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A case of multifocal presumed solitary circumscribed retinal astrocytic proliferation lesions in the same eye

Published:October 27, 2020DOI:https://doi.org/10.1016/j.jcjo.2020.10.001
      Presumed solitary circumscribed retinal astrocytic proliferation (PSCRAP) is a recently described retinal tumour that is a distinct entity from other white lesions of the retina such as astrocytic hamartoma and acquired astrocytoma.
      • Shields J.A.
      • Bianciotto C.G.
      • Kivela T.
      • Shields C.L.
      Presumed solitary circumscribed retinal astrocytic proliferation: The 2010 Jonathan W. Wirtschafter Lecture.
      The exact origin of these lesions is of some debate within the literature as no histological diagnosis exists to date.
      • Shields C.L.
      • Roe R.
      • Yannuzzi L.A.
      • Shields J.A.
      Solitary circumscribed “pearl white” retinal mass (so-called retinal astrocytic proliferation) resides in deep retina or beneath retina: Findings on multimodal imaging in 4.
      ,
      • Goldberg R.A.
      • Raja K.M.
      Presumed solitary circumscribed retinal astrocytic proliferation in the fovea with OCT angiography: A misnomer.
      These lesions are not associated with tuberous sclerosis complex or related syndromes and can be distinguished from astrocytic hamartomas with imaging.
      • Schwartz S.G.
      • Harbour J.W.
      Spectral-domain optical coherence tomography of presumed solitary circumscribed retinal astrocytic proliferation versus astrocytic hamartoma.
      They have been reported in middle-aged patients and do not appear to be congenital in nature and are not associated with features of any systemic disease.
      • Shields J.A.
      • Bianciotto C.G.
      • Kivela T.
      • Shields C.L.
      Presumed solitary circumscribed retinal astrocytic proliferation: The 2010 Jonathan W. Wirtschafter Lecture.
      ,
      • Shields C.L.
      • Roe R.
      • Yannuzzi L.A.
      • Shields J.A.
      Solitary circumscribed “pearl white” retinal mass (so-called retinal astrocytic proliferation) resides in deep retina or beneath retina: Findings on multimodal imaging in 4.
      In the majority of the reported cases, visual acuity is maintained at better than 20/50; however, there is one report of a PSCRAP lesion involving the fovea.
      • Goldberg R.A.
      • Raja K.M.
      Presumed solitary circumscribed retinal astrocytic proliferation in the fovea with OCT angiography: A misnomer.
      With foveal involvement, the patient reported a gradual change in vision in adulthood, supporting the hypothesis that these lesions are acquired rather than congenital. This unique case reports a patient with multifocal PSCRAP lesions in 1 eye, whereas PSCRAP lesions have been previously reported only as solitary.
      A 33-year-old, asymptomatic man with no personal or family history of tuberous sclerosis or neurofibromatosis was referred for evaluation of 2 pearly-white concretions in the retina of the left eye with no associated subretinal fluid, hemorrhage, or traction. His medical history was significant for asthma and type 2 diabetes mellitus. Visual acuity at presentation was 20/25 OD and 20/30 OS. Anterior segment was unremarkable. Dilated fundus examination (Fig. 1A, B) revealed a normal-appearing optic disc with 2 peripapillary pearl white retinal lesions, one temporal to the optic disc measuring approximately 1.0–1.2 mm in size and another small, white lesion superotemporal to the optic disc measuring approximately 0.5 mm in size (Fig. 1A). There was retinal pigment epithelial hyperplasia adjacent to or surrounding the lesions (Fig. 1B). Fundus autofluorescence demonstrated moderate hyperautofluorescence of both lesions (Fig. 1C). Optical coherence tomography–angiography (OCTA; Zeiss Cirrus 5000, Carl Zeiss, North York, Ont.) demonstrated the presence of an outer intraretinal mass deep to the overlying retinal vasculature (Fig. 1D). The superficial retinal vascular plexus appeared intact with no intrinsic vascularity. The vasculature, similar to the neuroretinal layers overlying the lesion, appeared compressed forward. Spectral domain optical coherence tomography (OCT) (SD-OCT; Zeiss Cirrus 5000) demonstrated a hyper-reflective, intraretinal mass with an abrupt elevation, optical shadowing, and a smooth surface with draping of the overlying retinal tissue (Fig. 1E, F). The lesions appeared to be originating from the outer retina or retinal pigment epithelium (RPE) with no underlying subretinal fluid. This was more apparent in Figure 1F as the lesion was much smaller, clearly demonstrating the preserved inner retinal layers. B-scan ultrasonography demonstrated absence of calcium in both lesions. The lesions remained stable with no changes after 6 months of follow-up.
      Fig 1
      Fig. 1(A) Colour fundus photograph of the right eye, demonstrating the position of the 2 white retinal lesions. (B) Higher magnification of the posterior pole of the right eye, highlighting the 2 distinct presumed solitary circumscribed retinal astrocytic proliferation (PSCRAP) lesions. (C) Fundus autofluorescence of the right eye; note the low-moderate hyperautofluorescence of the larger lesion and isoautofluorescence of the smaller lesion in the right eye. (D) Optical coherence tomography–angiography (OCTA) analysis of the superficial retinal vasculature of the right eye. Spectral domain OCT analysis of the right eye demonstrating the larger lesion (E) and the smaller lesion (F).
      PSCRAP was first described by Shields et al. in 2011.
      • Shields J.A.
      • Bianciotto C.G.
      • Kivela T.
      • Shields C.L.
      Presumed solitary circumscribed retinal astrocytic proliferation: The 2010 Jonathan W. Wirtschafter Lecture.
      These pearl-white lesions are rare benign retinal tumours that are discrete entities from other white retinal lesions and have unique and consistent characteristics on multimodal imaging.
      • Shields J.A.
      • Bianciotto C.G.
      • Kivela T.
      • Shields C.L.
      Presumed solitary circumscribed retinal astrocytic proliferation: The 2010 Jonathan W. Wirtschafter Lecture.
      ,
      • Shields C.L.
      • Roe R.
      • Yannuzzi L.A.
      • Shields J.A.
      Solitary circumscribed “pearl white” retinal mass (so-called retinal astrocytic proliferation) resides in deep retina or beneath retina: Findings on multimodal imaging in 4.
      ,
      • Goldberg R.A.
      • Raja K.M.
      Presumed solitary circumscribed retinal astrocytic proliferation in the fovea with OCT angiography: A misnomer.
      All lesions reported tend to remain stable over long-term follow-up aside from 2 cases that spontaneously resolved.
      • Shields J.A.
      • Bianciotto C.G.
      • Kivela T.
      • Shields C.L.
      Presumed solitary circumscribed retinal astrocytic proliferation: The 2010 Jonathan W. Wirtschafter Lecture.
      ,
      • Asensio-Sánchez V.M.
      Presumed solitary circumscribed retinal astrocytic proliferation: a lesion that can regress.
      Table 1 summarizes all published cases of PSCRAP and the corresponding characteristics of these lesions. PSCRAP can be distinguished from similar-appearing lesions such as astrocytic hamartomas by the appearance on OCT.
      • Shields C.L.
      • Roe R.
      • Yannuzzi L.A.
      • Shields J.A.
      Solitary circumscribed “pearl white” retinal mass (so-called retinal astrocytic proliferation) resides in deep retina or beneath retina: Findings on multimodal imaging in 4.
      ,
      • Schwartz S.G.
      • Harbour J.W.
      Spectral-domain optical coherence tomography of presumed solitary circumscribed retinal astrocytic proliferation versus astrocytic hamartoma.
      ,
      • Goel N.
      • Pangtey B.
      • Bhushan G.
      • Raina U.K.
      • Ghosh B.
      Spectral-domain optical coherence tomography of astrocytic hamartomas in tuberous sclerosis.
      SD-OCT analysis in several recently published cases demonstrates the discrete separation of PSCRAP lesions from the overlying retinal nerve fibre layer (RNFL).
      • Shields C.L.
      • Roe R.
      • Yannuzzi L.A.
      • Shields J.A.
      Solitary circumscribed “pearl white” retinal mass (so-called retinal astrocytic proliferation) resides in deep retina or beneath retina: Findings on multimodal imaging in 4.
      • Goldberg R.A.
      • Raja K.M.
      Presumed solitary circumscribed retinal astrocytic proliferation in the fovea with OCT angiography: A misnomer.
      • Schwartz S.G.
      • Harbour J.W.
      Spectral-domain optical coherence tomography of presumed solitary circumscribed retinal astrocytic proliferation versus astrocytic hamartoma.
      This is in contrast to OCT analysis of astrocytic hamartomas, which show an elevated or dome-shaped appearance confined to the RNFL, which is hyper-reflective, with subtle posterior shadowing of the deeper retinal structures and a normal RPE.
      • Goel N.
      • Pangtey B.
      • Bhushan G.
      • Raina U.K.
      • Ghosh B.
      Spectral-domain optical coherence tomography of astrocytic hamartomas in tuberous sclerosis.
      Furthermore, PSCRAP is not associated with extension or adhesions into the vitreous like those reported with astrocytic hamartomas.
      • Goel N.
      • Pangtey B.
      • Bhushan G.
      • Raina U.K.
      • Ghosh B.
      Spectral-domain optical coherence tomography of astrocytic hamartomas in tuberous sclerosis.
      In addition, OCTA in the present case demonstrated a normal retinal vasculature of the surrounding superficial plexus as well as no intrinsic vascularity within the PSCRAP lesions. This is consistent with intravenous fluorescein angiography and OCTA in previous reports.
      • Shields J.A.
      • Bianciotto C.G.
      • Kivela T.
      • Shields C.L.
      Presumed solitary circumscribed retinal astrocytic proliferation: The 2010 Jonathan W. Wirtschafter Lecture.
      ,
      • Goldberg R.A.
      • Raja K.M.
      Presumed solitary circumscribed retinal astrocytic proliferation in the fovea with OCT angiography: A misnomer.
      In contrast, astrocytic hamartomas demonstrate an intrinsic blood supply.
      • Asensio-Sánchez V.M.
      Presumed solitary circumscribed retinal astrocytic proliferation: a lesion that can regress.
      Therefore, OCTA analysis is a noninvasive method to further delineate PSCRAP from other white retinal lesions.
      Table 1Summary of all published reports of presumed solitary circumscribed retinal astrocytic proliferation lesions, including the present multifocal case
      Age (y), SexVisual AcuityPresenting ComplaintAnatomical LocationStabilityReference
      33, M20/25NilMultifocal: juxtapapillary and superotemporal to discStablePresent study
      46, M20/20NilTemporal equatorStableShields et al. (2011)
      • Shields J.A.
      • Bianciotto C.G.
      • Kivela T.
      • Shields C.L.
      Presumed solitary circumscribed retinal astrocytic proliferation: The 2010 Jonathan W. Wirtschafter Lecture.
      53, M20/30Decrease vision/cataractInferotemporal, postequatorialStableShields et al. (2011)
      • Shields J.A.
      • Bianciotto C.G.
      • Kivela T.
      • Shields C.L.
      Presumed solitary circumscribed retinal astrocytic proliferation: The 2010 Jonathan W. Wirtschafter Lecture.
      37, M20/20NilJuxtapapillaryStableShields et al. (2011)
      • Shields J.A.
      • Bianciotto C.G.
      • Kivela T.
      • Shields C.L.
      Presumed solitary circumscribed retinal astrocytic proliferation: The 2010 Jonathan W. Wirtschafter Lecture.
      43, M20/20NilSuperior equatorStableShields et al. (2011)
      • Shields J.A.
      • Bianciotto C.G.
      • Kivela T.
      • Shields C.L.
      Presumed solitary circumscribed retinal astrocytic proliferation: The 2010 Jonathan W. Wirtschafter Lecture.
      78, M20/30Decrease vision/cataractNasal equatorStableShields et al. (2011)
      • Shields J.A.
      • Bianciotto C.G.
      • Kivela T.
      • Shields C.L.
      Presumed solitary circumscribed retinal astrocytic proliferation: The 2010 Jonathan W. Wirtschafter Lecture.
      85, F20/20NilSuperior to discStableShields et al. (2011)
      • Shields J.A.
      • Bianciotto C.G.
      • Kivela T.
      • Shields C.L.
      Presumed solitary circumscribed retinal astrocytic proliferation: The 2010 Jonathan W. Wirtschafter Lecture.
      76, F20/25NilInferior to discRegressionShields et al. (2011)
      • Shields J.A.
      • Bianciotto C.G.
      • Kivela T.
      • Shields C.L.
      Presumed solitary circumscribed retinal astrocytic proliferation: The 2010 Jonathan W. Wirtschafter Lecture.
      57, F20/20NilJuxtapapillaryNot reportedSchwartz and Harbour (2015)
      • Schwartz S.G.
      • Harbour J.W.
      Spectral-domain optical coherence tomography of presumed solitary circumscribed retinal astrocytic proliferation versus astrocytic hamartoma.
      61, M20/50NilMaculaNot reportedShields et al. (2017)
      • Shields C.L.
      • Roe R.
      • Yannuzzi L.A.
      • Shields J.A.
      Solitary circumscribed “pearl white” retinal mass (so-called retinal astrocytic proliferation) resides in deep retina or beneath retina: Findings on multimodal imaging in 4.
      75, M20/25NilNasal equatorNot reportedShields et al. (2017)
      • Shields C.L.
      • Roe R.
      • Yannuzzi L.A.
      • Shields J.A.
      Solitary circumscribed “pearl white” retinal mass (so-called retinal astrocytic proliferation) resides in deep retina or beneath retina: Findings on multimodal imaging in 4.
      75, F20/40FloatersSuperior equatorNot reportedShields et al. (2017)
      • Shields C.L.
      • Roe R.
      • Yannuzzi L.A.
      • Shields J.A.
      Solitary circumscribed “pearl white” retinal mass (so-called retinal astrocytic proliferation) resides in deep retina or beneath retina: Findings on multimodal imaging in 4.
      46, F20/20NilTemporal equatorNot reportedShields et al. (2017)
      • Shields C.L.
      • Roe R.
      • Yannuzzi L.A.
      • Shields J.A.
      Solitary circumscribed “pearl white” retinal mass (so-called retinal astrocytic proliferation) resides in deep retina or beneath retina: Findings on multimodal imaging in 4.
      58, M20/20NilInferonasal to discStableAsensio-Sánchez and Díaz-Cabanas (2017)
      • Asensio-Sánchez V.M.
      • Díaz-Cabanas L.
      Presumed solitary circumscribed retinal astrocytic proliferation.
      74, F20/250MetamorphopsiaMacularNot reportedGoldberg and Raja (2018)
      • Goldberg R.A.
      • Raja K.M.
      Presumed solitary circumscribed retinal astrocytic proliferation in the fovea with OCT angiography: A misnomer.
      56, F20/20NilJuxtapapillaryRegressionAsensio-Sánchez (2019)
      • Asensio-Sánchez V.M.
      Presumed solitary circumscribed retinal astrocytic proliferation: a lesion that can regress.
      These pearl-white retinal lesions first coined as PSCRAP lesions originally were thought to derive from astrocytes of the RNFL.
      • Shields J.A.
      • Bianciotto C.G.
      • Kivela T.
      • Shields C.L.
      Presumed solitary circumscribed retinal astrocytic proliferation: The 2010 Jonathan W. Wirtschafter Lecture.
      With the aid of extended depth SD-OCT more recently, it was postulated that PSCRAP lesions may originate from fibrous metaplasia of the RPE or, alternatively, may be of retinal glial origin rather than derived from retinal astrocytes as first suggested.
      • Shields C.L.
      • Roe R.
      • Yannuzzi L.A.
      • Shields J.A.
      Solitary circumscribed “pearl white” retinal mass (so-called retinal astrocytic proliferation) resides in deep retina or beneath retina: Findings on multimodal imaging in 4.
      ,
      • Goldberg R.A.
      • Raja K.M.
      Presumed solitary circumscribed retinal astrocytic proliferation in the fovea with OCT angiography: A misnomer.
      However, on fundus autofluorescence imaging, RPE metaplasia should appear hypoautofluorescent because there is no intrinsic or extrinsic lipofuscin production. Both lesions in our case produced slightly increased autofluorescence. Lipofuscin and calcium may appear hyperautofluorescent, but this was not present on clinical examination or B-scan ultrasonography.
      To our knowledge, this is the first case of multifocal PSCRAP lesions in the same eye confirmed with multimodal imaging. All previous reports of PSCRAP described these lesions as unifocal and solitary in nature (see Table 1).
      • Shields J.A.
      • Bianciotto C.G.
      • Kivela T.
      • Shields C.L.
      Presumed solitary circumscribed retinal astrocytic proliferation: The 2010 Jonathan W. Wirtschafter Lecture.
      • Shields C.L.
      • Roe R.
      • Yannuzzi L.A.
      • Shields J.A.
      Solitary circumscribed “pearl white” retinal mass (so-called retinal astrocytic proliferation) resides in deep retina or beneath retina: Findings on multimodal imaging in 4.
      • Goldberg R.A.
      • Raja K.M.
      Presumed solitary circumscribed retinal astrocytic proliferation in the fovea with OCT angiography: A misnomer.
      • Schwartz S.G.
      • Harbour J.W.
      Spectral-domain optical coherence tomography of presumed solitary circumscribed retinal astrocytic proliferation versus astrocytic hamartoma.
      • Asensio-Sánchez V.M.
      Presumed solitary circumscribed retinal astrocytic proliferation: a lesion that can regress.
      ,
      • Asensio-Sánchez V.M.
      • Díaz-Cabanas L.
      Presumed solitary circumscribed retinal astrocytic proliferation.
      Furthermore, multimodal imaging suggests that these lesions do not appear to be astrocytic in etiology, but rather may originate from RPE or glial cells in the outer retina.
      • Shields C.L.
      • Roe R.
      • Yannuzzi L.A.
      • Shields J.A.
      Solitary circumscribed “pearl white” retinal mass (so-called retinal astrocytic proliferation) resides in deep retina or beneath retina: Findings on multimodal imaging in 4.
      ,
      • Goldberg R.A.
      • Raja K.M.
      Presumed solitary circumscribed retinal astrocytic proliferation in the fovea with OCT angiography: A misnomer.
      Therefore, as recently proposed, the term “PSCRAP” appears to be a misnomer in addressing these retinal pearl-like lesions.
      • Shields C.L.
      • Roe R.
      • Yannuzzi L.A.
      • Shields J.A.
      Solitary circumscribed “pearl white” retinal mass (so-called retinal astrocytic proliferation) resides in deep retina or beneath retina: Findings on multimodal imaging in 4.
      ,
      • Goldberg R.A.
      • Raja K.M.
      Presumed solitary circumscribed retinal astrocytic proliferation in the fovea with OCT angiography: A misnomer.
      With the emerging use of swept-source OCT and extended depth imaging it may be possible to further elucidate the origin of these “retinal pearls” in the near future.

      Footnotes and Disclosure

      The authors have no proprietary or commercial interest in any materials discussed in this article.

      References

        • Shields J.A.
        • Bianciotto C.G.
        • Kivela T.
        • Shields C.L.
        Presumed solitary circumscribed retinal astrocytic proliferation: The 2010 Jonathan W. Wirtschafter Lecture.
        Arch Ophthalmol. 2011; 129: 1189-1194
        • Shields C.L.
        • Roe R.
        • Yannuzzi L.A.
        • Shields J.A.
        Solitary circumscribed “pearl white” retinal mass (so-called retinal astrocytic proliferation) resides in deep retina or beneath retina: Findings on multimodal imaging in 4.
        Retin Cases Brief Rep. 2017; 11: 18-23
        • Goldberg R.A.
        • Raja K.M.
        Presumed solitary circumscribed retinal astrocytic proliferation in the fovea with OCT angiography: A misnomer.
        Ophthalmic Surg Lasers Imaging Retina. 2018; 49: 212-214
        • Schwartz S.G.
        • Harbour J.W.
        Spectral-domain optical coherence tomography of presumed solitary circumscribed retinal astrocytic proliferation versus astrocytic hamartoma.
        Ophthalmic Surg Lasers Imaging Retina. 2015; 46: 586-588
        • Asensio-Sánchez V.M.
        Presumed solitary circumscribed retinal astrocytic proliferation: a lesion that can regress.
        Int Med Case Rep J. 2019; 12: 85-88
        • Goel N.
        • Pangtey B.
        • Bhushan G.
        • Raina U.K.
        • Ghosh B.
        Spectral-domain optical coherence tomography of astrocytic hamartomas in tuberous sclerosis.
        Int Ophthalmol. 2012; 32: 491-493
        • Asensio-Sánchez V.M.
        • Díaz-Cabanas L.
        Presumed solitary circumscribed retinal astrocytic proliferation.
        Arch Soc Esp Oftalmol. 2017; 92: 141-144