Publications by authors named "Bercin Tarlan"

26 Publications

  • Page 1 of 1

Primary mucosa-associated lymphoid tissue (MALT) lymphoma of the lacrimal drainage system in two pediatric patients.

Eur J Ophthalmol 2020 May 12;30(3):NP18-NP23. Epub 2019 Mar 12.

Department of Ophthalmology, Gazi University Medical School, Ankara, Turkey.

Primary non-Hodgkin lymphoma (NHL) of lacrimal drainage system (LDS) is quite rare in children, but it is important to expedite early diagnosis in an effort to alter possible life-threatening disease since they are usually misdiagnosed as chronic dacryocystitis. In the literature, there are few examples of tumors of LDS in children. The authors herein report two pediatric cases of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) originating from lacrimal sac in an attempt to increase the knowledge about the clinical course of NHL of LDS. Considerable care must be taken since tumors of lacrimal drainage can mimic dacryocystitis clinically and macroscopically. Two patients both attended with painless swelling in the left lacrimal sac region and epiphora of the left eye. Orbital magnetic resonance imaging showed a tumoral lesion in the left lacrimal sac region and histopathological examination of excisional biopsy specimen demonstrated MALT lymphoma in both patients. The treatment regimen comprises lacrimal sac excision within the tumor, canalicular dacryocystorhinostomy (DCR) with bicanalicular silicone intubation (BSI) combined with chemotherapy and regional radiotherapy in one case, whereas the second case received only radiotherapy after canalicular DCR with BSI. Both of them maintained clinical remission along follow-up.
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http://dx.doi.org/10.1177/1120672119837085DOI Listing
May 2020

Gene Expression Profiling and PRAME Status Versus Tumor-Node-Metastasis Staging for Prognostication in Uveal Melanoma.

Am J Ophthalmol 2018 11 6;195:154-160. Epub 2018 Aug 6.

Ocular Oncology Service, Bascom Palmer Eye Institute and Sylvester Comprehensive Cancer Center, University of Miami Miller School of Medicine, Miami, Florida, USA. Electronic address:

Purpose: To compare the prognostic accuracy of gene expression profiling (GEP) combined with PRAME status vs the clinical Tumor-Node-Metastasis (TNM) staging in patients with uveal melanoma (UM).

Design: Retrospective cohort study.

Methods: The study included 240 consecutive patients with UM. Tumors were assessed for GEP status (Class 1 or Class 2) using a validated 15-gene assay and PRAME expression status using quantitative polymerase chain reaction. TNM staging was according to the American Joint Committee on Cancer 8th edition. Statistical analysis included univariate and multivariate Cox proportional hazard models. Metastasis was the primary endpoint.

Results: GEP was Class 1 in 128 (53.3%) cases and Class 2 in 112 (46.7%) cases. PRAME status was negative in 157 (65.4%) cases and positive in 83 (34.6%) cases. TNM was stage I in 26 (10.8%) cases, IIA in 67 (27.9%) cases, IIB in 50 (20.8%) cases, IIIA in 59 (24.6%) cases, and IIIB in 38 (15.8%) cases. Metastatic disease was detected in 59 (24.6%) cases after median follow-up of 29 months (mean 42 months; range 1-195 months). Variables associated with metastasis included (in order of decreasing significance): GEP class (P = 1.5 × 10), largest basal tumor diameter (P = 2.5 × 10), PRAME status (P = 2.6 × 10), and TNM stage (P = 3.7 × 10). The prognostic accuracy of an optimized 3-category GEP/PRAME model (P = 8.6 × 10) was superior to an optimized TNM model (P = 1.3 × 10).

Conclusions: In UM, molecular prognostic testing using GEP and PRAME provides prognostic accuracy that is superior to TNM staging.
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http://dx.doi.org/10.1016/j.ajo.2018.07.045DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6214741PMC
November 2018

Stereotactic Radiosurgery and Fractionated Stereotactic Radiation Therapy for the Treatment of Uveal Melanoma.

Int J Radiat Oncol Biol Phys 2017 05 17;98(1):152-158. Epub 2017 Feb 17.

Department of Radiation Oncology, Faculty of Medicine, Hacettepe University, Ankara, Turkey. Electronic address:

Purpose: To evaluate treatment results of stereotactic radiosurgery or fractionated stereotactic radiation therapy (SRS/FSRT) for uveal melanoma.

Methods And Materials: We retrospectively evaluated 181 patients with 182 uveal melanomas receiving SRS/FSRT between 2007 and 2013. Treatment was administered with CyberKnife.

Results: According to Collaborative Ocular Melanoma Study criteria, tumor size was small in 1%, medium in 49.5%, and large in 49.5% of the patients. Seventy-one tumors received <45 Gy, and 111 received ≥45 Gy. Median follow-up time was 24 months. Complete and partial response was observed in 8 and 104 eyes, respectively. The rate of 5-year overall survival was 98%, disease-free survival 57%, local recurrence-free survival 73%, distant metastasis-free survival 69%, and enucleation-free survival 73%. There was a significant correlation between tumor size and disease-free survival, SRS/FSRT dose and enucleation-free survival; and both were prognostic for local recurrence-free survival. Enucleation was performed in 41 eyes owing to progression in 26 and complications in 11.

Conclusions: The radiation therapy dose is of great importance for local control and eye retention; the best treatment outcome was achieved using ≥45 Gy in 3 fractions.
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http://dx.doi.org/10.1016/j.ijrobp.2017.02.017DOI Listing
May 2017

Uveal Melanoma: Current Trends in Diagnosis and Management.

Turk J Ophthalmol 2016 Jun 6;46(3):123-137. Epub 2016 Jun 6.

Hacettepe University Faculty of Medicine, Department of Ophthalmology, Ankara, Turkey.

Uveal melanoma, which is the most common primary intraocular malignancy in adults, arises from melanocytes within the iris, ciliary body and choroid. The diagnosis is based principally on clinical examination of the tumor with biomicroscopy and indirect ophthalmoscopy and confirmed by diagnostic techniques such as ultrasonography, fundus fluorescein angiography and optical coherence tomography. The clinical diagnosis of posterior uveal melanomas can be made when the classical appearance of a pigmented dome-shaped mass is detected on dilated fundus exam. Uveal melanomas classically show low to medium reflectivity on A-scan ultrasonography and on B-scan ultrasonography the tumor appears as a hyperechoic, acoustically hollow intraocular mass. Management of a suspicious pigmented lesion is determined by its risk factors of transforming into a choroidal melanoma, such as documentation of growth, thickness greater than 2 mm, presence of subretinal fluid, symptoms and orange pigment, margin within 3 mm of the optic disc, and absence of halo and drusen. Advances in the diagnosis and local and systemic treatment of uveal melanoma have caused a shift from enucleation to eye-conserving treatment modalities including transpupillary thermotherapy and radiotherapy over the past few decades. Prognosis can be most accurately predicted by genetic profiling of fine needle aspiration biopsy of the tumor before the treatment, and high-risk patients can now be identified for clinical trials that may lead to target-based therapies for metastatic disease and adjuvant therapy which aims to prevent metastatic disease.
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http://dx.doi.org/10.4274/tjo.37431DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5076295PMC
June 2016

Epigenetic reprogramming and aberrant expression of PRAME are associated with increased metastatic risk in Class 1 and Class 2 uveal melanomas.

Oncotarget 2016 Sep;7(37):59209-59219

Bascom Palmer Eye Institute, Sylvester Comprehensive Cancer Center and Interdisciplinary Stem Cell Institute, University of Miami Miller School of Medicine, Miami, FL, USA.

Background: We previously identified PRAME as a biomarker for metastatic risk in Class 1 uveal melanomas. In this study, we sought to define a threshold value for positive PRAME expression (PRAME+) in a large dataset, identify factors associated with PRAME expression, evaluate the prognostic value of PRAME in Class 2 uveal melanomas, and determine whether PRAME expression is associated with aberrant hypomethylation of the PRAME promoter.

Results: Among 678 samples analyzed by qPCR, 498 (73.5%) were PRAME- and 180 (26.5%) were PRAME+. Class 1 tumors were more likely to be PRAME-, whereas Class 2 tumors were more likely to be PRAME+ (P < 0.0001). PRAME expression was associated with shorter time to metastasis and melanoma specific mortality in Class 2 tumors (P = 0.01 and P = 0.02, respectively). In Class 1 tumors, PRAME expression was directly associated with SF3B1 mutations (P < 0.0001) and inversely associated with EIF1AX mutations (P = 0.004). PRAME expression was strongly associated with hypomethylation at 12 CpG sites near the PRAME promoter.

Materials And Methods: Analyses included PRAME mRNA expression, Class 1 versus Class 2 status, chromosomal copy number, mutation status of BAP1, EIF1AX, GNA11, GNAQ and SF3B1, and genomic DNA methylation status. Analyses were performed on 555 de-identified samples from Castle Biosciences, 123 samples from our center, and 80 samples from the TCGA.

Conclusions: PRAME is aberrantly hypomethylated and activated in Class 1 and Class 2 uveal melanomas and is associated with increased metastatic risk in both classes. Since PRAME has been successfully targeted for immunotherapy, it may prove to be a companion prognostic biomarker.
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http://dx.doi.org/10.18632/oncotarget.10962DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5312306PMC
September 2016

Orbital Extension of an Unsuspected Choroidal Melanoma Presumably through an Aqueous Tube Shunt.

Ocul Oncol Pathol 2016 Apr 25;2(3):144-7. Epub 2015 Nov 25.

Department of Ophthalmology, Kozluk State Hospital, Batman, Turkey.

Aims: To report a patient with unrecognized small macular melanoma, who gradually developed neovascular glaucoma that was treated with an Ahmed valve. We presume that tumor cells gained access to the orbit through this aqueous drainage device.

Methods: The medical records pertaining to 5 different centers, the results of imaging studies, the clinical course of the patient and histopathological findings were retrospectively reviewed.

Results: A 61-year-old man presented with a blind and painful proptotic right eye. Retinal hemorrhage had been suspected 3 years earlier and followed accordingly thereafter. Yearly performed MRI studies demonstrated the steady growth of the hemorrhagic lesion/tumor. Meanwhile, he developed neovascular glaucoma first treated unsuccessfully with cyclocryotherapy then with implantation of an Ahmed valve. Right proptosis became evident within a year, and a final MRI study revealed a large retrobulbar mass. Orbital exenteration was performed and histopathological examination showed intraocular and orbital epithelioid cell melanoma. Tumor cells were also found within the reservoir of the Ahmed valve. The patient died of widespread metastases 1 year later.

Conclusions: Ahmed valve implantation in an eye with unsuspected macular choroidal melanoma may ultimately be associated with extraocular extension and orbital tumor formation.
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http://dx.doi.org/10.1159/000441726DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4881271PMC
April 2016

Intra-Arterial Chemotherapy for Retinoblastoma: A Single-Center Experience.

Ophthalmologica 2015 15;234(4):227-32. Epub 2015 Sep 15.

Department of Pediatric Oncology, Cancer Institute, Hacettepe University, Ankara, Turkey.

Background: Studies conducted in recent years have reported promising results regarding the treatment of retinoblastoma with the intra-arterial use of melphalan. In the present study, we intended to report the results of intra-arterial chemotherapy with melphalan (IACT) in the treatment of newly diagnosed or relapsed-refractory retinoblastoma patients at the Department of Pediatric Oncology of Hacettepe University, Ankara, Turkey.

Materials And Methods: This was a retrospective study of patients with intraocular retinoblastoma who were treated with IACT from December 2011 to May 2014. A total of 56 eyes of 46 consecutive patients (30 males and 16 females) were included in the study. Forty-four eyes received systemic chemotherapy upon diagnosis (systemic chemotherapy group, SCG), and 12 eyes were those of newly diagnosed patients (primary intra-arterial melphalan group, PIAG). The choice of the IACT dose was based on age. Tumor control and globe salvage with IACT were analyzed. Complete blood counts were examined 7 days after the IACT for systemic toxicity. Ocular toxicities such as proptosis, eyelid edema, ocular motility, and retinal and optic atrophy were assessed by an ocular oncologist with regular ophthalmologic examinations.

Results: Enucleation was avoided overall in 66% (37/56) of the eyes, including 75% (9/12) in the PIAG and 64% (28/44) in the SCG patients. The 1-year enucleation-free survival rate was 56.7% at a median follow-up time of 11.9 months (range 0.27-27.6). IACT was administered in a total of 124 cycles (ranging from 1 to 7 cycles, mean 2.3). The responses were as follows: regression of the retinal tumor in 27 eyes and improvements in vitreous seeding in 5 of 15 eyes. The further treatment requirements after IACT were as follows: enucleation in 19 eyes (10 with vitreous seeding), radiotherapy in 3 eyes, systemic chemotherapy in 1 eye, and local therapy in 1 eye. No severe systemic side effects occurred. Transient swelling of the eyelids (22 patients), conjunctival chemosis (12 patients), upper eyelid ptosis (5 patients), redness over the frontal area (3 patients), limitation of ocular motility (3 patients) and mild proptosis (1 patient) were detected. Retinal pigment epithelial alterations (30 patients) and optic atrophy (3 patients) were seen in the late follow-up.

Conclusions: Globe salvage and avoidance of radiotherapy may be achieved by IACT with limited toxicity. This treatment is efficient, repeatable and safe.
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http://dx.doi.org/10.1159/000439357DOI Listing
May 2016

Total clinical regression of an orbital macrocystic lymphatic malformation following intralesional sodium tetradecyl sulphate injection.

J AAPOS 2015 Feb;19(1):78-80

Ocular Oncology Service, Department of Ophthalmology, Hacettepe University School of Medicine, Ankara, Turkey.

Lymphatic malformations are usually managed by observation, although significant cosmetic abnormalities, compressive optic neuropathy, risk of amblyopia, exposure keratopathy, or elevated intraocular pressure mandate treatment. We report the case of a 10-year-old boy with a deep orbital macrocystic lymphatic malformation that was successfully treated with intralesional injection of sodium tetradecyl sulphate, a sclerozing agent mainly used in the treatment of small varicose veins in the lower extremity.
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http://dx.doi.org/10.1016/j.jaapos.2014.08.012DOI Listing
February 2015

Adult orbital xanthogranulomas: clinical features and management.

Eur J Ophthalmol 2015 Jul-Aug;25(4):288-92. Epub 2015 Jan 15.

1 Ocular Oncology Service, Department of Ophthalmology, Hacettepe University School of Medicine, Ankara - Turkey.

Purpose: Adult-onset asthma with periocular xanthogranuloma and adult-onset xanthogranuloma are 2 rare subtypes of non-Langerhans cell histiocytic disorder and much remains unknown regarding optimal treatment. The authors describe their experience in the management of these 2 disease subtypes.

Methods: This is a retrospective case series with histopathologically proven orbital xanthogranuloma over a period of 12 years. Clinical, imaging, and histopathologic features; associated systemic conditions; treatment modality; and outcome during follow-up of 6 adult patients who had adult-onset asthma with periocular xanthogranuloma and adult-onset xanthogranuloma were reviewed.

Results: The age range was 29-75 years (median 56 years). The duration of symptoms and signs varied from 10 months to 9 years. All patients had bilateral and asymmetric involvement. Palpebral swelling with yellow discoloration and upper eyelid ptosis were the most common signs. Adult-onset asthma was present in 2 patients. Imaging studies demonstrated ill-defined infiltrative lesions involving the preseptal area, lacrimal glands, extraocular muscles, retrobulbar fat, and optic nerves. The median follow-up was 50 months. Complete regression of all clinical signs was obtained at 8 months, whereas imaging findings disappeared at 18 months with treatment. No recurrence was observed.

Conclusions: Treatment consisting of debulking as much affected soft tissue as possible followed by a 3-day course of intravenous pulse methylprednisolone administration and then by oral prednisone for at least 6 months may provide adequate regression of the granulomas without recurrence and satisfactory cosmesis in patients with adult orbital xanthogranuloma with and without asthma.
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http://dx.doi.org/10.5301/ejo.5000555DOI Listing
September 2015

Genome-wide homozygosity mapping in families with leber congenital amaurosis identifies mutations in AIPL1 and RDH12 genes.

DNA Cell Biol 2014 Dec;33(12):876-83

1 Metabolism Unit, Department of Pediatrics, Institute of Child Health, Hacettepe University , Ankara, Turkey .

Leber congenital amaurosis (LCA) causes severe visual impairment and blindness very early in life. Mutant alleles of several genes acting in different pathways, of which all have critical roles for normal retinal function, were involved in LCA development. The purpose of this study was to use genome-wide genotyping to identify LCA-causing loci in two Turkish families. Genome-wide genotyping and haplotype analysis were performed for prioritization of candidate genes for mutation screening in families with LCA. Identified informative critical choromosomal regions obtained by homozygosity mapping from the families were searched for overlapping of any LCA causative genes. Corresponding clinical phenotypes of the patients with identified mutations were evaluated. In this study, two families were shown to be linked to two different LCA loci covering retinol dehydrogenase 12 (RDH12) and aryl-hydrocarbon-interacting protein-like1 (AIPL1) genes. Mutation screening revealed a novel p.Gln141* mutation in the AIPL1 gene and a previously described p.Thr49Met mutation in the RDH12 gene in a homozygous state. Our patients with the RDH12 mutation had the distinct macular coloboma sign, and the patient with the AIPL1 mutation developed microphthalmia and severe widespread retinal pigment epithelial atrophy, in contrast to previously reported cases. It is currently evident that mutation screening needs to be done in at least 18 genes known to be associated with LCA. Thus, homozygosity mapping is an alternative technique to improve the molecular diagnosis in LCA, which is a group of genetically and clinically heterogeneous diseases causing retinal degeneration. The patients without mutation in known genes may further be analyzed by using next-generation sequencing.
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http://dx.doi.org/10.1089/dna.2014.2554DOI Listing
December 2014

Renal Carcinoid Tumor Metastatic to the Uvea, Medial Rectus Muscle, and the Contralateral Lacrimal Gland.

Ophthalmic Plast Reconstr Surg 2015 Jul-Aug;31(4):e91-3

*Ocular Oncology Service, Department of Ophthalmology, and †Department of Pathology, Hacettepe University School of Medicine, Ankara, Turkey.

Renal carcinoid tumor is an exceedingly rare malignancy. A 57-year-old man with a renal carcinoid tumor discovered after metastasizing to intraocular and bilateral orbital structures is described. The patient presented with a blind painful OS and a right superotemporal subconjunctival mass. Imaging studies revealed a large left intraocular tumor, a mass in the left medial rectus muscle, and right lacrimal gland enlargement. The OS was enucleated, and incisional biopsies were performed from the other 2 lesions. Histopathological studies demonstrated metastatic neuroendocrine tumor with chromogranin and synaptophysin positivity. Systemic work up revealed a right renal mass and multiple hepatic metastatic lesions. Radical nephrectomy was performed, and octreotide, capecitabine, and temozolomide were administered. Removal of the primary tumor and the eye that had no prospect for useful vision and further treatment with octreotide, capecitabine, and temozolomide provided a disease progression-free period of 24 months and allowed the patient to function normally.
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http://dx.doi.org/10.1097/IOP.0000000000000112DOI Listing
February 2016

Optical coherence tomography characteristics of epi-iridic membrane in a child with recurrent hyphema and presumed juvenile xanthogranuloma.

J AAPOS 2014 Feb;18(1):93-5

Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania. Electronic address:

We report a case of spontaneous hyphema in a 6-month-old girl with no history of trauma and no visible iris mass. Subtle green-blue heterochromia was noted in the right eye. The iris crypts in the right eye appeared flattened by a thin, transparent layer on the iris surface. Anterior segment optical coherence tomography (AS-OCT) disclosed a thin homogenous membrane overlying the entire iris surface in the right eye. Fluorescein angiography revealed diffuse hyperfluorescence without neovascularization. These features were suggestive of diffuse iris juvenile xanthogranuloma. Sub-Tenon's triamcinolone acetate plus topical corticosteroids eyedrops resolved the condition within 1 month.
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http://dx.doi.org/10.1016/j.jaapos.2013.10.022DOI Listing
February 2014

Regression of choroidal hemangioma following systemic paclitaxel and trastuzumab use.

Graefes Arch Clin Exp Ophthalmol 2014 Jan 3;252(1):177-9. Epub 2013 Dec 3.

Ocular Oncology Service, Department of Ophthalmology, Hacettepe University School of Medicine, 06100, Sihhiye, Ankara, Turkey,

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http://dx.doi.org/10.1007/s00417-013-2521-2DOI Listing
January 2014

Orbital richter syndrome.

Orbit 2013 Dec 29;32(6):381-3. Epub 2013 Jul 29.

Ocular Oncology Service, Department of Ophthalmology, Hacettepe University School of Medicine , Ankara , Turkey and.

We report two patients with previously diagnosed chronic lymphocytic leukemia who developed Richter syndrome in the orbit as the sole extranodal site. The medical history, clinical findings, orbital imaging and histopathological features of the patients were reviewed. Treatment protocols and the outcomes were also assessed. The first patient developed Richter syndrome at the age of 64 years, 3 years after the diagnosis of chronic lymphocytic leukemia. The tumor was located at the inferotemporal quadrant of the orbit. The second patient was 59 years old when Richter syndrome arose in the lacrimal gland, 4 years after the diagnosis of chronic lymphocytic leukemia. Incisional biopsy from the orbital tumors were performed. Histopathological findings included diffuse CD20, CD 23, CD5, bcl2, bcl6 positive lymphocytic infiltration. Both patients were treated with chemotherapy and rituximab. During 3 years of follow-up, there was no orbital or systemic recurrence of the disease. Richter syndrome may develop in the orbital soft tissue and the lacrimal gland, and the orbital disease appears to have a better prognosis compared to patients with systemic involvement.
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http://dx.doi.org/10.3109/01676830.2013.815223DOI Listing
December 2013

Subconjunctival hemorrhage: risk factors and potential indicators.

Clin Ophthalmol 2013 12;7:1163-70. Epub 2013 Jun 12.

Department of Ophthalmology, Kozluk State Hospital, Batman, Turkey.

Subconjunctival hemorrhage is a benign disorder that is a common cause of acute ocular redness. The major risk factors include trauma and contact lens usage in younger patients, whereas among the elderly, systemic vascular diseases such as hypertension, diabetes, and arteriosclerosis are more common. In patients in whom subconjunctival hemorrhage is recurrent or persistent, further evaluation, including workup for systemic hypertension, bleeding disorders, systemic and ocular malignancies, and drug side effects, is warranted.
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http://dx.doi.org/10.2147/OPTH.S35062DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3702240PMC
July 2013

A case of 22q11.2 deletion syndrome with right microphthalmia and left corneal staphyloma.

Ophthalmic Genet 2014 Dec 8;35(4):248-51. Epub 2013 Jul 8.

Department of Ophthalmology and.

Unlabelled: Abstract Background: A microdeletion in the chromosome 22q11.2 (DiGeorge or velocardiofacial syndrome) is the most common human deletion syndrome. Patients with 22q11.2 deletion may have a wide range of ocular findings but severe ocular involvement is uncommon. Here, we describe a 2-year-old boy who had growth retardation, developmental delay, right renal agenesis, ventricular septal defect and severe bilateral ocular anomalies.

Materials And Methods: The systemic and ocular findings and cranial magnetic resonance imaging study results were reviewed. Fluorescence in situ hybridization analysis was performed on his peripheral blood.

Results: The patient presented with the oculodigital sign. On examination, he had severe right microphthalmia with no light perception and his left eye could not fix and follow. The left eye had anterior segment dysgenesis, mild sclerocornea, corneal staphyloma and congenital aphakia. Systemic findings included growth deficiency, microcephaly, micrognathia, ventricular septal defect, atrial septal defect and right renal agenesis. Fluorescence in situ hybridization analysis of this patient was significant for a heterozygous deletion covering DiGeorge critical region 2 and spanning a 250 kb region in the 22q11.2 locus.

Conclusion: The 22q11.2 deletion syndrome may be associated with severe bilateral ocular malformations including microphthalmia, sclerocornea, corneal staphyloma, anterior segment dysgenesis and congenital aphakia. Corneal staphyloma might have resulted from the oculodigital phenomenon or increased intraocular pressure.
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http://dx.doi.org/10.3109/13816810.2013.811269DOI Listing
December 2014

Papillary thyroid carcinoma: bilateral choroidal metastases with extrascleral extension.

Korean J Ophthalmol 2013 Jun 2;27(3):215-8. Epub 2013 Apr 2.

Ocular Oncology Service, Department of Ophthalmology, Hacettepe University School of Medicine, Ankara, Turkey.

Here, we present the case of a patient with bilateral choroidal metastases with extraocular extension in one eye. Metastasis of papillary thyroid carcinoma to the uvea is extremely rare, with only 6 patients reported in the literature. A 62-year-old man with a prior history of papillary thyroid carcinoma suffered the rapid loss of vision in his right eye. He had neovascular glaucoma, total retinal detachment, and a solitary choroidal mass. A month later, his left visual acuity also decreased because of a small macular choroidal mass. The right eye was enucleated and a nodular lesion over the sclera representing extraocular extension was observed. This tumor and the intraocular lesion were composed of papillary excrescences and cystic spaces and stained positively for thyroid transcription factor 1 and thyroglobulin, all confirming the diagnosis of metastatic papillary thyroid carcinoma. The tumor in the left eye was successfully treated with diode laser transpupillary thermotherapy. The patient expired within a month as a result of widespread pulmonary metastases. Papillary thyroid carcinoma may metastasize to the uvea bilaterally, cause rapid visual function loss, erode the sclera, and may extend outside the globe similar to choroidal melanoma. This aggressive ocular involvement was associated with a dismal prognosis in our patient.
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http://dx.doi.org/10.3341/kjo.2013.27.3.215DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3663067PMC
June 2013

Paraneoplastic vitelliform retinopathy following ciliochoroidal melanoma.

Ophthalmic Surg Lasers Imaging Retina 2013 May-Jun;44(3):290-2

Ocular Oncology Service, Department of Ophthalmology, Hacettepe University School of Medicine, Ankara, Turkey.

A case of a paraneoplastic vitelliform retinopathy is described in the fellow eye of a 40-year-old woman 4 years after enucleation of her left eye for a ciliochoroidal melanoma. There were multiple yellow subretinal vitelliform-like lesions at the posterior pole, which were hyperautofluorescent and hypofluorescent on fluorescein and indocyanine green angiographies. Spectral-domain optical coherence tomography showed hyperreflective material between the retinal pigment epithelium and the photoreceptor layer. Paraneoplastic vitelliform retinopathy may appear concomitantly with the development of distant metastases years after treatment of a ciliochoroidal melanoma and may present with symptoms resembling melanoma-associated retinopathy and electroretinogram findings. However, unlike with melanoma-associated retinopathy, the retinal lesions are located between the retinal pigment epithelium and the photoreceptor layer.
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http://dx.doi.org/10.3928/23258160-20130503-16DOI Listing
December 2013

Epitheloid hemangioendothelioma of the palpebral lobe of the lacrimal gland.

Orbit 2013 Apr 20;32(2):120-3. Epub 2013 Mar 20.

Ocular Oncology Service, Department of Ophthalmology, Hacettepe University School of Medicine, Ankara, Turkey.

Purpose: To describe a case of an epitheloid hemangioendothelioma which is a tumor of endothelial origin with borderline malignancy between hemangioma and angiosarcoma and which is very rare in the orbit.

Methods: Clinical features, results of imaging and histopathological studies and postoperative clinical course of a 22-year-old female patient who presented with a gradual swelling of the left upper eyelid were reviewed.

Results: Magnetic resonance imaging studies suggested a solid lesion with moderate contrast enhancement in the palpebral lobe of the lacrimal gland. A short course of systemic corticosteroid therapy failed to resolve the lesion. Excisional biopsy of the tumor yielded the diagnosis of epitheloid hemangioendothelioma of the lacrimal gland. The tumor was composed of nests of epitheloid cells, some forming CD31 positive intracytoplasmic vascular channels containing erythrocytes. CD34 and EMA were also positive and desmin, SMA, p63, S100, Factor VIII and HHV-8 were negative on immunohistochemical studies. During 44 months of follow-up, there has been no recurrence or systemic metastasis.

Conclusion: Epitheloid hemangioendothelioma can occur in the palpebral lobe of the lacrimal gland as a hard, painless, immobile mass. Simple excisional biopsy of the tumor, assumed to have an intermediate malignancy grade, without further local or systemic therapy provided a disease-free 3-year survival.
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http://dx.doi.org/10.3109/01676830.2013.764443DOI Listing
April 2013

Langerhans cell histiocytosis of the orbit.

Eur J Ophthalmol 2013 Jul-Aug;23(4):578-83. Epub 2013 Mar 6.

Department of Ophthalmology, Ocular Oncology Service, Hacettepe University School of Medicine, Ankara, Turkey.

Purpose: The management of Langerhans cell histiocytosis is controversial. This study evaluated our clinical experience and therapeutic results in orbital Langerhans cell histiocytosis.

Methods: This is a retrospective, noncomparative interventional case series involving 17 consecutive patients with biopsy-proven orbital Langerhans cell histiocytosis. Response to surgery and chemotherapy and development of diabetes insipidus were the main outcome measures.

Results: Thirteen (76.5%) of the patients were male and the mean age at diagnosis was 10.7 years (range 2-39 years). The most frequent presenting sign was proptosis (8 patients) and upper eyelid edema (4 patients). Pain was present in 5 cases and periocular redness in 6. No patient reported a history of trauma. The frontal bone was involved in 16 patients followed by the zygomatic in 9 cases. Five patients also had lesions in the calvarium, femur, facial, temporal, and parietal bones. No patients had systemic disease. Ten patients were managed with vinblastine (0.2 mg/kg, 6-12 months) chemotherapy because of major residual tumor burden (5 cases) and multi-bone involvement (5 cases). Four patients were observed following macroscopically complete tumor removal. Three patients with limited anterior orbital soft tissue tumors and single bone involvement received systemic corticosteroids (40 mg/m2/d, 6-10 weeks). No patients developed diabetes insipidus after a median follow-up of 46 months.

Conclusions: Macroscopically complete excision of the unifocal tumors may not necessitate any further treatment. Vinblastine chemotherapy following incomplete tumor removal and in patients with multifocal bone disease resulted in recurrence-free survival at 3 years in 90% of patients.
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http://dx.doi.org/10.5301/ejo.5000244DOI Listing
February 2014

Orbital chondrosarcoma arising from paranasal sinuses.

Int Ophthalmol 2013 Aug 6;33(4):403-7. Epub 2012 Nov 6.

Ocular Oncology Service, Department of Ophthalmology, Hacettepe University School of Medicine, Sıhhiye, Ankara, Turkey.

Orbital chondrosarcoma arising from paranasal sinuses or from the nasal cavity with orbital extension is highly unusual. The aim of this report is to describe our multidisciplinary experience in the treatment of three patients with extensive sino-orbital chondrosarcomas. The patients were aged 43, 75 and 63 years, and the tumors originated from the maxillary, sphenoidal, and ethmoidal sinuses, respectively. Magnetic resonance imaging studies showed homogeneously hypointense lesions on T1-weighted scans, which were hyperintense on T2-weighted images. Intranasal endoscopic surgery alone or in combination with other conventional surgical approaches was the main therapeutic choice. Two patients had recurrences treated with wider surgical removal. Fractionated stereotactic radiotherapy was used in two cases. Two patients had histopathological grade I tumor and one had grade III chondrosarcoma. No patient had regional spread or distant metastasis. All patients were disease-free at 4, 2, and 3 years, respectively, following their last therapeutic interventions. Endoscopic surgery results in lesser morbidity for chondrosarcomas arising from paranasal sinuses and extending into posterior parts of the orbit. However, because control of surgical margins may not be complete, recurrences may occur justifying more radical approaches.
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http://dx.doi.org/10.1007/s10792-012-9668-4DOI Listing
August 2013

Primary sebaceous carcinoma of the lacrimal gland.

Orbit 2012 Oct 21;31(5):352-4. Epub 2012 Aug 21.

Department of Ophthalmology, Hacettepe University School of Medicine, Ankara, Turkey.

Primary sebaceous carcinoma is an exceptionally rare tumor of the lacrimal gland and less than 10 cases have been so far published in the literature. Two adult patients aged 38 and 81 years, respectively, who suffered unilateral painful massive swelling of the lacrimal gland are described. The disease in the first patient initially manifested as ipsilateral parotid gland metastasis and the primary tumor could be detected 3 months later. Both tumors were rock hard and fixed on palpation, caused partial upper eyelid ptosis, displaced the globe anteromedially and impaired ocular motility. Magnetic resonance imaging studies showed mostly homogeneous, well-delineated and moderately contrast-enhancing lacrimal gland fossa tumors without bone destruction. The management consisted of incisional biopsy for the diagnosis, immediately followed by exenteration. The younger patient further underwent radical neck dissection, parotidectomy and orbital and neck radiotherapy, which provided him a 2-year disease-free survival. Histopathological examination showed poorly differentiated sebaceous carcinoma destructing completely the lacrimal gland with predominantly comedo pattern. Despite its rarity and lack of specific clinical and imaging signs, sebaceous carcinoma should be considered in rapidly evolving painful and hard lacrimal gland fossa tumors. Also noteworthy is the early propensity of this tumor to spread to regional draining lymph nodes and the parotid gland in particular.
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http://dx.doi.org/10.3109/01676830.2012.678921DOI Listing
October 2012

The treatment of retinoblastoma with four-drug regimen including cisplatin, etoposide, vincristine, and cyclophosphamide.

Pediatr Hematol Oncol 2012 Sep 3;29(6):529-37. Epub 2012 Jul 3.

Department of Pediatric Oncology, Institute of Oncology, Hacettepe University, Ankara, Turkey.

Over an 11-year period, 59 patients (83 eyes) were treated with four-drug chemotherapy (cisplatin, etoposide, cyclophosphamide, and vincristine) at Hacettepe University, Departments of Ophthalmology and Pediatric Oncology. We evaluated the clinical features, treatment modalities, and outcome of these patients with a median follow-up of 55 months (range 9-130 months). Enucleation was performed as a first-line treatment for 30 eyes due to iris neovascularization and neovascular glaucoma, tumor in the anterior chamber regardless of the tumor stage, and for the patients with the Reese-Ellsworth (RE) group Vb. Chemotherapy was given regardless of tumor stages according to the RE groups in all 59 patients (83 eyes). Fifty-three eyes were treated with chemoreduction (CRD) and focal treatment. The rates of globe preservation were 87% for bilateral tumors and 35% for unilateral tumors in the CRD group. The 5-year overall (OS) and enucleation-free survival (EnFS) was 86.9% and 40%, respectively, for the whole group. At 3rd year, ocular survival rate for the eyes with vitreal or subretinal seeding was 58% and without seeding was 66% (P = .78). Seeding or subretinal collection may not indicate poor prognosis under intensive chemotherapy. The intensive four-drug chemotherapy protocol might have satisfactory results in the retinoblastoma (RBL) patients.
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http://dx.doi.org/10.3109/08880018.2012.700387DOI Listing
September 2012

Recurrent subconjunctival hemorrhage due to cavernous hemangioma of the conjunctiva.

Can J Ophthalmol 2012 Jun 8;47(3):318-20. Epub 2012 May 8.

Ocular Oncology Service, Department of Ophthalmology, Ankara, Turkey.

Objective: To report 3 patients with conjunctival cavernous hemangioma in association with multiple recurrent episodes of subconjunctival hemorrhage.

Design: Retrospective clinical case series.

Participants: We studied 3 patients, 11, 13, and 21 years of age, respectively, each of whom had a small, solitary, and irregular multiloculated vascular mass on the temporal bulbar conjunctiva of the right eye. The tumours were excised for cosmetic reasons.

Methods: The medical records and histopathologic slides of the 3 patients were reviewed.

Results: Histopathologic examination showed that the tumours were composed of large, congested blood vessels separated by thin connective tissue. The patients did not have any recurrence of the tumour or hemorrhage.

Conclusions: Cavernous hemangioma had a tendency to develop rapidly in young people on the temporal bulbar conjunctiva of the right eye, causing recurrent subconjunctival hemorrhages.
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http://dx.doi.org/10.1016/j.jcjo.2012.02.002DOI Listing
June 2012