METASTATIC CHORIOCARCINOMA TO THE CHOROID WITH CONCURRENT OPTIC NERVE INVOLVEMENT: A CASE REPORT AND REVIEW OF THE LITERATURE Christine N. Pham Lagler, MD, Donna E. Siracuse-Lee, MD
Purpose: To describe a case of choroidal metastasis of choriocarcinoma, which presented as a rapidly progressive hemorrhagic retinal detachment with concurrent optic nerve involvement suggestive of inflammation or metastasis. Method: This is a case report of a 43-year-old patient with a known history of choriocarcinoma metastases to both lungs who presented with 1 week of right eye pain and decreased vision. Dilated fundus examination was significant for a large, bullous, hemorrhagic retinal detachment, with the appearance of an underlying choroidal mass in the superonasal retina. Magnetic resonance imaging result was significant for a 1.6 × 1.3-cm heterogeneous mass in the right globe, suggestive of metastasis, and inflammatory changes in the sclera, optic nerve, and optic nerve sheath. Additionally, hemorrhagic metastases were found in the right occipital and left frontal lobes. Results: To our knowledge, this is the first case of metastatic choriocarcinoma to the choroid with concurrent optic nerve involvement. Conclusion: Choroidal metastases may be diagnosed in the setting of more widespread central nervous system involvement and should be investigated accordingly. RETINAL CASES & BRIEF REPORTS 6:313–316, 2012
should prompt urgent evaluation for other metastatic lesions of the brain.
From the Department of Ophthalmology, Boston University School of Medicine, Boston Medical Center, Boston, Massachusetts.
Methods
C
horoidal metastases are a rare complication of choriocarcinoma. The literature reports approximately 22 cases of similar lesions. To date, this is the first reported case of choroidal metastasis of choriocarcinoma with concurrent optic nerve and optic nerve sheath involvement, which occurred in the setting of previously undiagnosed cerebral metastases. This constellation of ophthalmic findings on examination
This review retrospectively analyzed the published literature of patients with metastatic choriocarcinoma to the choroid and/or other ocular structures to examine and summarize this rare clinical presentation. PubMed/MEDLINE and the Cochrane database were searched for all literature from 1950 to the present. Published English abstracts were searched for all literature containing the key terms metastatic choriocarcinoma, choroid, uveal, eye, ocular, optic neuropathy, and optic nerve. The requirements for inclusion were that the article or abstract was written in English, the patient had a documented ophthalmic examination, and the diagnosis of metastatic choriocarcinoma to the eye was confirmed clinically or by biopsy if feasible.
The authors declare no conflict of interest. Reprint requests: Donna E. Siracuse-Lee, MD, Department of Ophthalmology, Boston University School of Medicine, Boston Medical Center, 85 East Concord Street, 8th Floor, Boston, MA 02118; e-mail: [email protected]
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We included case reports only if they contributed new information about characteristics, diagnosis, or treatment of the disease.
Case Report A 43-year-old patient presented with a 1-week history of right eye pain and decreased vision. She had a history of choriocarcinoma with known metastases to both lungs. The patient was first diagnosed with a molar pregnancy 15 months before the occurrence of ocular symptoms. Her last menstrual period had been 5 months before this diagnosis. At that time she underwent uterine biopsy, which was positive for gestational choriocarcinoma. She initially received seven cycles of EMA-CO chemotherapy (etoposide/methotrexate/dactinomycin and cyclophosphamide/vincristine) and had a partial response. However, 1 month later, she had a rise in serum beta-human chorionic gonadotropin (bHCG) and was found to have disease in the uterus and in both lungs by positron emission tomography scan. Subsequently, she underwent a total abdominal hysterectomy and bilateral salpingo-oopherectomy. This was followed by an additional four cycles of EMA-EP chemotherapy (etoposide/methotrexate/dactinomycin and etoposide/cisplatin). Before presentation for her ocular symptoms, her oncology team had planned surgical removal of one pulmonary lesion and another subsequent course of chemotherapy. Magnetic resonance imaging of the head 6 months before presentation of ocular symptoms had been normal. Upon presentation of her ocular symptoms, the patient appeared fatigued and uncomfortable because of moderate pain in the right eye and right side of her head. Best-corrected visual acuity was 20/ 40 in the right eye and 20/20 in the left eye. She had a small afferent pupillary defect in the right eye. Her confrontational visual field demonstrated severe generalized constriction in the right eye but was normal in the left. Extraocular motility was normal, but with increased pain in elevation in the right eye. There was slight ptosis of the right upper lid. On slit-lamp examination, the right eye had trace injection of the nasal conjunctiva. The conjunctiva of the left eye was white and quiet. She had trace cell in the right eye. She had no iris lesions, and the lenses were clear in both eyes. Intraocular pressure was 15 in both eyes. Dilated fundoscopic examination was significant for 2+ erythrocytes and 2+ leukocytes in the vitreous right eye. There was a large, bullous, hemorrhagic retinal detachment, with the appearance of an underlying choroidal mass in the superonasal retina, limited at the posterior edge by the superior arcade. The right optic nerve appeared normal, without edema or pallor, and was uninvolved in the mass. The examination was otherwise normal in the left eye. B-scan ultrasonography in the right eye demonstrated a large choroidal mass in the superonasal quadrant, with a hemorrhagic retinal detachment overlying the mass (Figure 1). Subretinal hemorrhage was also found inferiorly. B-scan in the left eye was normal. The patient was subsequently treated with cycloplegia and topical steroids for symptomatic relief of the inflammatory component. She was sent for a repeat magnetic resonance imaging of the head in conjunction with her oncology team. The decision regarding treatment with oral steroids was deferred to her oncologist, who placed her on oral dexamethasone. The magnetic resonance imaging result was significant for a 1.6 × 1.3-cm heterogeneous mass in the right globe, suggestive of metastasis with a hemorrhagic component (Figure 2). In addition, inflammatory changes of the globe were seen peripherally, suggestive of scleritis. An increase in signal density was noted within the right optic nerve, and read as a possible sign of inflammation. Additionally, the right optic nerve sheath demonstrated
Fig. 1. B-scan ultrasonography imaging demonstrating large choroidal mass with overlying hemorrhagic retinal detachment in right eye.
a mild peripheral enhancement, interpreted as representing inflammatory changes versus metastases. Last and most importantly, foci representing hemorrhagic metastases were found in the right occipital and left frontal lobes, with acute infarct from tumor deposition in the right frontal lobe. Four days after initial presentation for ocular symptoms, she lost light perception in the right eye. Intraocular pressure was measured at 32 mmHg, and slit-lamp examination demonstrated a significant increase in intraocular inflammation, with keratic precipitates and 1 + cell noted in the anterior chamber. The size of the bullous retinal detachment had increased to the point where dilated fundus examination (DFE) was no longer possible (Figure 3). She was
Fig. 2. T2-weighted axial head magnetic resonance imaging showing heterogeneous mass in right globe and enhancement of optic nerve.
CHORIOCARCINOMA WITH NERVE DISEASE started on topical glaucoma therapy, and topical steroids were increased. Per the patient’s oncology team, she was hospitalized in preparation for radiation therapy of her cerebral and ocular metastases, followed by additional chemotherapy. Before radiotherapy, the patient noted decreased vision in the left eye. A followup magnetic resonance imaging of the brain exhibited a new 2 × 2.5 × 4-cm hemorrhage with surrounding edema in the right occipital lobe, in the area with previously noted hemorrhagic metastases. She also had an associated subarachnoid hemorrhage. Unfortunately, she subsequently had a cardiac arrest the next day and was noted on head computed tomography postarrest to have a significant increase in the intraparenchymal hemorrhage and new intraventricular hemorrhage. The patient died later that same day.
Discussion Gestational trophoblastic disease may range from a benign hydatidiform mole to the rare, highly malignant choriocarcinoma.1 Gestational choriocarcinoma may be associated with normal pregnancy (25%), abortion (25%), or a hydatidaform mole (50%), and follows 1/50,000 live births.2 Malignancy can also occur as a result of nongestational disease originating from germ cells of the ovary or testis, but in this setting occurs most commonly in males.3 Twenty-two cases of ocular metastases from choriocarcinoma have been reported in the literature dating back to 1926, with 21 localized to the choroid and 1 to the anterior segment.2,3 No reported cases have presented with concurrent optic nerve findings, suggestive of inflammation or metastasis. There are also case reports in the literature of cavernous sinus involvement with associated fistula formation and cavernous sinus syndrome.4 As choriocarcinomas metastasize via the bloodstream, most reported ocular lesions have been located in the choroid.1,2 The choroid, with its abundant blood supply, accounts for 88% of uveal lesions.5 Although most ocular choriocarcinomas occur within the setting
Fig. 3. Slit-lamp photograph of right eye demonstrating bullous retinal detachment visible through dilated pupil.
315
of known metastatic disease, in five cases in the literature, the ocular metastasis was the presenting symptom.1,3,6 Choriocarcinoma, by its vascular nature, commonly metastasizes to other highly vascular tissues, such as the liver, lung, and brain.5,6 These highly vascular lesions may be associated with some amount of hemorrhage at presentation.6 Therefore, patients with resistant disease are at risk of death from severe hemorrhage of the associated organs.3 Symptoms of uveal metastases commonly include decreased vision, with only 26% presenting with 20/30 or better best-corrected visual acuity. Flashes and floaters (12%) and pain (7%) secondary to glaucoma, inflammation, tumor necrosis, or rarely scleral involvement may be present.5 Other associated ocular findings include elevated IOP (4%), optic disk metastases (5%), and serous retinal detachment (91%).5,7 Progressive glaucoma secondary to choroidal and ciliary body hemorrhage is rare.7 However, in case reports of metastatic choriocarcinoma, accompanying subretinal and vitreous hemorrhage has been noted.1,6 Ultrasound normally demonstrates diffuse thickening of the choroid with moderate to high internal acoustic reflectivity. Chemotherapy for choriocarcinoma is usually curative in 98% of female patients, even in the setting of metastatic disease, but with only an 80% cure rate in those with brain metastases.6,7 A recent Cochrane systematic review of combination chemotherapy for high-risk gestational trophoblastic tumors failed to reveal any firm conclusions about the optimal chemotherapy regimen.8 Poor prognostic signs include long duration of the disease before diagnosis, a markedly increased bHCG before treatment, cerebral or hepatic metastases, or nongestational disease.7 Previous reports of life expectancy with uveal metastases have been found to be 2 months to 13 months, depending on diagnosis, with occasional outliers.7 Choroidal metastases are generally associated with a poor prognosis.2 In general, choroidal metastases may be treated via several modalities, including chemotherapy (8%), external beam radiation (39%), plaque radiotherapy (7%), hormonal therapy (1%), resection (6%), and observation (14%).5 Plaque radiation therapy may also be considered to reduce visual loss. External beam radiation therapy may be used as local therapy, but cataract, radiation retinopathy, optic neuropathy, exposure keratopathy, and intraocular neovascularization may complicate the visual course thereafter.9 Progression to a blind, painful eye has been noted to occur in as little as 5 days from advancing hemorrhage and secondary glaucoma.1 Rapid posterior extension through the sclera, in as little as several weeks,
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has also been noted.1 Death often occurs within several weeks to months of diagnosis of ocular disease.1,2 One other patient reportedly died of sudden intracerebral hemorrhage 6 months postdiagnosis of ocular disease, and in our case, within ,2 weeks.2 However, several cases have had ophthalmic and/or life-saving responses to chemotherapy.1,6,10 Brain metastases may coexist, and sudden cerebral hemorrhage and death from these metastases, such as in the presented patient, may quickly follow.1 Patients with choroidal metastases should have neuroimaging and receive urgent treatment in conjunction with their oncology team. In the case of our patient, while computed tomography imaging confirmed optic nerve involvement, a pathologic diagnosis of the optic nerve was not available to distinguish an inflammatory versus metastatic etiology for the optic nerve and sheath enhancement. Nonetheless, as previous case reports have not demonstrated involvement of the optic nerve, it should be emphasized that any change in the optic nerve examination, that is, an afferent pupillary defect, are highly suggestive of optic nerve involvement in the form of inflammation or metastasis, and possibly brain metastases. Future studies are necessary to determine the most effective treatment approach for both systemic and ocular manifestations of choriocarcinoma. Key words: brain metastases, choriocarcinoma, choroidal metastases, hemorrhagic retinal detachment, molar pregnancy, tumor.
Acknowledgment We would like to acknowledge Dr. Edward Feinberg for his retinal consultation. References 1. Conlon MR, Collyer RT, Joseph MG, Siebert LF. Metastatic choroidal choriocarcinoma: a clinicopathological study. Can J Ophthalmol 1991;26:321–324. 2. Flam F, Kock E. Metastatic choroidal choriocarcinoma. Acta Obstet Gynecol Scand 1996;75:688–689. 3. Frank KW, Sugar S, Sherman AI, et al. Anterior segment metastasis from an ovarian choriocarcinoma. Am J Ophthalmol 1979;87:778–782. 4. Sharkawi E, Tumuluri K, Olver JM. Metastastic choriocarcinoma causing cavernous sinus syndrome. Br J Ophthalmol 2006;90:654–655. 5. Shields CL, Shields JA, Gross NE, et al. Survey of 520 eyes with uveal metastases. Ophthalmology 1997;104:1265–1276. 6. Barondes MJ, Hamilton AM, Hungerford J, Rustin GJ. Treatment of choroidal metastasis from choriocarcinoma. Case report. Arch Ophthalmol 1989;107:796–798. 7. Stephens RF, Shields JA. Diagnosis and management of cancer metastatic to the uvea: a study of 70 cases. Ophthalmology 1979;86:1336–1349. 8. Deng L, Yan X, Zhang J, Wu T. Combination chemotherapy for high-risk gestational trophoblastic tumour. Cochrane Database Syst Rev 2009;2:CD005196. 9. Venkatesh P, Garg S. Regression of choroidal metastases from breast carcinoma following Letrazole therapy. Clin Exper Ophthalmol 2007;35:492–494. 10. Khurana RN, Dibernardo C, Handa JT. Improved systemic chemotherapy for metastatic testicular choriocarcinoma can result in excellent prognosis for life and vision. Arch Ophthalmol 2008;126:1008–1009.
Purpose: To describe a case of choroidal metastasis of choriocarcinoma, which presented as a rapidly progressive hemorrhagic retinal detachment with concurrent optic nerve involvement suggestive of inflammation or metastasis. Method: This is a case report of a 43-year-old patient with a known history of choriocarcinoma metastases to both lungs who presented with 1 week of right eye pain and decreased vision. Dilated fundus examination was significant for a large, bullous, hemorrhagic retinal detachment, with the appearance of an underlying choroidal mass in the superonasal retina. Magnetic resonance imaging result was significant for a 1.6 × 1.3-cm heterogeneous mass in the right globe, suggestive of metastasis, and inflammatory changes in the sclera, optic nerve, and optic nerve sheath. Additionally, hemorrhagic metastases were found in the right occipital and left frontal lobes. Results: To our knowledge, this is the first case of metastatic choriocarcinoma to the choroid with concurrent optic nerve involvement. Conclusion: Choroidal metastases may be diagnosed in the setting of more widespread central nervous system involvement and should be investigated accordingly. RETINAL CASES & BRIEF REPORTS 6:313–316, 2012
should prompt urgent evaluation for other metastatic lesions of the brain.
From the Department of Ophthalmology, Boston University School of Medicine, Boston Medical Center, Boston, Massachusetts.
Methods
C
horoidal metastases are a rare complication of choriocarcinoma. The literature reports approximately 22 cases of similar lesions. To date, this is the first reported case of choroidal metastasis of choriocarcinoma with concurrent optic nerve and optic nerve sheath involvement, which occurred in the setting of previously undiagnosed cerebral metastases. This constellation of ophthalmic findings on examination
This review retrospectively analyzed the published literature of patients with metastatic choriocarcinoma to the choroid and/or other ocular structures to examine and summarize this rare clinical presentation. PubMed/MEDLINE and the Cochrane database were searched for all literature from 1950 to the present. Published English abstracts were searched for all literature containing the key terms metastatic choriocarcinoma, choroid, uveal, eye, ocular, optic neuropathy, and optic nerve. The requirements for inclusion were that the article or abstract was written in English, the patient had a documented ophthalmic examination, and the diagnosis of metastatic choriocarcinoma to the eye was confirmed clinically or by biopsy if feasible.
The authors declare no conflict of interest. Reprint requests: Donna E. Siracuse-Lee, MD, Department of Ophthalmology, Boston University School of Medicine, Boston Medical Center, 85 East Concord Street, 8th Floor, Boston, MA 02118; e-mail: [email protected]
313
314
RETINAL CASES & BRIEF REPORTS´ 2012 VOLUME 6 NUMBER 3
We included case reports only if they contributed new information about characteristics, diagnosis, or treatment of the disease.
Case Report A 43-year-old patient presented with a 1-week history of right eye pain and decreased vision. She had a history of choriocarcinoma with known metastases to both lungs. The patient was first diagnosed with a molar pregnancy 15 months before the occurrence of ocular symptoms. Her last menstrual period had been 5 months before this diagnosis. At that time she underwent uterine biopsy, which was positive for gestational choriocarcinoma. She initially received seven cycles of EMA-CO chemotherapy (etoposide/methotrexate/dactinomycin and cyclophosphamide/vincristine) and had a partial response. However, 1 month later, she had a rise in serum beta-human chorionic gonadotropin (bHCG) and was found to have disease in the uterus and in both lungs by positron emission tomography scan. Subsequently, she underwent a total abdominal hysterectomy and bilateral salpingo-oopherectomy. This was followed by an additional four cycles of EMA-EP chemotherapy (etoposide/methotrexate/dactinomycin and etoposide/cisplatin). Before presentation for her ocular symptoms, her oncology team had planned surgical removal of one pulmonary lesion and another subsequent course of chemotherapy. Magnetic resonance imaging of the head 6 months before presentation of ocular symptoms had been normal. Upon presentation of her ocular symptoms, the patient appeared fatigued and uncomfortable because of moderate pain in the right eye and right side of her head. Best-corrected visual acuity was 20/ 40 in the right eye and 20/20 in the left eye. She had a small afferent pupillary defect in the right eye. Her confrontational visual field demonstrated severe generalized constriction in the right eye but was normal in the left. Extraocular motility was normal, but with increased pain in elevation in the right eye. There was slight ptosis of the right upper lid. On slit-lamp examination, the right eye had trace injection of the nasal conjunctiva. The conjunctiva of the left eye was white and quiet. She had trace cell in the right eye. She had no iris lesions, and the lenses were clear in both eyes. Intraocular pressure was 15 in both eyes. Dilated fundoscopic examination was significant for 2+ erythrocytes and 2+ leukocytes in the vitreous right eye. There was a large, bullous, hemorrhagic retinal detachment, with the appearance of an underlying choroidal mass in the superonasal retina, limited at the posterior edge by the superior arcade. The right optic nerve appeared normal, without edema or pallor, and was uninvolved in the mass. The examination was otherwise normal in the left eye. B-scan ultrasonography in the right eye demonstrated a large choroidal mass in the superonasal quadrant, with a hemorrhagic retinal detachment overlying the mass (Figure 1). Subretinal hemorrhage was also found inferiorly. B-scan in the left eye was normal. The patient was subsequently treated with cycloplegia and topical steroids for symptomatic relief of the inflammatory component. She was sent for a repeat magnetic resonance imaging of the head in conjunction with her oncology team. The decision regarding treatment with oral steroids was deferred to her oncologist, who placed her on oral dexamethasone. The magnetic resonance imaging result was significant for a 1.6 × 1.3-cm heterogeneous mass in the right globe, suggestive of metastasis with a hemorrhagic component (Figure 2). In addition, inflammatory changes of the globe were seen peripherally, suggestive of scleritis. An increase in signal density was noted within the right optic nerve, and read as a possible sign of inflammation. Additionally, the right optic nerve sheath demonstrated
Fig. 1. B-scan ultrasonography imaging demonstrating large choroidal mass with overlying hemorrhagic retinal detachment in right eye.
a mild peripheral enhancement, interpreted as representing inflammatory changes versus metastases. Last and most importantly, foci representing hemorrhagic metastases were found in the right occipital and left frontal lobes, with acute infarct from tumor deposition in the right frontal lobe. Four days after initial presentation for ocular symptoms, she lost light perception in the right eye. Intraocular pressure was measured at 32 mmHg, and slit-lamp examination demonstrated a significant increase in intraocular inflammation, with keratic precipitates and 1 + cell noted in the anterior chamber. The size of the bullous retinal detachment had increased to the point where dilated fundus examination (DFE) was no longer possible (Figure 3). She was
Fig. 2. T2-weighted axial head magnetic resonance imaging showing heterogeneous mass in right globe and enhancement of optic nerve.
CHORIOCARCINOMA WITH NERVE DISEASE started on topical glaucoma therapy, and topical steroids were increased. Per the patient’s oncology team, she was hospitalized in preparation for radiation therapy of her cerebral and ocular metastases, followed by additional chemotherapy. Before radiotherapy, the patient noted decreased vision in the left eye. A followup magnetic resonance imaging of the brain exhibited a new 2 × 2.5 × 4-cm hemorrhage with surrounding edema in the right occipital lobe, in the area with previously noted hemorrhagic metastases. She also had an associated subarachnoid hemorrhage. Unfortunately, she subsequently had a cardiac arrest the next day and was noted on head computed tomography postarrest to have a significant increase in the intraparenchymal hemorrhage and new intraventricular hemorrhage. The patient died later that same day.
Discussion Gestational trophoblastic disease may range from a benign hydatidiform mole to the rare, highly malignant choriocarcinoma.1 Gestational choriocarcinoma may be associated with normal pregnancy (25%), abortion (25%), or a hydatidaform mole (50%), and follows 1/50,000 live births.2 Malignancy can also occur as a result of nongestational disease originating from germ cells of the ovary or testis, but in this setting occurs most commonly in males.3 Twenty-two cases of ocular metastases from choriocarcinoma have been reported in the literature dating back to 1926, with 21 localized to the choroid and 1 to the anterior segment.2,3 No reported cases have presented with concurrent optic nerve findings, suggestive of inflammation or metastasis. There are also case reports in the literature of cavernous sinus involvement with associated fistula formation and cavernous sinus syndrome.4 As choriocarcinomas metastasize via the bloodstream, most reported ocular lesions have been located in the choroid.1,2 The choroid, with its abundant blood supply, accounts for 88% of uveal lesions.5 Although most ocular choriocarcinomas occur within the setting
Fig. 3. Slit-lamp photograph of right eye demonstrating bullous retinal detachment visible through dilated pupil.
315
of known metastatic disease, in five cases in the literature, the ocular metastasis was the presenting symptom.1,3,6 Choriocarcinoma, by its vascular nature, commonly metastasizes to other highly vascular tissues, such as the liver, lung, and brain.5,6 These highly vascular lesions may be associated with some amount of hemorrhage at presentation.6 Therefore, patients with resistant disease are at risk of death from severe hemorrhage of the associated organs.3 Symptoms of uveal metastases commonly include decreased vision, with only 26% presenting with 20/30 or better best-corrected visual acuity. Flashes and floaters (12%) and pain (7%) secondary to glaucoma, inflammation, tumor necrosis, or rarely scleral involvement may be present.5 Other associated ocular findings include elevated IOP (4%), optic disk metastases (5%), and serous retinal detachment (91%).5,7 Progressive glaucoma secondary to choroidal and ciliary body hemorrhage is rare.7 However, in case reports of metastatic choriocarcinoma, accompanying subretinal and vitreous hemorrhage has been noted.1,6 Ultrasound normally demonstrates diffuse thickening of the choroid with moderate to high internal acoustic reflectivity. Chemotherapy for choriocarcinoma is usually curative in 98% of female patients, even in the setting of metastatic disease, but with only an 80% cure rate in those with brain metastases.6,7 A recent Cochrane systematic review of combination chemotherapy for high-risk gestational trophoblastic tumors failed to reveal any firm conclusions about the optimal chemotherapy regimen.8 Poor prognostic signs include long duration of the disease before diagnosis, a markedly increased bHCG before treatment, cerebral or hepatic metastases, or nongestational disease.7 Previous reports of life expectancy with uveal metastases have been found to be 2 months to 13 months, depending on diagnosis, with occasional outliers.7 Choroidal metastases are generally associated with a poor prognosis.2 In general, choroidal metastases may be treated via several modalities, including chemotherapy (8%), external beam radiation (39%), plaque radiotherapy (7%), hormonal therapy (1%), resection (6%), and observation (14%).5 Plaque radiation therapy may also be considered to reduce visual loss. External beam radiation therapy may be used as local therapy, but cataract, radiation retinopathy, optic neuropathy, exposure keratopathy, and intraocular neovascularization may complicate the visual course thereafter.9 Progression to a blind, painful eye has been noted to occur in as little as 5 days from advancing hemorrhage and secondary glaucoma.1 Rapid posterior extension through the sclera, in as little as several weeks,
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RETINAL CASES & BRIEF REPORTS´ 2012 VOLUME 6 NUMBER 3
has also been noted.1 Death often occurs within several weeks to months of diagnosis of ocular disease.1,2 One other patient reportedly died of sudden intracerebral hemorrhage 6 months postdiagnosis of ocular disease, and in our case, within ,2 weeks.2 However, several cases have had ophthalmic and/or life-saving responses to chemotherapy.1,6,10 Brain metastases may coexist, and sudden cerebral hemorrhage and death from these metastases, such as in the presented patient, may quickly follow.1 Patients with choroidal metastases should have neuroimaging and receive urgent treatment in conjunction with their oncology team. In the case of our patient, while computed tomography imaging confirmed optic nerve involvement, a pathologic diagnosis of the optic nerve was not available to distinguish an inflammatory versus metastatic etiology for the optic nerve and sheath enhancement. Nonetheless, as previous case reports have not demonstrated involvement of the optic nerve, it should be emphasized that any change in the optic nerve examination, that is, an afferent pupillary defect, are highly suggestive of optic nerve involvement in the form of inflammation or metastasis, and possibly brain metastases. Future studies are necessary to determine the most effective treatment approach for both systemic and ocular manifestations of choriocarcinoma. Key words: brain metastases, choriocarcinoma, choroidal metastases, hemorrhagic retinal detachment, molar pregnancy, tumor.
Acknowledgment We would like to acknowledge Dr. Edward Feinberg for his retinal consultation. References 1. Conlon MR, Collyer RT, Joseph MG, Siebert LF. Metastatic choroidal choriocarcinoma: a clinicopathological study. Can J Ophthalmol 1991;26:321–324. 2. Flam F, Kock E. Metastatic choroidal choriocarcinoma. Acta Obstet Gynecol Scand 1996;75:688–689. 3. Frank KW, Sugar S, Sherman AI, et al. Anterior segment metastasis from an ovarian choriocarcinoma. Am J Ophthalmol 1979;87:778–782. 4. Sharkawi E, Tumuluri K, Olver JM. Metastastic choriocarcinoma causing cavernous sinus syndrome. Br J Ophthalmol 2006;90:654–655. 5. Shields CL, Shields JA, Gross NE, et al. Survey of 520 eyes with uveal metastases. Ophthalmology 1997;104:1265–1276. 6. Barondes MJ, Hamilton AM, Hungerford J, Rustin GJ. Treatment of choroidal metastasis from choriocarcinoma. Case report. Arch Ophthalmol 1989;107:796–798. 7. Stephens RF, Shields JA. Diagnosis and management of cancer metastatic to the uvea: a study of 70 cases. Ophthalmology 1979;86:1336–1349. 8. Deng L, Yan X, Zhang J, Wu T. Combination chemotherapy for high-risk gestational trophoblastic tumour. Cochrane Database Syst Rev 2009;2:CD005196. 9. Venkatesh P, Garg S. Regression of choroidal metastases from breast carcinoma following Letrazole therapy. Clin Exper Ophthalmol 2007;35:492–494. 10. Khurana RN, Dibernardo C, Handa JT. Improved systemic chemotherapy for metastatic testicular choriocarcinoma can result in excellent prognosis for life and vision. Arch Ophthalmol 2008;126:1008–1009.
