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CASE SNIPPET
Year : 2020  |  Volume : 66  |  Issue : 4  |  Page : 220-221

An unusual case of metastatic choroidal deposits of renal cell carcinoma presenting with loss of vision


Department of Pathology, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra, India

Date of Submission20-Apr-2020
Date of Decision25-Apr-2020
Date of Acceptance04-May-2020
Date of Web Publication07-Oct-2020

Correspondence Address:
A Shah
Department of Pathology, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jpgm.JPGM_378_20

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How to cite this article:
Dave V, Shah A, Menon S. An unusual case of metastatic choroidal deposits of renal cell carcinoma presenting with loss of vision. J Postgrad Med 2020;66:220-1

How to cite this URL:
Dave V, Shah A, Menon S. An unusual case of metastatic choroidal deposits of renal cell carcinoma presenting with loss of vision. J Postgrad Med [serial online] 2020 [cited 2020 Nov 28];66:220-1. Available from: https://www.jpgmonline.com/text.asp?2020/66/4/220/297498




A 71-year-old man presented in August 2018 with gradual diminution of vision in the left eye followed by complete vision loss for 4 months. The patient, a known diabetic, had undergone right radical nephrectomy in 2013 for renal cell carcinoma (RCC). On examination, there was no perception of light and projection of rays in the left eye in comparison to normal perception in the right eye. Pupillary reaction was also sluggish on the left side. Magnetic resonance imaging (MRI) showed focal thickening in the chorioretinal membrane posteriorly at the level of optic attachment which was hypointense on T2W and iso to hyperintense on T1W. B-scan of left eye showed dome-shaped choroidal lesion suspicious of choroidal melanoma. Positron emission tomography with 18F-labeled fluoro-2-deoxyglucose integrated with computed tomography (18F-FDG-PET/CT) revealed a 14 mm × 14 mm FDG avid well-defined soft-tissue lesion in the left lower lung lobe (SUV max 5.37) with multiple lesions in both lung fields, FDG avid lytic lesion in neck of the left femur (SUV max 3.37) along with multiple FDG avid mediastinal lymph nodes (SUV max 6.61). There was no FDG uptake in the eyes. Patient underwent myo-conjunctival enucleation and the specimen was sent for histopathological examination. Gross evaluation revealed a 1.8 cm × 1.2 cm × 0.6 cm whitish firm tumor in the inferotemporal quadrant of the globe. The tumor had an endophytic growth pattern. On microscopy, the tumor was arranged in an organoid pattern with polygonal cells having clear cytoplasm and prominent nucleolus on the choroid in the posterior segment (PS) of the left eye [Figure 1]a, [Figure 1]b and [Figure 2]a. The morphological diagnosis was of a metastatic conventional/clear RCC. On immunohistochemistry (IHC), the tumor showed sparse weak positivity for Paired box gene 8 (PAX8) [Figure 2]b, strong positivity for Cluster of Differentiation 10 (CD10) [Figure 2]c and was negative for Cytokeratin 7 (CK7), S100 [Figure 2]d, and Human Melanoma Black 45 (HMB45), thus confirming a metastatic RCC and ruling out a malignant melanoma. Patient was later started on multitargeted receptor tyrosine kinase inhibitor, Sunitinib.
Figure 1: (a) Metastatic renal cell carcinoma in the posterior segment (PS) of the eye (H and E, ×10), [C, cornea; AC, Anterior chamber; P, Posterior chamber; L, Lens; CB, Ciliary body; AS, Anterior segment; VC, Vitreous chamber; T, Tumor; PS, Posterior segment] (b) Tumor cells are arranged in an organoid to solid pattern (H and E, ×100) [T, tumor; Ch, choroid; S, sclera]

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Figure 2: (a) Tumor cells are composed of clear to eosinophilic cytoplasm with prominent nucleolus (H and E, ×200). (b) Tumor shows sparse weak positivity for PAX8 (IHC, ×100). (c) Tumor shows strong positivity for CD10 (IHC, ×200). (d) Tumor is negative for S100 (IHC, ×100)

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Choroid, by virtue of its rich vascular supply, has the risk of harboring distant metastases, which constitute the most common intraocular malignancy in adults.[1] It is generally a late manifestation of widespread disease and connotes poor prognosis. It can also represent as the primary manifestation of an unknown malignancy.[1] Choroidal metastasis from breast and lung are relatively more common than from the gastrointestinal tract, prostate, kidney, and skin.[1],[2] Shields et al. performed a retrospective chart review of 950 uveal metastasis in 420 patients for 20 years and found that the mean age at diagnosis of ocular metastasis was 58 years with choroid as the most common site of uveal metastasis.[2] However, in their study, only eight cases of metastasis to the choroid were from a renal primary.[2] In a review of case reports by Sountoulides et al., they studied 19 cases of ocular metastasis of RCC; the interval between nephrectomy and ocular metastases ranged from 1 month to 17 years in 10 cases.[3] The features of choroidal metastasis can be very similar to primary melanoma on MRI and therefore obtaining a correct history is of utmost value.[4] On microscopic examination, uveal melanomas are either spindle cell or epithelioid cell type. Epithelioid cell type of melanomas may mimic metastatic poorly differentiated carcinoma.[4] Lineage-specific markers such as CK7, estrogen receptor (ER), progesterone receptor (PR), GATA3 for breast, thyroid transcription factor-1 (TTF-1), Napsin A for lung, CDX2 for colon, PAX8 for kidney, and thyroglobulin for thyroid are helpful for a conclusive diagnosis.[5] Melanoma, however, is positive for melanocytic markers such as HMB-45 and S-100.[5] Following enucleation, specific targeted therapies are given for metastatic RCC, whereas immunotherapy, chemotherapy, or targeted therapies are the standard lines of management in melanomas.[6] The value of correct diagnosis for an optimum management is crucial. This patient presented with choroidal metastasis from a renal primary which is a very rare and unusual occurrence.

Declaration of patient consent

The authors certify that appropriate patient consent was obtained.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 :: References Top

1.
Arepalli S, Kaliki S, Shields CL. Choroidal metastases: Origin, features, and therapy. Indian J Ophthalmol 2015;63:122-7.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Shields CL, Shields JA, Gross NE, Schwartz GP, Lally SE. Survey of 520 eyes with uveal metastases. Ophthalmology 1997;104:1265-76.  Back to cited text no. 2
    
3.
Sountoulides P, Metaxa L, Cindolo L. Atypical presentations and rare metastatic sites of renal cell carcinoma: A review of case reports. J Med Case Rep 2011;5:429-37.  Back to cited text no. 3
    
4.
Houle V, Bélair M, Allaire GS. AIRP best cases in radiologic-pathologic correlation: Choroidal melanoma. Radiographics 2011;31:1231-6.  Back to cited text no. 4
    
5.
Selves J, Long-Mira E, Mathieu MC, Rochaix P, Ilié M. Immunohistochemistry for diagnosis of metastatic carcinomas of unknown primary site. Cancers (Basel) 2018;10:108-31.  Back to cited text no. 5
    
6.
Yang J, Manson DK, Marr BP, Carvajal RD. Treatment of uveal melanoma: Where are we now? Ther Adv Med Oncol 2018;10:1-17.  Back to cited text no. 6
    


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