Extensively Necrotic Retinoblastoma Is Associated With High-Risk Prognostic Factors

2006 ◽  
Vol 130 (11) ◽  
pp. 1669-1672 ◽  
Author(s):  
Eva-Marie Chong ◽  
Robert E. Coffee ◽  
Murali Chintagumpala ◽  
Richard L. Hurwitz ◽  
Mary Y. Hurwitz ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Optic Nerve ◽  
High Risk ◽  
Metastatic Disease ◽  
Tumor Necrosis ◽  
Patient Survival ◽  
Nerve Invasion ◽  
Choroidal Invasion ◽  
Extensive Necrosis ◽  
Histopathologic Features

Abstract Context.—Retinoblastoma is the most common malignant intraocular tumor in children. It has been shown that adjuvant therapy following enucleation in patients with high-risk histopathologic features significantly decreases the mortality. We describe the association of extensive necrosis of tumor and intraocular structures with 2 of the major risk factors: optic nerve invasion and choroidal invasion. This may alert the pathologist who makes the observation of extensive necrosis to carefully search for histologic features associated with adverse outcome. Objective.—To determine whether extensively necrotic retinoblastoma is associated with high-risk histologic prognostic factors for metastatic disease and patient survival. Design.—Retrospective case series. Forty-three eyes of 43 patients with retinoblastoma who underwent enucleation between 1990 and 2001 were evaluated. Medical records, histopathology specimens, pathology reports, and clinical photographs were reviewed. Tumors were designated as exhibiting extensive necrosis if more than 95% of tumor cells and intraocular tissues were necrotic. The main outcome measure was the association of extensive tumor necrosis with 3 high-risk histopathologic features: extraocular extension, optic nerve invasion, or choroidal invasion. Metastatic disease, patient survival, and associations with pathologic findings were also analyzed. Results.—Optic nerve head invasion (P < .001), post-lamina-cribrosal invasion (P < .001), and choroidal invasion by tumor (P = .004) were observed more frequently in eyes with extensive necrosis compared with eyes without extensive necrosis. Two of the 11 patients with extensively necrotic intraocular retinoblastoma died from metastatic disease (P = .06). None of the 32 patients without extensive necrosis developed metastatic disease or died. Conclusions.—Extensive ocular tissue and tumor necrosis is associated with histologic high-risk prognostic factors for tumor metastasis and mortality.

The predictive value of magnetic resonance imaging of retinoblastoma for the likelihood of high-risk pathologic features

2018 ◽  
Vol 29 (2) ◽  
pp. 262-268 ◽  
Author(s):  
Jamila G Hiasat ◽  
Alaa Saleh ◽  
Maysa Al-Hussaini ◽  
Ibrahim Al Nawaiseh ◽  
Mustafa Mehyar ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Optic Nerve ◽  
High Risk ◽  
Magnetic Resonance ◽  
Predictive Value ◽  
Resonance Imaging ◽  
Cut Edge ◽  
Pathologic Features ◽  
Nerve Invasion ◽  
Choroidal Invasion

Purpose: To evaluate the predictive value of magnetic resonance imaging in retinoblastoma for the likelihood of high-risk pathologic features. Methods: A retrospective study of 64 eyes enucleated from 60 retinoblastoma patients. Contrast-enhanced magnetic resonance imaging was performed before enucleation. Main outcome measures included demographics, laterality, accuracy, sensitivity, and specificity of magnetic resonance imaging in detecting high-risk pathologic features. Results: Optic nerve invasion and choroidal invasion were seen microscopically in 34 (53%) and 28 (44%) eyes, respectively, while they were detected in magnetic resonance imaging in 22 (34%) and 15 (23%) eyes, respectively. The accuracy of magnetic resonance imaging in detecting prelaminar invasion was 77% (sensitivity 89%, specificity 98%), 56% for laminar invasion (sensitivity 27%, specificity 94%), 84% for postlaminar invasion (sensitivity 42%, specificity 98%), and 100% for optic cut edge invasion (sensitivity100%, specificity 100%). The accuracy of magnetic resonance imaging in detecting focal choroidal invasion was 48% (sensitivity 33%, specificity 97%), and 84% for massive choroidal invasion (sensitivity 53%, specificity 98%), and the accuracy in detecting extrascleral extension was 96% (sensitivity 67%, specificity 98%). Conclusions and relevance: Magnetic resonance imaging should not be the only method to stratify patients at high risk from those who are not, eventhough it can predict with high accuracy extensive postlaminar optic nerve invasion, massive choroidal invasion, and extrascleral tumor extension.

scholarly journals Retinoblastoma: An institutional experience

2015 ◽  
Vol 5 (9) ◽  
pp. 723-726
Author(s):  
S Karki ◽  
P Upadhyaya ◽  
M Agarwal ◽  
KK Maharjan ◽  
P Lavaju
Keyword(s):  
Optic Nerve ◽  
High Risk ◽  
Tumor Staging ◽  
Tertiary Care ◽  
Prognostic Significance ◽  
Routine Practice ◽  
Ocular Involvement ◽  
Department Of Ophthalmology ◽  
Nerve Invasion ◽  
Choroidal Invasion

Background: This article aims to describe histopathologic high risk tumor characteristics in our patient population of retinoblastoma. It is based on consensus criteria for definitions of choroidal and optic nerve invasion as outlined in The International RetinoblastomaStaging Working Group (IRSWG) 2009.Materials and Methods: Fifty histopathologically diagnosed cases of retinoblastoma were archived from records of Pathology department during years 2004 to 2014. Re-evaluation of slides to identify choroidal and optic nerve invasion as per IRSWG along with Pathologic tumor staging was done. Data were entered into Microsoft excel sheets and results expressed in percentages. Department of Ophthalmology was consulted for recurrence of Retinoblastoma.Results: Among fifty cases, Choroidal invasion was absent in 62% cases. Minimal invasion (<3mm) was seen in 18% cases, massive (>3mm) in 14% cases and extra ocular involvement in 6% cases. The optic nerve was free of tumor in more than three forth of the cases (78%). Prelaminar and retro laminar involvement of optic nerve was observed in 6% and 10% cases respectively. Intraocular spread of tumor was observed in 6% of cases. The cut margin of optic nerve was involved in 42% while it was free of tumor in 58% of cases. Significant number of tumours were pathologically classified as pT1 (58%) followed by pT2a (22%). pT3a and pT4b were found in 6% each and pT3b and pT4a were found in 4% each. Recurrence was observed in two cases of PT3a and one of pT4b.Conclusion: We conclude identifying low percentages of high risk charateristics in a retrospective histologic experience with Retinoblastoma. Recurrence observed in two tumours staged pT3a sheds light on prognostic significance of reporting massive choroidal invasion despite free cut margin. These observations call for routine practice of standardized histopathologic reporting and processing of enucleated eye samples at our tertiary care centre.Journal of Pathology of Nepal (2015) Vol. 5, 723-726

scholarly journals Histopathological assessment of optic nerve invasion guided by radiological findings in enucleated globes with retinoblastoma.

2020 ◽  
Author(s):  
Mohammed Abusayf ◽  
Hind Manaa Alkatan ◽  
Sahar Elkhamary ◽  
Saleh Almesfer ◽  
Azza MY Maktabi
Keyword(s):  
Optic Nerve ◽  
High Risk ◽  
Predictive Value ◽  
Histopathological Examination ◽  
Demographic Data ◽  
Mri Findings ◽  
Level Of Invasion ◽  
Nerve Invasion ◽  
Unilateral Involvement ◽  
Magnetic Resonance Imaging Mri

Abstract Background: Optic nerve (ON) invasion is an important high-risk feature, and an indicator for neoadjuvant chemotherapy and prognosis. We aim through this study to correlate the detected-ON invasion by Magnetic resonance imaging (MRI) with the corresponding confirmed histopathological level of invasion. Methods: A retrospective study of enucleated globes with the diagnosis of retinoblastoma received in the histopathology department(s) from January 2015 to December 2016 (2 years). Slides were reviewed for ON invasion assessment, charts were reviewed for basic demographic data. All patients underwent MRI under sedation upon diagnosis and MRI findings were collected for the above correlation. Results: A total of 38 patients were included: 21 males and 17 females. 29 (77.3%) had unilateral involvement, 7 (18.4%) had bilateral involvement and 2 cases had trilateral disease. The overall mean age at diagnosis was 22.63 ±15.15 months. Histopathological examination revealed ON invasion in 28 cases (74%) distributed as follows: prelaminar (31.6%), laminar (18.4%), and post-laminar (23.7%). MRI confirmed post-laminar ON invasion in 8 cases (true positive) but failed to detect this in 1 case. Additionally, MRI detected another 8 cases of ON invasion that were false positive on histopathology (accuracy: 63.3%; sensitivity: 88.9%; specificity: 72.4%; Positive predictive value (PPV): 50%; Negative predictive value (NPV): 95.5%). Conclusions: MRI is found to be less sensitive in evaluating prelaminar and laminar ON invasion (0.0 and 42.9%) compared to post-laminar invasion (88.9%). MRI has generally better specificity in detecting ON invasion irrespective of the invasion level. In our study, obtaining deeper and/or additional histologic sections from the other surface of the tissue block in cases where a post-laminar ON invasion by MRI is found but not confirmed histopathologically in routine sections is essential to avoid missing such an important high-risk feature.

scholarly journals Diagnostic Accuracy of Intraocular Tumor Size Measured with MR Imaging in the Prediction of Postlaminar Optic Nerve Invasion and Massive Choroidal Invasion of Retinoblastoma

Radiology ◽  
2016 ◽  
Vol 279 (3) ◽  
pp. 817-826 ◽  
Author(s):  
Marcus C. De Jong ◽  
Fenna J. S. van der Meer ◽  
Sophia L. Göricke ◽  
Hervé J. Brisse ◽  
Paolo Galluzzi ◽  
...  
Keyword(s):  
Optic Nerve ◽  
Mr Imaging ◽  
Diagnostic Accuracy ◽  
Tumor Size ◽  
Intraocular Tumor ◽  
Nerve Invasion ◽  
Choroidal Invasion

scholarly journals Clinical and histopathological correlation: A study on 334 eyes with retinoblastoma from Vietnam

2016 ◽  
Vol 16 (1) ◽  
pp. 23-34
Author(s):  
Ha Ngan Nguyen ◽  
Van Trong Pham ◽  
Tuan Anh Hoang ◽  
Van Hong Pham ◽  
Chung Ngoc Nguyen ◽  
...  
Keyword(s):  
Optic Nerve ◽  
High Risk ◽  
Clinical Features ◽  
Risk Group ◽  
Clinical Signs ◽  
Group Versus ◽  
High Risk Group ◽  
Iris Neovascularization ◽  
Nerve Invasion ◽  
Vitreous Seeding

Purpose: To elucidate the clinical features which predict high-risk histopathological factors for subsequent metastatic disease as well as to report the incidence of these high-risk histopathological factors in a cohort of Asian patients with retinoblastoma. Design: A retrospective and non-randomized sequential cases series. Methods: A retrospective study was done on 334 eyes with retinoblastoma at Vietnam National Institute of Ophthalmology during a 10 year period (January 2004 – December 2013). All pathology specimens and medical records were reviewed and assessed for invasion and clinical signs. Results: Among 334 eyes, 225 (67.4%) had high-risk retinoblastoma and 109 (22.6%) had non-high-risk features on histopathology. The high-risk histopathological features included anterior chamber seeding (48.2%), iris infiltration (14.7%), ciliary body involvement (14.1%), massive choroidal invasion (29.9 %), post-laminar optic nerve invasion (21.2%), invasion of optic nerve transection (9.6 %), combined choroidal and optic nerve invasion (9.6 %), scleral invasion (3.3%), and extra-scleral infiltration (11.4%). The significant clinical features in high-risk group versus non-high-risk group included hyphema (19.6% vs 3.7%, p < 0.001), pseudohypopyon (19.1% vs 6.4%, p = 0.001), iris neovascularization  (25.3% vs 5.5%, p < 0.001), vitreous seeding (72.4% vs 37.6%, p < 0.001), staphyloma (24% vs 4.6%, p < 0.001) and scleritis (20% vs 3.7%, p < 0.001). Conclusions: Clinical signs including hyphema, pseudohypopyon, iris neovascularization, vitreous seeding, staphyloma, and scleritis were significantly associated with high-risk features on histopathology. Globe preserving methods should be used with caution in patients with these signs.  

scholarly journals Histopathological assessment of optic nerve invasion guided by radiological findings in enucleated globes with retinoblastoma.

2019 ◽  
Author(s):  
Mohammed Abusayf ◽  
Hind Manaa Alkatan ◽  
Azza MY Maktabi ◽  
Sahar Elkhamary ◽  
Saleh Almesfer
Keyword(s):  
Magnetic Resonance Imaging ◽  
Optic Nerve ◽  
High Risk ◽  
Magnetic Resonance ◽  
Predictive Value ◽  
Resonance Imaging ◽  
Important Indicator ◽  
Histopathological Features ◽  
Level Of Invasion ◽  
Nerve Invasion

Abstract Purpose: High-risk histopathological features in primary enucleated retinoblastoma globes affect the indication for neoadjuvant chemotherapy to avoid the increased risk of metastasis and to improve survival in such children. Optic nerve (ON) invasion is an important indicator of prognosis and we aim through this study to correlate the detected-ON invasion by imaging with the corresponding confirmed histopathological level of invasion. Methods: This is an approved retrospective study with collaborative agreement between the 2 centres. All enucleated globes with the diagnosis of retinoblastoma received in the histopathology department(s) from January 2015 to December 2016 (2 years) were collected for review by 2 pathologists and the charts were reviewed for basic demographic data. All patients underwent Magnetic resonance imaging (MRI) under sedation upon diagnosis and MRI reading was done by an experienced single neuroradiologist. Results: A total of 38 patients were included: 21 males and 17 females. 29 (77.3%) had unilateral involvement, 7 (18.4%) had bilateral involvement and 2 cases had trilateral disease. The overall mean age at diagnosis was 22.63 ±15.15 months. 28 cases (74%) had pathologically-proven ON invasion: prelaminar (31.6%), laminar (18.4%), and post-laminar (23.7%). Post-laminar ON invasion was identified in 8 cases (true positive), while another 8 were false positive. Only 1 case was missed on MRI (accuracy: 63.3%; sensitivity: 88.9%; specificity: 72.4%; Positive predictive value (PPV): 50%; Negative predictive value (NPV): 95.5%). Conclusions: MRI was found to be less sensitive in evaluating pre-laminar and laminar ON invasion (0.0 and 42.9%) compared to post-laminar invasion (88.9%) but had better specificity in detecting various levels of invasion (72.4 to 83.9%). Obtaining deeper histologic sections in some cases where there are mismatching ON level of invasion between MRI and histopathological examination is recommended to avoid misleading assessment of the high-risk histopathological features. There are no solid international protocols of how many sections should be obtained to evaluate the optic nerve invasion in these globes and this should be established for universal use especially in less developed countries where experienced pathologists might be lacking. Key words: Retinoblastoma; High-risk, Histopathology; Optic nerve invasion; Lamina cribrosa; Radiology; Magnetic resonance imaging; Chemotherapy.

scholarly journals Histopathological assessment of optic nerve invasion guided by radiological findings in enucleated globes with retinoblastoma

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Mohammed M. Abusayf ◽  
Hind M. Alkatan ◽  
Sahar Elkhamary ◽  
Saleh A. Almesfer ◽  
Azza M. Y. Maktabi
Keyword(s):  
Optic Nerve ◽  
High Risk ◽  
Predictive Value ◽  
Histopathological Examination ◽  
Demographic Data ◽  
Mri Findings ◽  
Level Of Invasion ◽  
Nerve Invasion ◽  
Unilateral Involvement ◽  
Magnetic Resonance Imaging Mri

Abstract Background Optic nerve (ON) invasion is an important high-risk feature, and an indicator for neoadjuvant chemotherapy and prognosis. We aim through this study to correlate the detected-ON invasion by Magnetic resonance imaging (MRI) with the corresponding confirmed histopathological level of invasion. Methods A retrospective study of enucleated globes with the diagnosis of retinoblastoma received in the histopathology department(s) from January 2015 to December 2016 (2 years). Slides were reviewed for ON invasion assessment, charts were reviewed for basic demographic data. All patients underwent MRI under sedation upon diagnosis and MRI findings were collected for the above correlation. Results A total of 38 patients were included: 21 males and 17 females. 29 (77.3%) had unilateral involvement, 7 (18.4%) had bilateral involvement and 2 cases had trilateral disease. The overall mean age at diagnosis was 22.63 ± 15.15 months. Histopathological examination revealed ON invasion in 28 cases (74%) distributed as follows: prelaminar (31.6%), laminar (18.4%), and post-laminar (23.7%). MRI confirmed post-laminar ON invasion in 8 cases (true positive) but failed to detect this in 1 case. Additionally, MRI detected another 8 cases of ON invasion that were false positive on histopathology (accuracy: 63.3%; sensitivity: 88.9%; specificity: 72.4%; Positive predictive value (PPV): 50%; Negative predictive value (NPV): 95.5%). Conclusions MRI is found to be less sensitive in evaluating prelaminar and laminar ON invasion (0.0 and 42.9%) compared to post-laminar invasion (88.9%). MRI has generally better specificity in detecting ON invasion irrespective of the invasion level. In our study, obtaining deeper and/or additional histologic sections from the other surface of the tissue block in cases where a post-laminar ON invasion by MRI is found but not confirmed histopathologically in routine sections is essential to avoid missing such an important high-risk feature.

scholarly journals Adjuvant therapy of histopathological risk factors of retinoblastoma in Europe: a survey by the European Retinoblastoma group (EURbG)

2020 ◽  
Author(s):  
Sabine Dittner-Moormann ◽  
Madlen Reschke ◽  
Floor Abbink ◽  
Isabelle Aerts ◽  
Hatice Atalay ◽  
...  
Keyword(s):  
Risk Factors ◽  
Optic Nerve ◽  
Adjuvant Therapy ◽  
Adjuvant Treatment ◽  
Online Survey ◽  
Treatment Method ◽  
Optimal Choice ◽  
Treatment Regimens ◽  
Nerve Invasion ◽  
Choroidal Invasion

Introduction: Advanced intraocular retinoblastoma can be cured by enucleation, but spread of retinoblastoma cells beyond natural limits of the eye may lead to metastatic disease which is related to a high mortality. Adjuvant therapy after enucleation has been shown to prevent metastasis in children with risk factors for extraocular retinoblastoma. However, histological criteria and adjuvant treatment regimens vary and there is no unifying consensus on the optimal choice of treatment. Method: Data on guidelines for adjuvant treatment in European retinoblastoma referral centres were collected in an online survey among all members of the European Retinoblastoma group (EURbG) network. Extended information were gathered via personal Email communication. Results: Data were collected from 26 centres in 17 countries. Guidelines for adjuvant treatment were in place at 92.3% of retinoblastoma centres. There was a consensus on indication for and intensity of adjuvant treatment among more than 80% of all centres. The majority of centres use no adjuvant treatment for isolated focal choroidal invasion or prelaminar optic nerve invasion. Patients with massive choroidal invasion or postlaminar optic nerve invasion receive adjuvant chemotherapy, while microscopic invasion of the resection margin of the optic nerve or extension through the sclera are treated with combined chemo- and radiotherapy. Conclusion: Indications and adjuvant treatment regimens in European retinoblastoma referral centres are similar but not uniform. Further biomarkers in addition to histopathological risk factors could improve treatment stratification. The high consensus in European centres is an excellent foundation for a common European study with prospective validation of new biomarkers.

scholarly journals Characteristics and Patterns of Metastatic Disease from Chordoma

Sarcoma ◽  
2015 ◽  
Vol 2015 ◽  
pp. 1-7 ◽  
Author(s):  
Victoria A. Young ◽  
Kevin M. Curtis ◽  
H. Thomas Temple ◽  
Frank J. Eismont ◽  
Thomas F. DeLaney ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Local Recurrence ◽  
Median Survival ◽  
Metastatic Disease ◽  
Malignant Tumor ◽  
Retrospective Review ◽  
Patient Survival ◽  
Initial Diagnosis ◽  
Patient Records ◽  
Slow Growing

Chordoma is a rare, slow-growing malignant tumor arising from notochordal remnants. A retrospective review of patient records at two major referral centers was undertaken to assess the incidence, location, and prognostic factors of metastatic disease from chordoma. 219 patients with chordoma (1962–2009) were identified. 39 patients (17.8%) developed metastatic disease, most frequently to lung (>50%). Median survival from the time of initial diagnosis was 130.4 months for patients who developed metastatic disease and 159.3 months for those who did not (P=0.05). Metastatic disease was most common in the youngest patients (P=0.07), and it was 2.5 times more frequent among patients with local recurrence (26.3%) than in those without (10.8%) (P=0.003). Patient survival with metastatic disease was highly variable, and it was dependent on both the location of the tumor primary and the site of metastasis. Metastasis to distal bone was the most rapid to develop and had the worst prognosis.

scholarly journals Study of Unilateral Retinoblastoma With and Without Histopathologic High-Risk Features and the Role of Adjuvant Chemotherapy: A Children’s Oncology Group Study

2019 ◽  
Vol 37 (31) ◽  
pp. 2883-2891 ◽  
Author(s):  
Patricia Chévez-Barrios ◽  
Ralph C. Eagle ◽  
Mark Krailo ◽  
Jin Piao ◽  
Daniel M. Albert ◽  
...  
Keyword(s):  
Optic Nerve ◽  
High Risk ◽  
Adjuvant Chemotherapy ◽  
Nerve Involvement ◽  
Nerve Invasion ◽  
Adjuvant Chemotherapy Regimen ◽  
Metastatic Risk ◽  
Not At Risk ◽  
Optic Nerve Involvement

PURPOSE To prospectively determine the prevalence of high-risk histopathologic features (HRFs) in patients with unilateral retinoblastoma who undergo enucleation and to evaluate the role of chemotherapy in preventing recurrences. PATIENTS AND METHODS Children newly diagnosed with enucleated unilateral retinoblastoma were enrolled prospectively. After central histopathology review, patients with specific HRFs received chemotherapy; others were observed. Primary end points were event-free survivals (EFS). RESULTS Of the 331 patients enrolled during 2005 to 2010, 321 eligible patients had central histopathologic review. Discordance between central review and contributing institutions occurred in 23% of patients with HRFs and in 17% of patients without HRFs. Postlaminar optic nerve involvement was present in 53 patients; 42 had massive posterior uveal invasion (≥ 3 mm); 15 had concomitant peripapillary 3 mm or greater choroid and postlaminar optic nerve involvement; and 15 had focal (< 3 mm) choroidal concomitant with lamina or prelamina optic nerve involvement. Two-year EFS for patients with HRFs requiring adjuvant chemotherapy was 0.96 (95% CI, 0.89 to 0.98), and 2-year EFS for patients without HRFs for which observation was indicated was 0.99 (95% CI, 0.96 to 1.0). The 2-year EFS for all patients was 0.98 (95% CI, 0.96 to 0.99). CONCLUSION Adequate handling and interpretation of histopathology of eyes with retinoblastoma is necessary to assign metastatic risk. Concomitant less than 3 mm choroidal and any prelaminar/laminar optic nerve invasion show no recurrence and may warrant no adjuvant chemotherapy. In contrast, concomitant greater than 3 mm peripapillary choroidal invasion and 1.5 mm or greater of postlaminar optic nerve invasion have the poorest outcomes, supporting the need for a more intensive adjuvant chemotherapy regimen for this subgroup. Strict criteria for adjuvant therapy may improve outcomes of children who undergo enucleation at diagnosis and may avoid unnecessary adjuvant chemotherapy for those who are not at risk for recurrence.

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