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| CASE REPORT |
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| Ahead of print publication |
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Metastatic esophageal carcinoma to the eye masquerading as inflammatory vitreo-retinitis: A case report and literature review
Reham Shehada1, Rim Turfa2, Ahmad K H Ibrahimi3, Jakub Khzouz4, Azza Gharaibeh5, Yacoub A Yousef1, Mona Mohammad1
1 Department of Surgery/Ophthalmology, King Hussein Cancer Center, Amman, Jordan
2 Department of Medical Oncology, King Hussein Cancer Center, Amman, Jordan
3 Department of Radiation Oncology, King Hussein Cancer Center, Amman, Jordan
4 Department of Pathology, King Hussein Cancer Center, Amman, Jordan
5 Department of Diagnostic Radiology, King Hussein Cancer Center, Amman, Jordan
| Date of Submission | 10-May-2022 |
| Date of Acceptance | 03-Jul-2022 |
| Date of Web Publication | 25-Aug-2022 |
Correspondence Address:
Mona Mohammad,
Department of Surgery/Ophthalmology, King Hussein Cancer Center, Queen Rania Al Abdullah Street, P. O. Box: 1269, Amman 11941
Jordan
 Source of Support: None, Conflict of Interest: None DOI: 10.4103/2211-5056.354537
We report an unusual case of metastatic esophageal carcinoma to the vitreous associated with focal retinitis in a 44-year-old male. A 44-year-old male patient, known case of locally advanced esophageal carcinoma, presented with a 3-day history of left eye floaters. The initial diagnosis was inflammatory vitreo-retinitis that responded to systemic steroids. Four months later, the patient presented with relapsed intraocular inflammation, vitreous biopsy using a 25G needle was performed, and cytology confirmed the presence of metastatic carcinoma consistent with his primary esophageal cancer. The patient received external beam radiotherapy to his ocular and brain metastasis, and his eye examination was stable. Five months later, the patient passed away due to progressive brain metastasis.
Keywords: Carcinoma, metastasis, retina, vitreous, vitritis
How to cite this URL:
Shehada R, Turfa R, Ibrahimi AK, Khzouz J, Gharaibeh A, Yousef YA, Mohammad M. Metastatic esophageal carcinoma to the eye masquerading as inflammatory vitreo-retinitis: A case report and literature review. Taiwan J Ophthalmol [Epub ahead of print] [cited 2023 Mar 23]. Available from: https://www.e-tjo.org/preprintarticle.asp?id=354537 |
| Introduction | |  |
The most common intraocular cancer in adults is metastasis,[1] and the most common primary cancer sites for metastasis in males and females are lung and breast cancer, respectively.[2] The gastrointestinal tract is an uncommon site of origin for intraocular metastasis; moreover, vitreous and retinal metastases from gastrointestinal tract cancers are extremely rare.[1] Metastases to the intraocular structures are commonly detected in the choroid (88%), followed by the iris (9%), ciliary body (2%), and rarely in the retina (<1%).[1],[3] In view of the fact that retinal metastases caused by systemic cancers are very rare, only some case reports have been documented.[4] Herein, we are reporting on a unique case of metastatic carcinoma to the retina with vitreous seeding originating from a primary esophageal carcinoma. We obtained consent from the patients to use his data.
| Case Report | | |
In September 2021, a 44-year-old male with a history of moderately differentiated esophageal adenocarcinoma presented to the eye clinic with a 3-day history of left eye floaters. Twenty-nine months before presentation, he had been diagnosed with locally advanced adenocarcinoma of the esophagus and underwent neoadjuvant chemoradiotherapy, followed by surgical resection of yPT3N0 residual tumor. On physical examination, corrected vision was 6/6 for both eyes, the right eye was entirely normal, while the left eye examination showed normal anterior segment, grade-1 vitritis with focal area of localized periphlebitis located superonasal around the equator [Figure 1]a and [Figure 1]b. Serology for toxoplasmosis, cytomegalovirus, and herpes simplex virus was all negative. Brain and orbit magnetic resonance image (MRI) scans were also normal. The patient was evaluated in the rheumatology clinic for systemic diseases such as sarcoidosis and Behjet disease and was all negative as well. On the basis of the clinical findings, idiopathic inflammatory retinal vasculitis was initially suspected. As a result, our patient was treated with oral steroids, and on follow-up assessment, there was regression of his intraocular inflammation. His eye condition maintained ongoing stability for 3 months, during which he was on palliative chemotherapy for progressive systemic lung metastasis. | Figure 1: Left eye fundus and B-scan ultrasound photos: at first presentation showing focal retinitis with periphlebitis (arrow) (a), mild vitritis on B scan (b). Photos after 4 months showing progression in the area of retinitis with increase in perivascular infiltrates (arrow) (c), denser vitritis on B-scan (d), hazy fundus view due to vitreous clouds (e), and vitreous condensations in the inferior retina (arrow) (f)
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The patient stopped systemic chemotherapy, 3 weeks later, he presented with a significant drop in corrected vision in the left eye (6/60), dilated fundus examination revealed new-onset severe vitritis forming vitreous clouds, and dense white-to-yellow vitreous clumps in the inferior retina, the old superonasal periphlebitis showed new activity with more perivascular infiltrates [Figure 1]c, [Figure 1]d, [Figure 1]e, [Figure 1]f. Vitreous biopsy using a 25-gauge needle was performed, and cytology confirmed the presence of metastatic carcinoma consistent with his primary esophageal cancer [Figure 2]. We requested new brain and orbit MRI scans which showed new tiny contrast-enhancing right and left frontal subcortical white matter lesions suggestive of new brain metastasis [Figure 3]. Given the fact that the patient was in good general health, and to control his local metastasis, our patient received palliative local radiotherapy to the left eye and frontal lesions and was also started on systemic chemotherapy. | Figure 2: Light microscopy (H and E, ×40), confirmed the presence of metastatic carcinoma consistent with his primary esophageal cancer, which shows: (a) Cluster of tumor cell with hyperchromatic nuclei. (b) Tumor cell nuclei stain positive for CDX2 immunostain. (c) Signet ring cell. (d) Signet ring cell-CK7 immunostain. This is an epithelial marker
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 | Figure 3: Brain MRI scan, axial postcontrast T1-weighted images show small rim-enhancing metastatic lesion in the left frontal region (a) and tiny enhancing lesion in the subcortical white matter in the right frontal region (b). MRI = Magnetic resonance image
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In terms of local radiotherapy, the patient underwent computed topography (CT) simulation under a thermoplastic head mask with a 2-mm slice thickness. For the vitreous metastasis, the patient received 3750 centi-Gray (cGy) in 15 daily fractions over 3 weeks through three-dimensional conformal radiotherapy through a linear accelerator [Figure 4]. There was no gross tumor volume defined. The posterior eye globe from the lens and behind was considered the clinical target volume to account for all areas of microscopic spread. A 3-mm margin was added to create the planning target volume. The patient also underwent a second CT-simulation with Brainlab© dual mask with 1-mm slice thickness, and received stereotactic radiosurgery through Linac-based Novalis Brainlab© system as 2200 cGy in a single fraction to his two brain metastases. | Figure 4: 3dcrt radiotherapy plan, the thick green line represents the 95% of the prescription dose which encompassed at least. 95% of the PTV (the blue color wash). 3dcrt = Three dimensional conformal radiotherapy, PTV = Planning target volume
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The patient finished his radiation treatment course smoothly, he was doing well apart from mild eye dryness for which he was prescribed lubricating eye drops. At the last follow-up examination after 4 months, his vision in the left eye was stable (6/60), with stable vitreous seeds and inactive retinal lesions. This was based on dilated fundus examination assessment, but owing to the unstable patient condition at that time, we were not able to take fundus photos. Five months later, he passed away due to disease progression from brain metastasis.
| Discussion | | |
While metastatic carcinoma to the uveal tract is well identified in the literature,[1] metastasis to the retina or vitreous is quite rare with only few reported cases.[4],[5] Vitreous seeding of retinal tumor cells may be associated with different intraocular tumors such as metastatic cutaneous melanoma, retinoblastoma, large-cell lymphoma, retinal pigment epithelioma, melanocytoma, and ciliochoroidal melanoma. Of the primary tumors that demonstrated retinal metastasis, cutaneous melanoma is considered the most common one.[4] On the other hand, intraocular metastasis from the gastrointestinal tract is rare,[1] with common primary sites include the colon, ileum and rectum, and stomach.[4] In this case, a 44-year-old male had esophageal cancer that metastasized to the vitreous and retina, this was suspected clinically and confirmed by cytology. To the best of our knowledge, there are two other reported cases of metastatic esophageal cancer to the retina.[4],[6] The first case is for a 56-year-old male patient who presented at Wills Eye Hospital, the interval from primary cancer diagnosis to retinal metastasis was 26 months, the patient was only observed due to poor prognosis, and he died after a year.[4] The second case was described by Pal et al. for a 68-year-old patient, who presented with right eye chorioretinal metastasis 2 months following diagnosis with esophageal carcinoma; he was managed by a combination of local radiotherapy and palliative chemotherapy.[6]
Actually, the exact etiology of a tumor metastasizes to the vitreous could not be determined, but it is proposed that vitreous traction and hematogenous route through retinal circulation could play a paramount role.[7]
In the majority of cases, retinal metastasis appeared as yellow-white or brown lesions consistent with the primary source.[4] In this case, the tumor appeared yellow-to-white in color which is similar to other cases of primary gastrointestinal cancer.[4],[8]
There is no wide experience in the treatment of those cases due to its rarity. Most of the reported cases have been managed with enucleation, chemotherapy, external beam radiation, or observation.[4],[9],[10] Even though local control of intraocular metastasis is generally achieved, death usually occurs within 2 months to 2 years due to disseminated metastasis, which means that retinal metastasis appears to be detected near the terminal stage of the disease.[4],[5],[9]
In summary, retinal/vitreous metastases are uncommon, but they should be a differential diagnosis in patients with known cancers. Once diagnosed, the prognosis for life is poor, and therefore, treatment should be individualized based on the overall medical status.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
The authors declare that there are no conflicts of interests of this paper.
| References | | |
| 1. | Shields CL, Shields JA, Gross NE, Schwartz GP, Lally SE. Survey of 520 eyes with uveal metastases. Ophthalmology 1997;104:1265-76.  |
| 2. | Freedman MI, Folk JC. Metastatic tumors to the eye and orbit: Patient survival and clinical characteristics. Ophthalmic Plast Reconstr Surg 1987;105:1215-9. |
| 3. | Shields JA, Carol LS. Intraocular Tumors: An Atlas and Textbook. Philadelphia: Lippincott Williams and Wilkins; 2008. p. 198-227. |
| 4. | Shields CL, McMahon JF, Atalay HT, Hasanreisoglu M, Shields JA. Retinal metastasis from systemic cancer in 8 cases. JAMA Ophthalmol 2014;132:1303-8. |
| 5. | Leys AM, Van Eyck LM, Nuttin BJ, Pauwels PA, Delabie JM, Libert JA. Metastatic carcinoma to the retina. Clinicopathologic findings in two cases. Arch Ophthalmol 1990;108:1448-52. |
| 6. | Pal D, Bahl A, Sharma JB, Rao RR. Carcinoma esophagus: A rare primary malignancy for ocular metastasis. Indian J Cancer 2010;47:480-1. [ PUBMED] [Full text] |
| 7. | Cohen VM, Shields CL, Furuta M, Shields JA. Vitreous seeding from retinal astrocytoma in three cases. Retina 2008;28:884-8. |
| 8. | Kim CY, Ha CW, Lee SC. Vitreous and retinal metastasis from gastric cancer. Eur J Ophthalmol 2010;20:615-7. |
| 9. | Konstantinidis L, Rospond-Kubiak I, Zeolite I, Heimann H, Groenewald C, Coupland SE, et al. Management of patients with uveal metastases at the liverpool ocular oncology centre. Br J Ophthalmol 2014;98:92-8. |
| 10. | Payne JF, Rahman HT, Grossniklaus HE, Bergstrom CS. Retinal metastasis simulating cytomegalovirus retinitis. Ophthalmic Surg Lasers Imaging 2012;43:e90-3. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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