Case Study

Spontaneous Intraocular Hemorrhage in Metastatic Testicular Cancer

Daniel A. Chruscicki, MD • Stephanie Sutton, MS • Leigh Sutton, MS

A healthy, 21-year-old man presented with blurry vision, which started two days earlier. He said he felt a “pop” at the onset of blurry vision, which was followed by a severe head ache. His mother stated he was having neck pain over the last week. The night before, the patient went to an emergency room for nausea, vomiting and severe headaches. No imaging was performed at the ER. The patient had no past medical history, and the review of systems was otherwise negative.

On examination, his visual acuity was counting fingers at 1 ft in the right eye and 20/30 in the left eye. The intraocular pressures were 21 mm Hg in the right eye and 11 mm Hg in the left eye by applanation. Slit-lamp biomicroscopy was within normal limits. Extraocular movements were intact.

A dilated fundus exam of the right eye was significant for a clear vitreous. There was a normal optic nerve with a 0.3 cup-to-disc ratio. The macula and peripheral retinal exam were significant for extensive subretinal and choroidal hemorrhages in the superior and nasal periphery. B-scan ultrasonography confirmed the presence of the subretinal/choroidal hemorrhages (Figure 1). No subretinal or choroidal lesions were noted. The dilated fundus exam of the left eye was unremarkable.

Figure 1. B-scan ultrasonography confirmed the presence of subretinal/choroidal hemorrhages in this 21-year-old man with sudden vision loss.

The patient was put on timolol/alphagan combination drops and Diamox Sequels to prevent any IOP spikes. Given the patient's presentation, he was immediately sent back to the ER for CT of the head and CT angiography to rule out subarachnoid hemorrhage. The head CT, with and without contrast, was significant for “retinal hemorrhage superiorly and medially.” There was no evidence of “intra-cranial hemorrhage or intracranial abnormality” seen. The CT angiography was also normal. The subsequent physical exam at the ER failed to provide any addition information.

The patient was seen two days later for headaches and “eye pressure.” The IOPs were normal, so the pain was attributed to the choroidal hemorrhages. There was a new subconjunctival hemorrhage, and the anterior chamber was slightly shallow. There were no signs of proptosis on Hertel Exophthalmo meter. The patient was started on topical steroid eye drops, in addition to cyclogel BID and Azopt TID. He was sent to his primary care physician for a physical exam and a coagulability workup. The physical exam failed to provide any additional information, and the coagulability workup was negative.

The patient called and was examined two days later due to intractable nausea and vomiting. His vision at this visit was barely light perception. The IOP in the right eye was 37 mm Hg by applanation. The choroidal hemorrhages were worse on examination and on ultrasonography (Figure 2). The anterior chamber was shallowing as well.

Figure 2. Two days later, the choroidal hemorrhages were worse on examination and on ultrasonography.

The patient was admitted to the hospital for pain control in addition to hydration and was discharged two days later with follow-up to drain the choroidal hemorrhages. Per a family request, the patient was referred to a retinal surgeon in a different community. This surgeon drained the hemorrhagic choroidals and did not perform any cytology.

Two days after surgery, the patient presented to the ER again with headaches and continued nausea. He also reported feeling a lump in his neck forming over the past several days. A chest X-ray showed multiple pulmonary masses. CT of the chest, abdomen and pelvis showed bilateral pulmonary nodules, hilar and mediastinal adenopathy, and multiple nodules involving the liver. Re peat CT of the head showed a questionable subtle area of enhancement involving the subcortical white matter of the right frontal lobe.

A repeat CT a day later was normal, save for highly dense material within the right globe. Fine-needle aspiration of the neck mass showed metastatic carcinoma consistent with choroicarcinoma. A source was subsequently found with testicular ultrasound. Metastatic testicular cancer was diagnosed, and chemotherapy with bleomycin/etoposide/cisplatin began. After his first cycle, patient was switched to VIP chemotherapy.

The same day that the chemotherapy was started, the patient's eye became severely proptotic. The patient's vision fell to no light perception, and the IOP was elevated into the high 20s. Ultrasonography showed diffuse vitreous hemorrhage. MRI of the orbits, with and without contrast, was performed, given the proptotic nature of the eye.

Interestingly, there were no signs of a carotid-cavernous fistula, the superior ophthalmic vein was normal and visible, and there were no signs of retrobulbar mass or hemorrhage. Given the poor prognosis and lack of retrobulbar hemorrhage on MRI, cantholysis and canthotomy were not performed. The eye was monitored for pain control, while the patient started systemic treatment in the hospital.

The patient remained relatively pain-free for the next several weeks. Within eight weeks of the eye becoming proptotic, the eye rapidly became phthisical and was eventually enucleated. Pathology of the eye and biopsy of the lacrimal gland failed to show any signs of neoplasm or sympathetic ophthalmia. The patient is still actively undergoing treatment for his systemic disease.

DISCUSSION

A Pubmed search for metastatic testicular cancer was performed and revealed 18 cases involving choroidal metastases from testicular choriocarcinoma and one from mediastinal choriocarcinoma.

Metastasis to the uvea is the most common intraocular malignancy.^1-3 In a large series of uveal metastases, the choroid was involved 88% of the time, followed by the iris (9%) and the ciliary body (2%).⁴ In most cases of uveal metastases, the breast or lung cancer is the primary site.⁴ Breast cancer in women is most frequently responsible for uveal metastases, while lung cancer is most often the primary tumor in men.

Most metastases to the uvea are asymptomatic and located between the macula and equator.^3,4 If symptoms are present, blurry vision is most frequently reported, likely due to foveal involvement of the tumor or subretinal fluid as a byproduct of the tumor affecting the fovea.3 Contrary to the majority of uveal metastases, choriocarcinoma metastases to the choroid are unlikely to be asymptomatic.

Choriocarcinoma metastases to the choroid involve significant intraocular hemorrhages 82% to 85% of the time.^5,6 In one meta-analysis of 16 cases of choriocarinoma metastases to the choroid, approximately half of the patients had visual changes as one of their primary symptoms prior to the diagnosis of choriocarcinoma.⁵

Ideally, the original choroidal drainage procedure would have included cytology for confirmation in this case. That the pathology report postenucleation was negative is not surprising, considering the patient had already received chemotherapy for his testicular cancer. An initial rapid response to chemotherapy for choriocarcinoma is common. Although pathology did not confirm metastasis to the eye or orbit, it is highly likely that the patient's metastatic disease is responsible for the ocular presentations.

Rapid progression of hemorrhage, proptosis, and eventual phthisis would be uncommon for a spontaneous choroidal hemorrhage, and the conclusion that underlying testicular cancer is responsible for the rapid deterioration is warranted. RP

REFERENCES

1. Bloch RS, Gartner S. The incidence of ocular metastatic carcinoma. Arch Ophthalmol. 1971; 85:673-675.
2. Shields JA. Metastatic tumors to the uvea. Int Ophthalmol Clin. 1993; 33:155-161.
3. Giuliari GP, Sadaka A. Uveal metastatic disease: Current and new treatment options. Oncol Reports. 2012; 27:603-607.
4. Shields CL, Shields JA, Gross NE, et al. Survey of 520 eyes with uveal metastases. Ophthalmology. 1997; 104:1265-1276.
5. Kavanagh MC, Pakala SR, Hollander DA, O'Brien JM. Choriocarcinoma metastatic to the choroid. Br J Ophthalmol. 2006; 90:650-652.
6. Guber I, Zografos L, Schalenbourg A. Choroidal metastases in testicular choriocarcinoma, successful treatment with chemo- and radiotherapy: a case report. BMC Urol. 2011;11:24.