New York, in part by the Robert Beckman Grant Endowment, Philadelphia, Pennsylvania and in part by the Eye Tumor Research Foundation Inc., Gladwyne, Pennsylvania. This paper was presented at the 1990 Theobald Society Meeting, Philadelphia, PA, May 11, 1990.
References 1. Shields JA, Shakin EP, Shields CL. Metastatic malignant tumors. In: Gold DH, Weingiest TA, editors. The eye in systemic diseases. Philadelphia: Lippincott, 1990; 299-303. 2. Shakin EP, Shields JA. The eye and ocular adnexa in systemic malignancy. In: Duana TD, editor. Duane's clinical ophthalmology. Philadelphia: Lippincott, 1989; 5: 34. 3. Ferry AP, Font RL. Carcinoma metastatic to the eye and orbit. A clinicopathologic study of 227 cases. Arch Ophthalmol 1974; 92: 276-86. 4. Shields JA, Shields CL. Intraocular tumors. A text and atlas. Philadelphia: WB Saunders co, 1992. In press. 5. Nelson CC, Hertzberg BS, Klintworth GK. A histopathologic study of 716 unselected eyes in patients with cancer at the time of death. Am J Ophthalmol 1983; 95: 728-33. 6. BullockJD, Yanes B. Ophthalmic manifestations ofmetastatic breast cancer. Ophthalmology 1980; 87: 961-72. 7. Godtfredsen E. On the frequency of secondary carcinoma in the choroid. Acta Ophthalmol 1944; 22: 394-400. 8. Bloch RS, Gartner S. The incidence of ocular metastatic carcinoma. Arch Ophthalmol 1971; 85: 673-75. 9. Stephens RF, Shields JA. Diagnosis and management of cancer metastatic to the uvea: a study of 70 cases. Ophthalmology 1979; 86: 1336-49. 10. Shields CL, Shields JA, Peggs M. Metastatic tumors to the orbit. Ophthal Reconstr Plast Surg 1988; 4: 73-80. 11. Font RL, Spaulding AG, Zimmerman LE. Diffuse malignant melanoma of the uveal tract: a clinicopathologic report of 54 cases. Trans Am Acad Ophthalmol and Otolaryngol 1968; 72: 87%95. 12. Kindermann WR, Shields JA, Eiferman RA et al. Metastatic renal cell carcinoma to the eye and adnexae. Ophthalmology 1981; 88: 1347-50. 13. Freedman MI, Folk JC. Metastatic tumors to the eye and
44
P. De Potter et al.
orbit: patient survival and clinical characteristics. Arch Ophthalmol 1987; 107: 1215-19. 14. Kolodny NH, Gragoudas ES, D'Amico DJ et al. Proton and sodium-23 magnetic resonance imaging of human ocular tissues: A model study. Arch Ophthalmol 1987; 105: 1532-36. 15. Haik BG, Saint Louis L, Smith ME et al. Magnetic resonance imaging in choroidal tumors. Ann Ophthalmo11987; 19: 218-38. 16. Peyman GA, Mafee MF. Uveal melanomas and simulating lesions: the role of magnetic resonance imaging and com-
puted tomography. Radiol Clin North Am 1987; 25: 47186. 17. Shields JA. Metastatic cancer to the orbit. In: Diagnosis and Management of Orbital Tumors. Philadelphia: WB Saunders, 1989: 291-315.
Address for offprints: Jerry A. Shields, Oncology Service, Wills Eye Hospital, Ninth and Walnut Streets, Philadelphia, PA 19107, USA
