Program Book

have highlighted a role for specific cell signaling pathways and the
tumor microenvironment in the biology of this tumor. In this study we
performed immunohistochemical studies in a relatively large series of
OPG using tissue microarrays.
Methods. Tumors from 59 patients with a median age of 9 years (range
3 mo-66 years; 33 F,26 M) were tested using formalin-fixed paraffin
embedded material in tissue microarrays. Immunohistochemistry
was performed using antibodies recognizing mTOR pathway signaling
components (total mTOR protein, Phospho-4E-BP1, PhosphoeIf4G,
phosho-S6), microglia (CD68), markers of senescence (p16),
and mutant IDH1 protein. Scoring was performed using a 4-tiered
semiquantitative scale (0-4+). IDH1 was scored as positive or negative.
Immunohistochemistry for GFAP was performed to evaluate for adequacy
of immunoreactivity.
Results. Immunohistochemical stains demonstrated frequent staining
for mTOR pathway components, including moderate (2+) to strong (3+)
staining for total mTOR (50%), phospho-4E-BP1 (42%), phospho-eIf4G
(75%), and phospho-S6 (70%). Moderate to marked numbers of CD68
positive microglia were identified in 44% of cases. Moderate to diffuse
nuclear immunoreactivity for p16 was identified in 59% of cases. All
cases (except for 1) were immunonegative for IDH1 mutant protein.
Conclusions. OPG demonstrate increased total/activated levels of mTOR
signaling components supporting an important role for this pathway
in its biology. In addition, increased numbers of CD68+ microglia and
markers of senescence (p16) were identified. These findings merit
further study and correlation with additional molecular markers to
evaluate their clinical and biological significance.
Financial disclosure. None
EYELID, CONJUNTIVA & ORBIT
Abstract Posters
86
Other Intra-ocular Tumors Posters
131 OTp101
PHOTODYNAMIC THERAPY FOR ACQUIRED ASTRO-
CYTOMA
Cinzia Mazzini, Maria Carla Donati, Giulia Pieretti, Ugo Menchini (cinzia.
mazzini@unifi.it)
Department of Specialistic Surgery Sciences, Eye Clinic, University of
Florence, Florence, Italy.
Purpose. Retinal astrocytomas are glial tumours of the RNFL arising from
retinal astrocytes, often associated with systemic disorders ( tuberous
sclerosis or neurofibromatosis) but they can also be found on retinal
examination as isolated tumour. We report the case of epipapillary
retinal astrocytoma associated with serous retinal detachment treated
with photodynamic therapy (PDT).
Methods. A 32-years old man was referred for an elevated whitish
epipapillary lesion associated with retinal detachment. He had a history
of multicellular carcinoma and melanocytic nevus of the scapular region.
Visual acuity was 20/20 in both eyes. Fluorescein angiography revealed
a hyperfluorescence of the lesion; B-scan showed a solid epipapillary
tumour with high reflectivity, posterior shadowing, and a contiguous
serous retinal detachment. OCT showed a solid elevation of the retinal
layers associated with raised neuro-epithelium at the edge of the lesion.
The clinical picture was consistent with “acquired astrocytoma”.
Four years later, the patient complained of a severe visual loss in left
eye. BCVA was 20/50 and fundus examination, echography, fluorescein
angiography and OCT showed an enlargement of the lesion and retinal
detachment, with macular exudation. PDT with verteporfirin was performed.
Results. Three months after treatment visual acuity remained stable;OCT
and echography revealed a decrease of subretinal fluid. One year later
BCVA was 20/30, and retinal detachment was disappeared.
Conclusions. PDT with verteporfin can induce regression of aggressive
retinal astrocytomas and may prevent their progression to total retinal
detachment and enucleation. In selected severe cases, PDT may be
considered a first-line treatment for acquired astrocytomas.
Financial disclosure. None
530 OTp102
MANAGEMENT OF PERIPAPILLARY HEMANGIOMA IN
PEDIATRIC VHL DISEASE
Prithvi Mruthyunjaya, MD (mruth001@mc.duke.edu)
Ocular Oncology and Vitreoretinal Surgery Services, Duke Eye Center,
Durham, NC
Purpose. To describe the management of a peripapillary retinal
hemangioma in a pediatric patient with von-Hippel-Lindau disease and
to describe the early development of epiretinal proliferations in this
disease.
Methods. Case report of a 2 yo male with a strong family history of VHL
disease who presents with a new peripapillary hemangioma associated
with a dome-shaped epimacular elevation.
Results. Intraoperative photodynamic therapy was performed with
verteporfin and intravitreal Avastin on 2 occasions with subsequent
inactivation of the peripapillary hemangioma. The macular elevation
was likely due to vitreous traction on the macular hyloid bursa with