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.Key Words: Choroid, Optic nerve, Retinoblastoma, Eye 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 tümörle...

Histopathological Features of Group E Retinoblastoma Eyes after Upfront Enucleation

“…Histopathological high risk features in our group of patients, were found to be similar to other studies from India. 13 The reported incidence of choroidal invasion varies from 12% to 41% while optic nerve involvement with resection end range from 6.5% to 40%, discussed by Yousef et al 14 Similarly, current study showed majority (61.4%) of the eyes had choroidal invasion.…”

Metabolite profiling in retinoblastoma identifies novel clinicopathological subgroups

“…Conservative treatments such as cryotherapy and laser therapy, in some cases combined with systemic chemotherapy, are now well established for retinoblastomas classified as low grade (grades A–C according to the International Intraocular Classification of Retinoblastoma) ( Sastre et al , 2009 ). However, primary enucleation is more frequently the treatment of choice for high-grade tumours (grades D and E) as ∼25% of these will show adverse histopathological features following enucleation, for example, retrolaminar invasion of the optic nerve, massive choroidal invasion or involvement of the anterior chamber ( Yousef et al , 2014 ). These features are considered to present a significant risk for extraocular metastasis ( Shields and Shields, 2004 ) and thus patients also receive additional systemic chemotherapy post-enucleation.…”

Management outcome(s) in eyes with retinoblastoma previously inadequately treated with systemic chemotherapy alone without focal therapy

“…This is an option as long as the intraocular tumor does not harbor features of ICRB Group E eyes which are clinical features that suggest risk of extraocular spread since 18.5%–50% of enucleated Group E eyes harbor high-risk histopathologic features predisposing to an increased risk of systemic metastatic. [1213141516171819] Of interest, for eyes with Groups A, B, C, and D features, in our series (even with the delay of 9.5 months), not a single case had metastasis. This could be logically expected as these eyes did not harbor clinical features of tumor invasion to the vital structures such as the anterior chamber, ciliary body, choroid, or the optic nerve, and this also means that their management was justified mainly when the other eye was enucleated.…”

“…This could be logically expected as these eyes did not harbor clinical features of tumor invasion to the vital structures such as the anterior chamber, ciliary body, choroid, or the optic nerve, and this also means that their management was justified mainly when the other eye was enucleated. [14]…”