Ibrahim Nawaiseh

Team management, twinning, and telemedicine in retinoblastoma: A 3-tier approach implemented in the first eye salvage program in Jordan†

This study evaluated the outcome of retinoblastoma patients, when employing a telemedicine‐based twinning program in Jordan.

PREDICTIVE VALUE OF TNM CLASSIFICATION, INTERNATIONAL CLASSIFICATION, AND REESE-ELLSWORTH STAGING OF RETINOBLASTOMA FOR THE LIKELIHOOD OF HIGH-RISK PATHOLOGIC FEATURES.

Purpose: To evaluate the predictive value of the seventh edition American Joint Committee on Cancer (AJCC/UICC) TNM classification, the International Classification of Retinoblastoma (ICRB), and Reese–Ellsworth staging for retinoblastoma for the likelihood of high-risk pathologic features. Methods: A retrospective study of 50 primarily enucleated eyes from 49 retinoblastoma patients. Main outcome measures included demographics, TNM stage, ICRB group, Reese–Ellsworth stage, choroid, optic nerve, and anterior chamber invasion. Results: The median age at enucleation was 30 months. High-risk pathologic features mandating adjuvant chemotherapy were seen in 5 of T2 eyes (22%), in 15 of T3 eyes (56%) (P = 0.021), in 1 of ICRB Group C eyes (13%), 8 of Group D eyes (33%), and 11 of Group E eyes (61%) (P = 0.035). High-risk pathologic features were 4.61 and 3.68 times more likely to be diagnosed at a more advanced T stage and ICRB group consecutively, whereas 0.133 time less likely to be diagnosed at a more advanced Reese–Ellsworth stage. At median follow-up of 40 months, no single case had metastasis or was dead. Conclusion: The higher tumor clinical TNM stage and the more advanced ICRB group at presentation are associated with higher frequency of high-risk pathologic features and may predict which patients should receive adjuvant chemotherapy.

The impact of growth patterns of retinoblastoma (endophytic, exophytic, and mixed patterns).

ABSTRACT Objective: To study the impact of the retinoblastoma growth pattern (endophytic vs. exophytic) on the clinical and pathological features after primary enucleation. Material and Method: A retrospective case series of 42 eyes of 41 patients who had pathologically confirmed retinoblastoma. The main outcome measures included demographics, laterality, international intraocular retinoblastoma (IIRC) group, vitreous seeding, neovascular glaucoma, choroid invasion, optic nerve invasion, metastasis, and survival. Results: The median age at diagnosis was 30 months. Nineteen (46%) patients were males, and 17(41%) patients had bilateral retinoblastomas. Eight (19%) eyes were IIRC group C, 20 (48%) eyes were IIRC group D, and 14 (33%) eyes were IIRC group E. Nineteen (45%) tumors were endophytic, 14 (33%) were exophytic, and 9 (21%) were mixed. Choroid invasion was seen in 4 (21%) of the endophytic tumors, 5 (36%) of the exophytic tumors, and 8 (89%) of the mixed tumors (p=0.025). A mixed growth pattern was associated with massive choroid invasion in 5 (56%) of eyes. Neovascular glaucoma was seen in 5 (56%) of the mixed tumors (p=0.0376). Vitreous seeds were seen in 6 (67%) of the mixed tumors (p=0.0448). Optic nerve invasion as well as patients’ age at diagnosis, gender, and tumor laterality had no correlation with tumor growth pattern. At a median follow up of 36 months, no single case had metastasis or was dead. Conclusion: Exophytic tumors have higher risk of choroid invasion, while endophytic tumors have higher risk of vitreous seeding. The mixed tumor growth pattern is associated with more advanced IIRC group, more risk of neovascular glaucoma, and more risk of massive choroid invasion. ÖZ Amaç: Primer enükleasyon sonrası retinoblastomanın büyüme paternlerinin (endofitik, ekzofitik ve karışık) klinik ve patolojik özelliklere etkisini araştırmak. Gereç ve Yöntem: Retinoblastoması patolojik olarak onaylanmış 41 hastaya ait 42 gözden oluşan retrospektif olgu serisi. Dahil edilen ana ölçümler: Demografik bilgiler, lateralite, uluslararası intraoküler retinoblastoma grubu (UİRG/IIRC) sınıfandırması, vitröz yayılım, neovasküler glokom, koroid invazyonu, optik sinir invazyonu, metastaz ve yaşam süresi. Bulgular: Tanı anında medyan yaş 30 ay idi. Ondokuz (%46) hasta erkekti ve 17 (%41) hasta bilateral retinoblastomalıydı. Sekiz (%19) göz IIRC grup C, 20 (%48) göz IIRC grup D ve 14 göz IIRC grup E idi. Ondokuz (%45) tümör endofitik, 14 (%33) ekzofitik ve 9 (%21) karışıktır. Koroid inazyonu endofitik tümörlerin 4 (%21)’ünde, ekzofitik tümörlerin 5 (%36)’inde ve karışık tümörlerin 8 (%89)’inde görülmüştür (p=0,025). Karışık büyüme paterni gözlerin beşinde (%56) massif koroid invazyonu ile ilişkilidir. Neovasküler glokom karışık paternli tümörlerin 5 (%56)’inde görülmüştür (p=0,0376). Vitröz yayılım karışık tümörlerin 6 (%67)’sında görülmüştür (p=0,0448). Optik sinir invazyonu yanı sıra tanı anında yaş, cinsiyet ve tümör lateralitesi tümör büyüme paterni ile ilişkili değildir. Medyan 36 aylık takipte hiç bir olguda ölüm veya metastaz görülmemiştir. Sonuç: Ekzofitik tümörlerin daha fazla koroid invazyon riski, endofitik tümörlerin ise daha fazla vitröz yayılım riski vardır. Karışık büyüme paterni daha ileri IIRC grubu, daha fazla neovasküler glokom ve massif koroid invazyonu ile ilişkilidir.

Management and outcome of uveal melanoma in a single tertiary cancer center in Jordan

Abstract Objective: The aim of this study was evaluate the features and outcome of management of uveal melanoma in King Hussein Cancer Center as an example of a referral tertiary cancer center in the Middle East. Material and Method: This was aetrospective, observational case series of 46 eyes of 46 patients with uveal melanoma. Data collection required access to medical records, radiology and pathology reports, and laboratory results. The main outcome measures included age at diagnosis, tumor location and dimensions, TNM stage, treatment modality, visual outcome, metastasis, and mortality. Results: There was slight female preference, and the median age at diagnosis was 45 years. Eighteen (39%) eyes were treated by primary enucleation, and 28 (61%) eyes were treated by I-125 radioactive plaque. The melanoma was in the choroid in 40 (87%) eyes and in the ciliary body in 6 (13%) eyes, with no single tumor in the iris. According to the 7th edition of the American Joint Committee on Cancer staging system (UICC/AJCC); 8 (17%) were T1, 17 (36%) were T2, 16 (35%) were T3, and 5 (11%) were T4. One (2%) patient showed lymph node metastasis (N1), and 6 (13%) patients showed distant metastasis (M1). Pathologically, 2 (10%) of the enucleated eyes were spindle cell type, 4 (20%) were epithelioid cell type, and 14 (70%) were mixed type. Extrascleral extension was seen in three (15%) eyes, and optic nerve invasion in two (10%) eyes. After brachytherapy, 26 (93%) eyes were salvaged, and 2 eyes were consecutively enucleated; one for tumor recurrence, and one for uncontrolled painful neovascular glaucoma. The eye salvage rate post plaque was 93% (26/28), and the visual acuity for the salvaged eyes was equal or better than 0.5 in 11 (42%) eyes, 0.1-0.4 in 5 (19%) eyes, and less than 0.1 in 10 (38%) eyes. Conclusion: The incidence of uveal melanomas in our region is low compared to that in the West with a younger age at presentation. Candidate tumors for radioactive plaque therapy were successfully controlled in 93% of cases.

External Beam Radiation Therapy for RetinoblastomaResistant to Chemotherapy and Focal Treatment: Outcome and Predictive Factors

External Beam Radiation Therapy for Retinoblastoma Resistant to Chemotherapy and Focal Treatment: Outcome and Predictive Factors Abstract: Purpose: To evaluate the outcome of external beam radiation therapy (EBRT) for treatment of retinoblastoma resistant to chemotherapy and focal therapy. Methods and Materials: A retrospective case series of 24 eyes for 20 retinoblastoma patients treated by EBRT after failure of tumor control by chemotherapy and focal therapy. The main outcome measures included: international intraocular retinoblastoma stage (IIRC) and Reese Ellsworth (RE) stage, tumor seeding, treatment modalities, eye salvage, and survival. Results: The median age at diagnosis was 12 months. There were 12 (60%) males and 16 (80%) bilateral cases. All eyes were treated initially by systemic chemotherapy (range: 6-8 cycles). The dose of 45Gy was used in all eyes. The mean follow-up was 55months. Eye salvage rate after EBRT was 45% (11eyes); 67% ( 2/3) for IIRC group B, 63% (5/8) for group C, and 31% (4/13) for group D eyes. Vitreous seeds and tumor stage migration during management by chemotherapy were the most important significant predictive factors for tumor control (p=0.0327 and 0.0333 consecutively). Post EBRT complication rate was 80% (19/24) including; retinal detachment (3), vitreous hemorrhage (4), neovascular glaucoma (1), cataract (16), and radiation retinopathy (2). Conclusion: Eyes with retinoblastoma that failed chemotherapy were controlled with EBRT. However the presence of vitreous seeds, stage migration during chemotherapy, as well as good vision in the other eye may not justify the known risks of EBRT.

The Impact of Growth Patterns of Retinoblastoma (Endophytic, Exophytic, and Mixed Patterns) / Retinoblastomda Büyüme Paternlerinin Etkisi (Endofitik, Ekzofitik ve Karışık Paternler)

ABSTRACT Objective: To study the impact of the retinoblastoma growth pattern (endophytic vs. exophytic) on the clinical and pathological features after primary enucleation. Material and Method: A retrospective case series of 42 eyes of 41 patients who had pathologically confirmed retinoblastoma. The main outcome measures included demographics, laterality, international intraocular retinoblastoma (IIRC) group, vitreous seeding, neovascular glaucoma, choroid invasion, optic nerve invasion, metastasis, and survival. Results: The median age at diagnosis was 30 months. Nineteen (46%) patients were males, and 17(41%) patients had bilateral retinoblastomas. Eight (19%) eyes were IIRC group C, 20 (48%) eyes were IIRC group D, and 14 (33%) eyes were IIRC group E. Nineteen (45%) tumors were endophytic, 14 (33%) were exophytic, and 9 (21%) were mixed. Choroid invasion was seen in 4 (21%) of the endophytic tumors, 5 (36%) of the exophytic tumors, and 8 (89%) of the mixed tumors (p=0.025). A mixed growth pattern was associated with massive choroid invasion in 5 (56%) of eyes. Neovascular glaucoma was seen in 5 (56%) of the mixed tumors (p=0.0376). Vitreous seeds were seen in 6 (67%) of the mixed tumors (p=0.0448). Optic nerve invasion as well as patients’ age at diagnosis, gender, and tumor laterality had no correlation with tumor growth pattern. At a median follow up of 36 months, no single case had metastasis or was dead. Conclusion: Exophytic tumors have higher risk of choroid invasion, while endophytic tumors have higher risk of vitreous seeding. The mixed tumor growth pattern is associated with more advanced IIRC group, more risk of neovascular glaucoma, and more risk of massive choroid invasion. ÖZ Amaç: Primer enükleasyon sonrası retinoblastomanın büyüme paternlerinin (endofitik, ekzofitik ve karışık) klinik ve patolojik özelliklere etkisini araştırmak. Gereç ve Yöntem: Retinoblastoması patolojik olarak onaylanmış 41 hastaya ait 42 gözden oluşan retrospektif olgu serisi. Dahil edilen ana ölçümler: Demografik bilgiler, lateralite, uluslararası intraoküler retinoblastoma grubu (UİRG/IIRC) sınıfandırması, vitröz yayılım, neovasküler glokom, koroid invazyonu, optik sinir invazyonu, metastaz ve yaşam süresi. Bulgular: Tanı anında medyan yaş 30 ay idi. Ondokuz (%46) hasta erkekti ve 17 (%41) hasta bilateral retinoblastomalıydı. Sekiz (%19) göz IIRC grup C, 20 (%48) göz IIRC grup D ve 14 göz IIRC grup E idi. Ondokuz (%45) tümör endofitik, 14 (%33) ekzofitik ve 9 (%21) karışıktır. Koroid inazyonu endofitik tümörlerin 4 (%21)’ünde, ekzofitik tümörlerin 5 (%36)’inde ve karışık tümörlerin 8 (%89)’inde görülmüştür (p=0,025). Karışık büyüme paterni gözlerin beşinde (%56) massif koroid invazyonu ile ilişkilidir. Neovasküler glokom karışık paternli tümörlerin 5 (%56)’inde görülmüştür (p=0,0376). Vitröz yayılım karışık tümörlerin 6 (%67)’sında görülmüştür (p=0,0448). Optik sinir invazyonu yanı sıra tanı anında yaş, cinsiyet ve tümör lateralitesi tümör büyüme paterni ile ilişkili değildir. Medyan 36 aylık takipte hiç bir olguda ölüm veya metastaz görülmemiştir. Sonuç: Ekzofitik tümörlerin daha fazla koroid invazyon riski, endofitik tümörlerin ise daha fazla vitröz yayılım riski vardır. Karışık büyüme paterni daha ileri IIRC grubu, daha fazla neovasküler glokom ve massif koroid invazyonu ile ilişkilidir.

A histopathologic analysis of 50 eyes primarily enucleated for retinoblastoma in a tertiary cancer center in Jordan.

Abstract Objective: To analyze the histopathologic features of the eyes with intraocular retinoblastoma primarily treated by enucleation in a tertiary cancer center in Jordan. Material and Method: A retrospective case series of 50 eyes for 49 patients who had pathologically confirmed retinoblastoma after enucleation as primary therapy. The main outcome measures included demographics, laterality, international classification of intraocular retinoblastoma, choroid invasion, optic nerve invasion, anterior chamber invasion, and tumor differentiation. Results: The median age at enucleation was 30 months. Twenty-seven (55%) patients were males, and 19 (39%) patients had bilateral retinoblastoma. High risk pathological features were seen as massive choroid invasion in 9 (18%) eyes, post-laminar optic nerve invasion in 7 (14%) eyes, and anterior chamber, iris or ciliary body invasion in 7 (14%) eyes. Thirty-seven (74%) tumors were well/moderately differentiated, and 13 (26%) were poorly differentiated. Poorly differentiated tumors presented later (median 31 months) than well/ moderately differentiated tumors (26 months) and were associated with a higher incidence of high-risk pathological features. No single ICRB group C eye had high-risk pathological features, while 17% and 4% of group D eyes and 28% and 33% of group E eyes had massive choroid invasion and post-laminar optic nerve invasion, respectively. Eighteen (36%) patients received adjuvant chemotherapy for high risk pathological features, and at median follow up of 40 months, no single case had metastasis or was dead. Conclusion: Our pathologic findings were similar to the developed world. They were supportive of the predictive power of the international classification of retinoblastoma staging system for the likelihood of high risk pathological features. Poorly differentiated tumors were associated with a higher incidence of high risk pathological features than well/moderately differentiated tumors. Öz Amaç: Ürdün’ de üçüncü basamak kanser merkezinde primer olarak enükleasyon ile tedavi edilen intraoküler retinoblastomalı gözlerin histopatolojik özelliklerinin araştırılması. Gereç ve Yöntem: Primer tedavi olarak enükleasyon uygulandıktan sonra patolojiyle retinoblastoma tanısı alan 49 hastaya ait 50 gözden oluşan retrospektif olgu serisi. Demografik bulgular, lateralite, Uluslararası Retinoblastoma Sınıflaması, koroid invazyon, optik sinir invazyonu, ön kamera invazyonu ve tümör farklılaşması ana sonuç verileri olarak dahil edildi. Bulgular: Enükleasyon sırasında ortalama yaş 30 aydı. Yirmi yedi (%55) hasta erkekti ve 19 (%39) hastanın bilateral retinoblastoması vardı. Yüksek riskli patolojik özellikler; 9 (%18) gözde massif koroid invazyonu, 7’sinde (%14) post-laminar optik sinir invazyonu ve 7’sinde (%14) ön kamera, iris veya silier cisimcik invazyonu şeklindeydi. Otuz yedi (%74) tümör iyi/orta derecede diferansiye ve 13 (%26) az diferansiye idi. Az diferansiye tümörler (ortalama 31 ay), iyi/orta derecedeki tümörlere göre daha geç (ortalama 26 ay) ortaya çıkmakta ve yüksek riskli patolojik özellikler ile daha sıkı ilişki göstermektedir. Uluslararası Retinoblastoma Sınıflandırmasına göre grup D olanların %17 ve %4’ünde, grup E olanların %28 ve %33’ünde sırasıyla massif koroid invazyonu ve post-laminar optik sinir invazyonu varken, grup C olanlarda ise yüksek riskli patolojik özellik yoktu. On sekiz (%36) hasta yüksek riskli patolojik özellikler için adjuvan kemoterapi aldı ve 40 aylık ortalama takip süresince hiçbir olgunun metastazı yoktu ve hiçbiri ölmemişti. Sonuç: Sonuçlarımız, gelişmiş ülkelere benzerdir. Yüksek risk faktörleri için Uluslararası Retinoblastoma Sınıflandırma evreleme sisteminin öngörü değerini destekleyicidir. İyi/orta derecede diferansiye tümörlere göre az diferansiye tümörler, yüksek risk faktörleri ile daha sıkı ilişkilidir.

Chemoreduction of Progressive Intraocular Retinoblastoma by Systemic Topotecan

Abstract Purpose: To evaluate our experience with systemic Toptecan (TPT) chemotherapy as a second-line systemic chemotherapeutic regimen for treatment of refractory or recurrent intraocular retinoblastoma (RB). Methods and Materials: A retrospective case series of 14 eyes from patients with intraocular RB who received systemic TPT as second-line chemotherapy from April 2008 until June 2010. The following data were collected: patient demographics, laterality, international intraocular retinoblastoma stage (ICRB) at diagnosis, treatment received before and after TPT, side effects related to TPT, eye salvage, and survival. Results: The median age at diagnosis was 5 months (range, 1–16 months), and the median age at starting TPT was 10 months (range, 8–24 months). There were 6 (60%) females and 9 (90%) patients; all with bilateral retinoblastoma. The median number of TPT cycles was three per patient (range, 1–6), and the total number of administered cycles was 29. After TPT therapy; 4 (29%) eyes showed favorable response, 3 (21%) eyes showed minimal regression, 5 (36%) eyes had stable disease, and 2 (14%) eyes showed tumor progression. At a median follow-up of 48 months; 9 (64%) eyes were salvaged, 3 (21%) eyes received radiation therapy, and 3 (21%) eyes were enucleated (one was post radiation). Grade 3/4 neutropenia were noticed in a total of 59% of given cycles and admission for febrile neutropenia was required after seven cycles. Conclusions: Our report suggests that systemic TPT chemotherapy could be used as a salvage second-line regimen with low toxicity for patients with progressive intraocular retinoblastoma if systemic therapy is needed.

A Histopathologic Analysis of 50 Eyes Primarily Enucleated for Retinoblastoma in a Tertiary Cancer Center in Jordan Ürdün'de Üçüncü Basamak Kanser Merkezinde Retinoblastoma Nedeniyle Primer Enükleasyon Uygulanan 50 Gözün Histopatolojik İncelemesi

Abstract Objective: To analyze the histopathologic features of the eyes with intraocular retinoblastoma primarily treated by enucleation in a tertiary cancer center in Jordan. Material and Method: A retrospective case series of 50 eyes for 49 patients who had pathologically confirmed retinoblastoma after enucleation as primary therapy. The main outcome measures included demographics, laterality, international classification of intraocular retinoblastoma, choroid invasion, optic nerve invasion, anterior chamber invasion, and tumor differentiation. Results: The median age at enucleation was 30 months. Twenty-seven (55%) patients were males, and 19 (39%) patients had bilateral retinoblastoma. High risk pathological features were seen as massive choroid invasion in 9 (18%) eyes, post-laminar optic nerve invasion in 7 (14%) eyes, and anterior chamber, iris or ciliary body invasion in 7 (14%) eyes. Thirty-seven (74%) tumors were well/moderately differentiated, and 13 (26%) were poorly differentiated. Poorly differentiated tumors presented later (median 31 months) than well/ moderately differentiated tumors (26 months) and were associated with a higher incidence of high-risk pathological features. No single ICRB group C eye had high-risk pathological features, while 17% and 4% of group D eyes and 28% and 33% of group E eyes had massive choroid invasion and post-laminar optic nerve invasion, respectively. Eighteen (36%) patients received adjuvant chemotherapy for high risk pathological features, and at median follow up of 40 months, no single case had metastasis or was dead. Conclusion: Our pathologic findings were similar to the developed world. They were supportive of the predictive power of the international classification of retinoblastoma staging system for the likelihood of high risk pathological features. Poorly differentiated tumors were associated with a higher incidence of high risk pathological features than well/moderately differentiated tumors. Öz Amaç: Ürdün’ de üçüncü basamak kanser merkezinde primer olarak enükleasyon ile tedavi edilen intraoküler retinoblastomalı gözlerin histopatolojik özelliklerinin araştırılması. Gereç ve Yöntem: Primer tedavi olarak enükleasyon uygulandıktan sonra patolojiyle retinoblastoma tanısı alan 49 hastaya ait 50 gözden oluşan retrospektif olgu serisi. Demografik bulgular, lateralite, Uluslararası Retinoblastoma Sınıflaması, koroid invazyon, optik sinir invazyonu, ön kamera invazyonu ve tümör farklılaşması ana sonuç verileri olarak dahil edildi. Bulgular: Enükleasyon sırasında ortalama yaş 30 aydı. Yirmi yedi (%55) hasta erkekti ve 19 (%39) hastanın bilateral retinoblastoması vardı. Yüksek riskli patolojik özellikler; 9 (%18) gözde massif koroid invazyonu, 7’sinde (%14) post-laminar optik sinir invazyonu ve 7’sinde (%14) ön kamera, iris veya silier cisimcik invazyonu şeklindeydi. Otuz yedi (%74) tümör iyi/orta derecede diferansiye ve 13 (%26) az diferansiye idi. Az diferansiye tümörler (ortalama 31 ay), iyi/orta derecedeki tümörlere göre daha geç (ortalama 26 ay) ortaya çıkmakta ve yüksek riskli patolojik özellikler ile daha sıkı ilişki göstermektedir. Uluslararası Retinoblastoma Sınıflandırmasına göre grup D olanların %17 ve %4’ünde, grup E olanların %28 ve %33’ünde sırasıyla massif koroid invazyonu ve post-laminar optik sinir invazyonu varken, grup C olanlarda ise yüksek riskli patolojik özellik yoktu. On sekiz (%36) hasta yüksek riskli patolojik özellikler için adjuvan kemoterapi aldı ve 40 aylık ortalama takip süresince hiçbir olgunun metastazı yoktu ve hiçbiri ölmemişti. Sonuç: Sonuçlarımız, gelişmiş ülkelere benzerdir. Yüksek risk faktörleri için Uluslararası Retinoblastoma Sınıflandırma evreleme sisteminin öngörü değerini destekleyicidir. İyi/orta derecede diferansiye tümörlere göre az diferansiye tümörler, yüksek risk faktörleri ile daha sıkı ilişkilidir.

Ürdün'de üçüncü basamak kanser merkezinde retinoblastoma nedeniyle primer enükleasyon uygulanan 50 gözün histopatolojik İncelemesi

Abstract Objective: To analyze the histopathologic features of the eyes with intraocular retinoblastoma primarily treated by enucleation in a tertiary cancer center in Jordan. Material and Method: A retrospective case series of 50 eyes for 49 patients who had pathologically confirmed retinoblastoma after enucleation as primary therapy. The main outcome measures included demographics, laterality, international classification of intraocular retinoblastoma, choroid invasion, optic nerve invasion, anterior chamber invasion, and tumor differentiation. Results: The median age at enucleation was 30 months. Twenty-seven (55%) patients were males, and 19 (39%) patients had bilateral retinoblastoma. High risk pathological features were seen as massive choroid invasion in 9 (18%) eyes, post-laminar optic nerve invasion in 7 (14%) eyes, and anterior chamber, iris or ciliary body invasion in 7 (14%) eyes. Thirty-seven (74%) tumors were well/moderately differentiated, and 13 (26%) were poorly differentiated. Poorly differentiated tumors presented later (median 31 months) than well/ moderately differentiated tumors (26 months) and were associated with a higher incidence of high-risk pathological features. No single ICRB group C eye had high-risk pathological features, while 17% and 4% of group D eyes and 28% and 33% of group E eyes had massive choroid invasion and post-laminar optic nerve invasion, respectively. Eighteen (36%) patients received adjuvant chemotherapy for high risk pathological features, and at median follow up of 40 months, no single case had metastasis or was dead. Conclusion: Our pathologic findings were similar to the developed world. They were supportive of the predictive power of the international classification of retinoblastoma staging system for the likelihood of high risk pathological features. Poorly differentiated tumors were associated with a higher incidence of high risk pathological features than well/moderately differentiated tumors. Öz Amaç: Ürdün’ de üçüncü basamak kanser merkezinde primer olarak enükleasyon ile tedavi edilen intraoküler retinoblastomalı gözlerin histopatolojik özelliklerinin araştırılması. Gereç ve Yöntem: Primer tedavi olarak enükleasyon uygulandıktan sonra patolojiyle retinoblastoma tanısı alan 49 hastaya ait 50 gözden oluşan retrospektif olgu serisi. Demografik bulgular, lateralite, Uluslararası Retinoblastoma Sınıflaması, koroid invazyon, optik sinir invazyonu, ön kamera invazyonu ve tümör farklılaşması ana sonuç verileri olarak dahil edildi. Bulgular: Enükleasyon sırasında ortalama yaş 30 aydı. Yirmi yedi (%55) hasta erkekti ve 19 (%39) hastanın bilateral retinoblastoması vardı. Yüksek riskli patolojik özellikler; 9 (%18) gözde massif koroid invazyonu, 7’sinde (%14) post-laminar optik sinir invazyonu ve 7’sinde (%14) ön kamera, iris veya silier cisimcik invazyonu şeklindeydi. Otuz yedi (%74) tümör iyi/orta derecede diferansiye ve 13 (%26) az diferansiye idi. Az diferansiye tümörler (ortalama 31 ay), iyi/orta derecedeki tümörlere göre daha geç (ortalama 26 ay) ortaya çıkmakta ve yüksek riskli patolojik özellikler ile daha sıkı ilişki göstermektedir. Uluslararası Retinoblastoma Sınıflandırmasına göre grup D olanların %17 ve %4’ünde, grup E olanların %28 ve %33’ünde sırasıyla massif koroid invazyonu ve post-laminar optik sinir invazyonu varken, grup C olanlarda ise yüksek riskli patolojik özellik yoktu. On sekiz (%36) hasta yüksek riskli patolojik özellikler için adjuvan kemoterapi aldı ve 40 aylık ortalama takip süresince hiçbir olgunun metastazı yoktu ve hiçbiri ölmemişti. Sonuç: Sonuçlarımız, gelişmiş ülkelere benzerdir. Yüksek risk faktörleri için Uluslararası Retinoblastoma Sınıflandırma evreleme sisteminin öngörü değerini destekleyicidir. İyi/orta derecede diferansiye tümörlere göre az diferansiye tümörler, yüksek risk faktörleri ile daha sıkı ilişkilidir.