Ophthalmic Surgery, Lasers and Imaging Retina

Case Report 

Cortical Blindness and Choroidal Metastases Secondary to Metastatic Breast Carcinoma in a Male Patient

Neal Palejwala, MD; Steven Yeh, MD; Hans E. Grossniklaus, MD, MBA; Chris Bergstrom, MD, OD

Abstract

Male breast carcinoma is a rare entity that often goes undiagnosed until advanced stages. The authors describe the case of a patient with profound vision loss who was found to have bilateral choroidal metastases as well as advanced cerebral metastatic disease. Further medical work-up revealed widespread infiltrative ductal breast carcinoma. Given the presence of large occipital lobe lesions, the etiology of the patient’s vision loss was thought to be cortical blindness. Prompt diagnosis and neurologic evaluation with this presentation is crucial because it can be associated with significant morbidity and mortality.

From Emory Eye Center, Emory University School of Medicine, Atlanta, Georgia.

The Emory Eye Center is supported by an unrestricted grant from Research to Prevent Blindness, Inc., New York, New York.

The authors have no financial or proprietary interest in the materials presented herein.

Address correspondence to Chris Bergstrom, MD, Emory Eye Center, 1365B Clifton Road NE, Atlanta, GA 30322.

Received: January 30, 2012
Accepted: November 13, 2012

Abstract

Male breast carcinoma is a rare entity that often goes undiagnosed until advanced stages. The authors describe the case of a patient with profound vision loss who was found to have bilateral choroidal metastases as well as advanced cerebral metastatic disease. Further medical work-up revealed widespread infiltrative ductal breast carcinoma. Given the presence of large occipital lobe lesions, the etiology of the patient’s vision loss was thought to be cortical blindness. Prompt diagnosis and neurologic evaluation with this presentation is crucial because it can be associated with significant morbidity and mortality.

From Emory Eye Center, Emory University School of Medicine, Atlanta, Georgia.

The Emory Eye Center is supported by an unrestricted grant from Research to Prevent Blindness, Inc., New York, New York.

The authors have no financial or proprietary interest in the materials presented herein.

Address correspondence to Chris Bergstrom, MD, Emory Eye Center, 1365B Clifton Road NE, Atlanta, GA 30322.

Received: January 30, 2012
Accepted: November 13, 2012

Introduction

Breast carcinoma accounts for less than 1% of cancer in men,1 and the median age of diagnosis is 68 years.2 Ninety percent of all breast tumors in men are invasive carcinomas, with infiltrating ductal carcinoma being the predominant histologic subtype. Metastatic disease most commonly occurs in the lungs, liver, and bone, with infrequent reports of metastatic choroidal lesions.3 We present a case of metastatic male breast carcinoma presenting with choroidal metastases and severe vision loss out of proportion to the examination findings. This discrepancy prompted emergent neuro-imaging and malignancy work-up and revealed advanced cerebral metastatic disease.

Case Report

A 49-year-old Caucasian male with a history of tobacco use and progressively altered mental status presented with bilateral subacute, painless loss of vision of unclear duration.

Visual acuity (VA) was light perception in both eyes. Extraocular motility was intact. Pupils were reactive in both eyes, and no relative afferent pupillary defect was seen. Dilated funduscopic examination showed elevated peripheral amelanotic choroidal lesions and optic disc edema in both eyes (Fig. 1). Given the patient’s extensive history of tobacco use, metastatic lung cancer was suspected as a likely diagnosis.

(A) Fundus photo of the left eye with optic disc edema and an amelanotic choroidal lesion inferiorly. (B) Axial T1-weighted brain MRI shows bilateral occipital lobe densities, which are partially hemorrhagic. (C) Axial CT of the chest with contrast shows numerous pulmonary nodules and an enhancing soft tissue mass in the left breast.

Figure 1. (A) Fundus photo of the left eye with optic disc edema and an amelanotic choroidal lesion inferiorly. (B) Axial T1-weighted brain MRI shows bilateral occipital lobe densities, which are partially hemorrhagic. (C) Axial CT of the chest with contrast shows numerous pulmonary nodules and an enhancing soft tissue mass in the left breast.

Cerebral metastatic disease was suspected given the severe vision loss, disc edema, and altered mentation. Emergent work-up including magnetic resonance imaging (MRI) of the brain and a computed tomography (CT) scan of the chest, abdomen, and pelvis were obtained. MRI of the brain demonstrated multiple complex partially cystic and enhancing masses in the cerebrum and cerebellum, the largest lesions involving the bilateral occipital lobes. Chest CT showed innumerable bilateral pulmonary nodules, an asymmetric mass in the left breast with associated skin thickening, and multiple lytic lesions throughout the thoracic spine consistent with extensive metastatic disease (Fig. 1).

General physical examination including a breast exam revealed an erythematous, excoriated lesion within the left areola. Dermatology was consulted and a shave biopsy was obtained. Pathology showed a carcinoma with ductular differentiation diffusely infiltrating the dermis suggestive of breast carcinoma. Immunohistochemistry showed positive staining for estrogen receptors, progesterone receptors, and HER-2/neu (Fig. 2).

(A) The tumor cells contain pleomorphic vesiculated nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm (hematoxylin and eosin 100×). (B) Immunohisochemical stains are positive for progesterone receptor in tumor cell nuclei (peroxidase-antiperoxidase 100×). (C) Immunohistochemical stains are positive for estrogen receptor in tumor cell nuclei (peroxidase-antiperoxidase 100×). (D) Immunohistochemical stains are positive for HER-2/neu in tumor cell membranes (peroxidase-antiperoxidase 100×).

Figure 2. (A) The tumor cells contain pleomorphic vesiculated nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm (hematoxylin and eosin 100×). (B) Immunohisochemical stains are positive for progesterone receptor in tumor cell nuclei (peroxidase-antiperoxidase 100×). (C) Immunohistochemical stains are positive for estrogen receptor in tumor cell nuclei (peroxidase-antiperoxidase 100×). (D) Immunohistochemical stains are positive for HER-2/neu in tumor cell membranes (peroxidase-antiperoxidase 100×).

Palliative whole brain irradiation and tamoxifen were recommended by the radiation oncology and medical oncology services, respectively. However, owing to the patient’s poor functional status and poor prognosis, the patient’s family deferred further palliative therapy and elected patient transfer to hospice care.

Discussion

The combination of central nervous system (CNS) and ocular involvement from male breast carcinoma has rarely been reported.4, 5 Only 1,500 cases of male breast carcinoma are reported annually, accounting for less than 1% of all breast cancers.1 Given the profound level of vision loss and normal pupillary testing in our patient, there was significant concern for cortical blindness. An emergent CNS work-up revealed the presence of bilateral occipital lobe involvement in addition to other metastatic CNS lesions.

The most common cause of cortical blindness among all patients is ischemic injury to the occipital lobe, which unfortunately portends a poor visual prognosis.6 Other considerations in the setting of suspected malignancy include posterior reversible leukoencephalopathy, cerebral edema secondary to metastases, chemotherapeutics (eg, vinca alkaloids, tacrolimus, and cyclosporine A), leptomeningeal carcinomatosis, and infectious meningitis.

Posterior reversible leukoencephalopathy syndrome (PRES) is a subacute neurological syndrome manifesting with headache, seizures, and visual abnormalities. Visual symptoms vary from blurred vision to visual hallucinations to cortical blindness. Usually associated with malignant hypertension and eclampsia, a number of cases of PRES have been reported in association with malignancy. The exact mechanism is unknown; however, cytotoxic chemotherapeutics (platinum analogues, antimetabolites, and vinca alkaloids) have been implicated. Direct endothelial injury causing vasogenic edema has been seen after administration of such chemotherapeutics.7

Neurotoxicity secondary to vinca alkaloids has been implicated as a cause of cerebral blindness in a handful of patients on chemotherapy. Schouten et al.8 presented a case of an 8-year-old boy who developed multiple episodes of transient blindness while on vincristine therapy. Testing of the anterior visual pathway yielded results within normal limits. CT and MRI showed nonenhancing occipital lobe lesions. Three weeks later, a second round of chemotherapy without vincristine was administered and the patient did not experience any further visual symptoms. Follow-up MRI revealed no abnormalities.

Blindness in the setting of confirmed or suspected malignancy should prompt a thorough neurologic evaluation including both neuro-imaging and lumbar puncture because meningeal disease such as infectious meningitis and leptomeningeal carcinomatosis can be an etiology with significant mortality and morbidity. Our patient’s family, however, deferred further therapy or testing, and it is unclear whether he would have derived any visual benefit, particularly given that all treatment options were considered palliative.

References

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  2. Heller KS, Rosen PP, Schottenfeld D, Ashikari R, Kinne DW. Male breast cancer: a clinicopathologic study of 97 cases. Ann Surg. 1978;188(10:60–65. doi:10.1097/00000658-197807000-00010 [CrossRef]
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  4. Hood CT, Budd GT, Zakov ZN, Singh AD. Male breast carcinoma metastatic to the choroid: report of 3 cases and review of the literature. Eur J Ophthalmol. 2011;21(4):459–467. doi:10.5301/EJO.2010.6191 [CrossRef]
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  6. Aldrich MS, Alessi AG, Beck RW, Gilman S. Cortical blindness: etiology, diagnosis, and prognosis. Ann Neurol. 21(2):149–158.
  7. Zhang SZ, Wang ZH, Hu HS. Effect of intra-arterial perfusion of cisplatin combined with concomitant radiation therapy on the healing of microvasculature anastomoses: an experimental study in rabbits. Shanghai Kou Qiang Yi Xue. 2005;14(5):490–494.
  8. Schouten D, de Graaf SS, Verrips A. Transient cortical blindness following vincristine therapy. Med Pediatr Oncol. 2003;41(5):470. doi:10.1002/mpo.10377 [CrossRef]

10.3928/23258160-20121221-18

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