Abstract
Background
To present a case of melanoma-associated retinopathy (MAR) which manifested 26 months prior to a formal diagnosis of melanoma.
Methods
Case report.
Results
A 72-year-old female presented with bilateral continuous photopsia consistent with MAR of 7-months duration. At this point, visual function appeared normal with the exception of mildly impaired colour vision (10/17 Ishihara plates). The flash electroretinographic (ERG) revealed extinguished rod responses, a normal a-wave and reduced b-wave (electronegative ERG) on the maximal combined response, absent oscillatory potentials and broadened a-wave trough on the cone response. Multifocal ERG (mfERG) responses were delayed and demonstrated atypical morphology. Nineteen months after the initial presentation, her visual symptoms had progressed significantly with constant debilitating photopsia in combination with 13 kg weight loss. Biopsy of a now evident left axillary mass demonstrated a metastatic high-grade malignant melanoma. No primary was detected, and an axillary lymph node clearance was undertaken. Subsequently, visual symptoms resolved with corresponding improvement in the ERG over the next 18 months. Rod responses recovered such that the amplitude was at the lower limit of normal and the mfERG response delay lessened. Unfortunately, the melanoma recurred and the patient passed away 6 months later. Visual symptoms did not recur.
Conclusion
We present a case which demonstrates MAR may precede the formal diagnosis of melanoma by up to 26 months. The potential for improvement in the rod visual function persists over a period of years with normalisation of an electronegative waveform. In this case, cytoreductive surgery resulted in complete resolution of the MAR, which did not return even with a recurrence of the tumour.
References
Heckenlively JR, Ferreyra HA (2008) Autoimmune retinopathy: a review and summary. Semin Immunopathol 30(2):127–134
Audo I, Robson AG, Holder GE, Moore AT (2008) The negative ERG: clinical phenotypes and disease mechanisms of inner retinal dysfunction. Surv Ophthalmol 53(1):16–40
Keltner JL, Thirkill CE, Yip PT (2001) Clinical and immunologic characteristics of melanoma-associated retinopathy syndrome: eleven new cases and a review of 51 previously published cases. J Neuroophthalmol 21(3):173–187
Rappoport D, Leiba H (2012) Presumed melanoma-associated retinopathy (MAR): a presenting sign of primary small intestinal melanoma? Int Ophthalmol 32(4):387–391
Machida S, Ohguro H, Tateda M, Sato H, Kurosaka D (2011) Melanoma-associated retinopathy associated with intranasal melanoma. Doc Ophthalmol 122(3):191–197
Powell SF, Dudek AZ (2010) Treatment of melanoma-associated retinopathy. Curr Treat Options Neurol 12(1):54–63
Yamamoto S, Hanaya J, Mera K, Miyata K (2012) Recovery of visual function in patient with melanoma-associated retinopathy treated with surgical resection and interferon-beta. Doc Ophthalmol. PMID 22302292 [Epub ahead of print]
Marmor MF, Holder GE, Seeliger MW, Yamamoto S (2004) Standard for clinical electroretinography. Doc Ophthalmol 108:107–114
Marmor MF, Hood DC, Keating D, Kondo M, Seeliger MW, Miyake Y (2003) Guidelines for basic multifocal electroretinography (mfERG). Doc Ophthalmol 106:105–115
Lu Y, Jia L, He S, Hurley MC, Leys MJ, Jayasundera T, Heckenlively JR (2009) Melanoma-associated retinopathy: a paraneoplastic autoimmune complication. Arch Ophthalmol 127(12):1572–1580
MacKay CJ (1991) Retinitis pigmentosa misdiagnosed as complicated migraine. Arch Ophthalmol 109(2):174–175
DuBois L, Sadun AA, Lawton TB (1988) Inner retinal layer loss in complicated migraine. Arch Ophthalmol 106(8):1035–1037
Kiratli H, Thirkill CE, Bilgiç S, Eldem B, Keçeci A (1997) Paraneoplastic retinopathy associated with metastatic cutaneous melanoma of unknown primary site. Eye (Lond) 11(Pt 6):889–892
Hoffmann LA, Jarius S, Pellkofer HL, Schueller M, Krumbholz M, Koenig F, Johannis W, La Fougere C, Newman T, Vincent A, Voltz R (2008) Anti-Ma and anti-Ta associated paraneoplastic neurological syndromes: 22 newly diagnosed patients and review of previous cases. J Neurol Neurosurg Psychiatry 79(7):767–773
Darnell RB, Posner JB (2011) Paraneoplastic antinuclear and antinucleolar antibodies. In: Darnell RB, Posner JB (eds) Paraneoplastic syndromes, Ch 11, 1st edn. Oxford University Press, Oxford, p 329
Polans AS, Witkowska D, Haley TL, Amundson D, Baizer L, Adamus G (1995) Recoverin, a photoreceptor-specific calcium-binding protein, is expressed by the tumor of a patient with cancer-associated retinopathy. Proc Natl Acad Sci USA 92(20):9176–9180
Bazhin AV, Dalke C, Willner N, Abschütz O, Wildberger HG, Philippov PP, Dummer R, Graw J, de Angelis MH, Schadendorf D, Umansky V, Eichmüller SB (2009) Cancer-retina antigens as potential paraneoplastic antigens in melanoma-associated retinopathy. Int J Cancer 124(1):140–149
Chan C, O’Day J (2001) Melanoma-associated retinopathy: does autoimmunity prolong survival? Clin Exp Ophthalmol 29(4):235–238
Zhou G, Levitsky H (2012) Towards curative cancer immunotherapy: overcoming posttherapy tumor escape. Clin Dev Immunol 2012:124187
Remulla JF, Pineda R, Gaudio AR, Milam AH (1995) Cutaneous melanoma-associated retinopathy with retinal periphlebitis. Arch Ophthalmol 113(7):854–855
Jacobzone C, Cochard-Marianowski C, Kupfer I, Bettembourg S, Dordain Y, Misery L, Cochener B, Sassolas B (2004) Corticosteroid treatment for melanoma-associated retinopathy: effect on visual acuity and electrophysiologic findings. Arch Dermatol 140(10):1258–1261
Subhadra C, Dudek AZ, Rath PP, Lee MS (2008) Improvement in visual fields in a patient with melanoma-associated retinopathy treated with intravenous immunoglobulin. J Neuroophthalmol 28(1):23–26
Kim LS, Alexander KR, Fishman GA (2008) Spontaneous improvement of rod system function in a patient with melanoma-associated retinopathy. Retin Cases Brief Rep 2(2):166–171
Weleber RG, Watzke RC, Shults WT, Trzupek KM, Heckenlively JR, Egan RA, Adamus G (2005) Clinical and electrophysiologic characterization of paraneoplastic and autoimmune retinopathies associated with antienolase antibodies. Am J Ophthalmol 139(5):780–794
Hood DC, Frishman LJ, Saszik S, Viswanathan S (2002) Retinal origins of the primate multifocal ERG: implications for the human response. Invest Ophthalmol Vis Sci 43(5):1673–1685
Sen P, Roy R, Maru S, Ravi P (2010) Evaluation of focal retinal function using multifocal electroretinography in patients with X-linked retinoschisis. Can J Ophthalmol 45(5):509–513
Kondo M, Miyake Y, Kondo N, Tanikawa A, Suzuki S, Horiguchi M, Terasaki H (2001) Multifocal ERG findings in complete type congenital stationary night blindness. Invest Ophthalmol Vis Sci 42(6):1342–1348
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Stead, R.E., Fox, M.A., Staples, E. et al. Delayed presentation of melanoma-associated retinopathy and subsequent resolution with cytoreduction surgery. Doc Ophthalmol 127, 165–171 (2013). https://doi.org/10.1007/s10633-013-9398-6
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DOI: https://doi.org/10.1007/s10633-013-9398-6