Editorial proof done on 15

th
March 2010
Massive retinal gliosis: An unusual case with Immunohistochemical study.
Astract:
Massive retinal gliosis is a rare, benign intraocular condition which results from proliferation
of well-differentiated glial cells. Immunohistochemically these cells show positivity for glial
fibrillary acid protein (GFA!,neuron specific enolase("#$! and #-%&& protein 'e
encountered a case of () year female with loss of vision in left eye. #he had given a history of
trauma to that eye two years ago. $nucleation was carried out because malignancy was
suspected due to retinal calcification. *n the basis of light microscopy and
inmmunohistochemistry (I+,! done on enucleated eye, it was diagnosed as massive retinal
gliosis.
-eywords. Massive retinal gliosis, enucleation, immunohistochemistry
Introduction
Massive retinal gliosis (M/G! is a rare, benign intraocular condition resulting from
proliferation of well-differentiated glial cells.
0%1
2he term massive retinal gliosis was first used
by, Friedenwald in %345.
041
It is a non-neoplastic tissue response to retinal in6ury that may
develop in association with congenital malformations, trauma, glaucoma, intraocular
neoplasms, vascular disorders, and chronic inflammatory conditions resulting in atrophic
phthisis bulbi. .
071
It may also occur due to old retinal detachment, retinopathy of prematurity
or as complication of retinal detachment surgery .
0(,)1
2his condition may be mista8en for
intraocular tumors.
0(1
'e are presenting here a rare case of M/G diagnosed on the basis of
routine histopathology and immunohistochemical studies.
!ase report
A () year old female presented with loss of vision in left eye since two years. #he
gave history of trauma to that eye two years ago. ,linically, the diagnosis was anterior
staphyloma in left eye. 2he right eye was normal. *n ophthalmoscopic e9amination, large
tumor li8e mass was seen in the left eye. ,omputed tomogram (,2! scan of orbit revealed that
left eyeball was deformed with stretching and thinning of the anterior sclero-uveal coats. :ense
calcification was noted within the vitreous of left eye ball. 2here was homogenous ha;e within
the left eye ball associated with atrophic optic nerve 0Fig. %1. 2he right eyeball appeared
normal. <ased on these findings and clinical diagnosis of intraocular neoplasm, enucleation of
left eye was carried out.
*n gross e9amination eyeball was hard, shrun8en and deformed with opa=ue cornea.
*ptic nerve measuring )mm length was also identified attached to the eyeball. ,ut surface
showed the vitreous cavity replaced by a solid, firm, homogenous grey white mass measuring
7.& 9 %.> 9 %.) cm in si;e. *n light microscopy, the retina was replaced by spindle shaped glial
cells arranged in interlacing bundles and whorls 0Fig. 41. Individual cells were elongated with
abundant eosinophilic fibrillary cytoplasm 0Fig. 71. 2hin walled blood vessels with thic8
hyaline layer were noted. Foci of calcifications were also identified. *n immunohistochemical
study, the spindle shaped cells showed intense positivity for glial fibrillary acidic protein
(GFA! 0Fig. 71,neuron specific enolase ("#$! and #-%&&. #o, on the basis of light microscopy
and immunohistochemistry study findings, diagnosis of M/G was offered.
"iscussion:
M/G represents non-neoplastic proliferation of the retinal glia. 'e believe this is
the first reported case from Indian literature with immunohistochemical confirmation.
/egarding histogenesis, the nodule of M/G and the associated preretinal glial membrane result
from the proliferation and migration of M?ller cells.
0%1
In M/G, both se9es and all ages may be
affected nearly in e=ual fre=uency.
0%1
'e encountered massive retinal gliosis in a middle aged
female patient. Its onset often occurs ten or more years after predisposing disorder such as
chronic inflammation, vascular disorders, glaucoma, trauma, retinal detachment surgery or
congenital abnormalities.
0(,)1
In our case, the patient had trauma to the affected eye two years
bac8 which is a 8nown predisposing factor for the development of massive retinal gliosis.
2his lesion appears as a single or multiple well vasculari;ed nodules, which has a
predilection for the peripheral retina but may occur anywhere.
051
2he differential diagnosis of
such an intraocular lesion includes uveal melanoma, astrocytic hamartoma, retinal
hemangioblastomas, tumors of the retinal pigment epithelium, intraocular metastasis and
vasoproliferative tumors of the retina (@2/! .
0A1
Banoff and co-wor8ers
071
reported 7> cases of massive gliosis of the retina. 2he
author defined 7 criteria for massive gliosis of the retina. (a! segmental or total replacement of
the retina by glial tissue C(b! abnormal blood vessels within the glial massC and (c!obliteration
of the normal retinal architecture by the proliferating glial tissue. 2he current case fulfilled the
criteria mentioned aboveC in addition it showed positivity for GFA, "#$ and #-%&& on
immunohistochemical mar8er studies confirming the glial origin.
2his case is differentiated from these alternative diagnoses based on the
histopathologic features and immunohistochemistry of the lesions. +owever, the distinction
between M/G and @2/ is more difficult, since both entities share similar histologic features
consisting mainly of glial and vascular proliferations. 'hile in M/G the glial component
predominates, in @2/ both components are largely represented. 2his may e9plain the
e9udative features of @2/, which are usually not seen in M/G.
0>1
It must be mentioned that, massive retinal gliosis is a very rare condition.
:istinction of massive retinal gliosis from other intraocular neoplasm is clinically very
difficult, so eyeball may need to be enucleated to potentially save the patients lifeC however
light microscopy and Immunohistochemistry is mandatory for the final diagnosis. 2he
,linician should 8eep this in mind when they come across retinal calcification, especially in an
adult patient. 2rauma is a significant cause of blindness world-wide.
031
M/G is another way in
which trauma can cause loss of vision long after the instigating event.
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$egends
Figure % D ,omputed tomogram (,2!-scan showing deformed left eyeball
with homogenous ha;e
and areas of calcification.
Figure 4 - 2he retina was replaced by spindle shaped glial cells arranged in
interlacing bundles and whorls. Individual cells are elongated
with abundant eosinophilic fibrillary cytoplasm (Inset!.
Figure 7 - *n immunohistochemical study the spindle shaped cells showing
intense immunoreactivity for glial fibrillary acidic protein (GFA!