ECP 2017 - Abstract Book

PS-11-001

IgG4-related disease and non-Hodgkin lymphoma: A temporal spin

R. Luis

, C. Quadros, C. Ferreira, D. López-Presa

Hosp Santa Maria - CHLN, EPE, Serv Anatomia Patologica, Lisbon,

Portugal

Objective:

IgG4-related disease (IgG4-RD) is a fibro-inflammatory con-

dition which induces organ expansion, specific histological findings

—

storiform fibrosis, obliterative phlebitis and plasmacytic IgG4+ infil-

trates

—

and higher risk of malignancy. We report a case of orbital IgG4-

RD following a lymphoma diagnosis.

Method:

A 62 year-old male presented with ocular pain and progressive

vision loss. Imaging studies showed ciliary body thickening; subsequent

biopsies revealed a non-Hodgkin lymphoma of small mature B-cells, with

monoclonality. The patient underwent ocular radiotherapy.

Nevertheless, 2 years later, eye enucleation was performed due to inexo-

rable orbit enlargement.

Results:

Grossly, a white-yellow lesion expanded both sclera and choroid.

Microscopically, we observed storiform fibrosis, an intense T-lymphocyte

infiltrate with germinal centers and numerous plasma cells, displaying con-

served

ratio and >50 IgG4+ cells/HPF, supporting the sclerosing

pseudotumour (IgG4-RD) diagnosis. Further investigation unveiled increased

serum IgG4 and retroperitoneal fibrosis, confirming an IgG4-RD.

Conclusion:

The presentation of IgG4-RD as ocular pseudotumour is

rare, especially in the sclera, since the majority affect the lacrimal gland

and periorbital soft tissue. We present the second case of IgG4-RD fol-

lowing radiotherapy for lymphoma, illustrating an inversion in the pre-

sentation timing so far described. In our regard, this patient in fact suf-

fered from underlying

—

yet undiagnosed

—

IgG4-RD with a

superimposed lymphoma.

PS-11-002

Massive retinal gliosis masquerading as an intraocular tumour

I. A. Cianga Spiridon

, M. Danciu

Grigore T. Popa University of Medicine and Pharmacy, Iasi, Romania

Objective:

Massive retinal gliosis is a rare, pseudoneoplastic glial prolifera-

tion that may develop in association with congenital malformations or trauma.

Some are syndrome associated with entities such as neurofibromatosis type 1

or tuberous sclerosis. Herein, we report the clinicopathological features of a

massive retinal gliosis case in a 23-year-old male patient.

Method:

The surgical specimen was routinely processed by formalin

fixation and paraffin embedding. Hematoxylin and eosin staining and

immunohistochemical tests were performed.

Results:

: Histologic examination revealed the replacement of the anterior

and posterior ocular compartments with a proliferation of interweaving

bundles of spindle-shaped cells with uniform nuclei and delicate fibrillary

cytoplasm. Mitotic figures were rare. Thick-walled, hyalinized blood ves-

sels, microcystic spaces and the occasional presence of Rosenthal fibers

were observed. The process was accompanied by focal calcifications,

calcospherites and osseous metaplasia. A particular feature was the adipose

tissue infiltration of the central area. Immunohistochemistry was positive

for glial fibrillary acidic protein, vimentin and S-100 protein. Additionally,

moderate diffuse staining for Melan-A and Ki-67 < 3 % were observed.

Conclusion:

The aforementioned features are consistent with massive

retinal gliosis, a pseudoneoplastic reactive process developed on a con-

genital malformation. This rare condition raises differential diagnosis

issues with malignant melanoma and schwannoma.

PS-11-003

Ocular Pathology: A glance over 10 years of institutional experience

C. Quadros

, R. Luís, J. Monteiro, D. López-Presa

Hospital de Santa Maria, Lisbon, Portugal

Objective:

Hospital de Santa Maria (Lisbon, Portugal) is a tertiary

healthcare institution with an experienced Ophthalmology Department.

We present our expertise through a 10 year-long series.

Method:

Retrospective data pertaining to the 2007-2016 period were evalu-

ated for the following criteria: age, sample type, location and pathology.

Results:

A total of 661 reports were assessed, increasing from 41 in 2007

to 103 in 2016. Age ranged from 1 month to 93 years. The accounted

procedures were excisional biopsies (75 %), incisional biopsies (21 %)

and enucleations (4 %). The predominantly involved anatomical structures

were palpebral skin (37 %), conjunctiva (25 %) and cornea (14 %). In the

419 cases remaining after cutaneous palpebral pathology was excluded, the

majority were inflammatory/reactive changes (37 %), neoplasms (36 %)

and the remainder consisted mostly of degenerative conditions (18 %);

malignancies (20 %) were led by squamous cell carcinoma (6 %), primarily

from the conjunctiva (85 %); regarding melanoma (5 %), 53 % originated

in the uvea; melanocytic nevi (8 %) prevailed among benign neoplasms

(16 %). Particularly noteworthy were the unique diagnosis of metastatic

prostatic and lung carcinoma, and an IgG4-related disease.

Conclusion:

Throughout the 10-year this series comprises, the specimens

have almost tripled in number. To our knowledge this is the only retrospective

study that encompasses all the ocular pathologies in one institution.

PS-11-004

Ciliary body medulloepithelioma in an adolescent girl: Report of a case

S. Mata

, R. Adame Cabrera

Instituto Português Oncologia, Anatomia patológica, Lisbon, Portugal

Objective:

Ciliary body medulloepithelioma (CBME) is a rare congenital

neoplasm usually presenting in young children, with very few cases de-

scribed in adolescents. Its rarity and clinical presentation may contribute

to a delay in diagnosis. We report a case of a CBME with an aggressive

clinical course that presented during adolescence.

Method:

The clinicopathological features of a case of CBME are de-

scribed, with a review of the literature.

Results:

A 16-year-old female with a history of right eye enucleation for

glaucoma was referred to our institution, with a rapidly growing 7 cm

cranial invading orbital mass. The histopathological diagnosis of the enu-

cleation specimen was unavailable at the time and a biopsy was performed.

A small blue cell tumour was identified, and a diagnosis favoring PNET

was rendered. After a partial response to chemotherapy, she underwent

orbital exenteration. An embryonal tumour with neuroblastic and epithelial

elements consistent with medulloepithelioma was observed. The enucle-

ation specimen arrived 2 months later for review, and a diagnosis of teratoid

CBME with poor prognostic features was confirmed.

Conclusion:

CBME is rare neoplasm that can pursue an aggressive clinical

course. Awareness of the entity, which may occur even during adolescence,

could aid in its prompt diagnosis and to plan adequate treatment and follow-up.

PS-11-005

A rare case of ocular melanoma onset in a patient with vitreous

hemorrhage

A. S. Postolache

, I. Dumitru, S. A. Varban, M. Costache, M. Ghita

Emergency University Hospital, Dept. of Pathology, Bucharest,

Romania

Objective:

Ocular melanoma is the most frequent eye tumour found in

adult patients. This paper highlights a case of a small primary choroidal

melanoma with particularly unusual clinical onset: vitreous hemorrhage.

Monday, 4 September 2017, 09:30

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10:30, Hall 3

PS-11 Ophthalmic Pathology

Virchows Arch

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2017

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Suppl 1

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