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Nag A, Khetan V. Retinoblastoma - A comprehensive review, update and recent advances. Indian J Ophthalmol 2024; 72:778-788. [PMID: 38804799 PMCID: PMC11232864 DOI: 10.4103/ijo.ijo_2414_23] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/01/2023] [Revised: 11/28/2023] [Accepted: 12/02/2023] [Indexed: 05/29/2024] Open
Abstract
Retinoblastoma is the most common pediatric ocular malignancy. It is triggered by a biallelic mutation in the RB1 gene or MYCN oncogene amplification. Retinoblastomas can be unilateral (60%-70%) or bilateral (30%-40%); bilateral tumors are always heritable and present at an earlier age as compared to unilateral ones (18-24 months vs. 36 months in India). High prevalence rates, delayed presentation, and inaccessibility to healthcare lead to worse outcomes in developing countries. The past few decades have seen a paradigm change in the treatment of retinoblastomas, shifting from enucleation and external beam radiotherapy to less aggressive modalities for eye salvage. Multimodality treatment is now the standard of care and includes intraarterial or intravenous chemotherapy along with focal consolidation therapies such as transpupillary thermotherapy, cryotherapy, and laser photocoagulation. Intravitreal and intracameral chemotherapy can help in controlling intraocular seeds. Advanced extraocular or metastatic tumors still have a poor prognosis. Genetic testing, counseling, and screening of at-risk family members must be incorporated as essential parts of management. A better understanding of the genetics and molecular basis of retinoblastoma has opened up the path for potential targeted therapy in the future. Novel recent advances such as liquid biopsy, prenatal diagnosis, prognostic biomarkers, tylectomy, and chemoplaque point to promising future directions.
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Affiliation(s)
- Adwaita Nag
- Ocular Oncology Service, Department of Ophthalmology and Vision Sciences, University of Toronto, Princess Margaret Cancer Centre, Toronto, ON, Canada
| | - Vikas Khetan
- Formerly at Shri Bhagwan Mahavir Vitreoretinal Services, Sankara Nethralaya, Chennai, Tamil Nadu, India
- Professor, Department of Ophthalmology, Flaum Eye Institute, Rochester, NY, USA
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Chiranthan M, Meel R, Bakhshi S, Sharma S, Lomi N, Kashyap S, Bajaj MS. Baseline and Post-NACT Imaging in Retinoblastoma With Optic Nerve Involvement: Can MRI Predict Prognosis? J Pediatr Ophthalmol Strabismus 2024; 61:98-105. [PMID: 37615419 DOI: 10.3928/01913913-20230714-01] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 08/25/2023]
Abstract
PURPOSE To describe a staging system for optic nerve invasion using magnetic resonance imaging (MRI) and report any correlation with survival outcome. METHODS This was a ambispective study. Twenty-one patients with retinoblastoma who had optic nerve involvement on MRI were staged at baseline based on contrast enhancement and/or thickening and length of involvement. Response to neoadjuvant chemotherapy (NACT) was noted according to proposed response evaluation criteria and results were correlated with survival outcome. RESULTS Baseline MRI staging was able to predict event-free survival (EFS) (P = .0015) using the log-rank test for trends. Patients with optic nerve enhancement alone showed 100% survival prognosis. Optic nerve thickening cases with complete or partial response to NACT showed better EFS (P > .90) than those with stable disease according to response evaluation criteria. CONCLUSIONS The modified staging system for optic nerve invasion used in the current study significantly predicted EFS. The study also showed that response to NACT may be affected by baseline staging. The authors recommend that cases with optic nerve enhancement only, irrespective of the length of involvement (stage 0), may be treated with upfront enucleation. Cases with optic nerve thickening may be staged to evaluate the correlation with survival outcome in a larger cohort in future studies. [J Pediatr Ophthalmol Strabismus. 2024;61(2):98-105.].
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Chiranthan M, Meel R, Sharma S, Lomi N, Kashyap S, Bajaj MS. Can Enhancement Pattern in Normal-Sized Optic Nerves on Magnetic Resonance Imaging Better Predict Tumor Invasion in Retinoblastoma Eyes? Ocul Oncol Pathol 2023; 9:107-114. [PMID: 37900190 PMCID: PMC10601867 DOI: 10.1159/000531354] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/02/2022] [Accepted: 05/18/2023] [Indexed: 10/31/2023] Open
Abstract
Introduction Optic nerve (ON) enhancement alone without ON thickening on contrast-enhanced magnetic resonance imaging (CE-MRI) can be associated with post-laminar optic nerve invasion (PLONI) in eyes with group E retinoblastoma. A few case reports and retrospective studies in the literature show a poor correlation between ON enhancement on MRI and ON invasion on histopathological examination (HPE). There is no universal consensus on the management of such cases. It is desirable that the presence and extent of a true ON invasion be reliably picked up before planning upfront enucleation in order to avoid stage II disease. Methods In a prospective study conducted at a tertiary eye care center in North India, all retinoblastoma patients presenting with ON enhancement on imaging were evaluated. Demographic and imaging details, histopathological findings, and treatment details were recorded. The length and pattern of enhancement noted on MRI were correlated with histopathology. Follow-up was done till the end of the study period. Results Six group E retinoblastoma eyes were evaluated. 3 eyes (50%) showed solid enhancement, 2 eyes (33.33%) had tram track pattern and 1 eye (16.66%) showed punctate enhancement pattern on CE-MRI. On histopathology, 5 (83.33%) cases showed PLONI and all 6 (100%) had ON head infiltration. The cut end of the ON was free in all cases. On correlating MRI and HPE, all eyes with solid enhancement pattern showed PLONI, of which 2/3 (66.6%) had diffuse ON infiltration. Only 50% of eyes with tram track patterns showed PLONI. The case which showed a punctate enhancement pattern showed focal infiltration by tumor cells with vacuolated cytoplasm on HPE. At the last follow-up, all patients were alive and free of disease. Conclusion ON enhancement patterns may make it more predictive for PLONI on HPE. Solid enhancement pattern appears to correlate better with the extent of ON invasion on HPE, and longer lengths of solid ON enhancement may be considered for neoadjuvant chemotherapy rather than upfront enucleation.
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Affiliation(s)
- Madhu Chiranthan
- Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India
| | - Rachna Meel
- Department of Oculoplasty and Ocular Oncology, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India
| | - Sanjay Sharma
- Department of Ocular Radiology, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India
| | - Neiwete Lomi
- Department of Oculoplasty and Ocular Oncology, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India
| | - Seema Kashyap
- Department of Ocular Pathology, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India
| | - Mandeep S. Bajaj
- Department of Oculoplasty and Ocular Oncology, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India
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Orbital Tumors-Clinical, Radiologic and Histopathologic Correlation. Diagnostics (Basel) 2022; 12:diagnostics12102376. [PMID: 36292065 PMCID: PMC9600631 DOI: 10.3390/diagnostics12102376] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/17/2022] [Revised: 09/20/2022] [Accepted: 09/27/2022] [Indexed: 11/17/2022] Open
Abstract
Orbital masses include a broad spectrum of benign and malignant entities. Often these masses are asymptomatic or show a slow growth rate, so that emergence of clinical symptoms is prolonged. In this context, cross-sectional imaging plays an elementary role in the characterization of these lesions. Aside from the characterization of the underlying entity, an evaluation of the involved compartments is possible by sufficient imaging, which also facilitates optimal treatment and surgery planning. The purpose of this review is to explore different benign and malignant orbital tumors and their typical appearance in imaging together with histopathologic findings.
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Liu J, Xu X, Yan J, Guo J, Wang X, Xian J. Diffusion‐Weighted
MR
Imaging of the Optic Nerve Can Improve the Detection of Post‐Laminar Optic Nerve Invasion from Retinoblastoma. J Magn Reson Imaging 2022; 57:1587-1593. [PMID: 36106682 DOI: 10.1002/jmri.28429] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/21/2022] [Revised: 08/30/2022] [Accepted: 08/30/2022] [Indexed: 11/07/2022] Open
Abstract
BACKGROUND Post-laminar optic nerve invasion (PLONI) is a high-risk factor for the metastasis of retinoblastoma (RB). Unlike conventional MRI, diffusion-weighted imaging (DWI) reflects histopathological features, and may aid the assessment of PLONI. PURPOSE To determine the value of conventional MRI plus DWI in detecting PLONI in RB patients. STUDY TYPE Retrospective. POPULATION Eighty-three RB patients, including 28 with histopathologically proven PLONI and 55 without PLONI. FIELD STRENGTH/SEQUENCE 3.0 T, precontrast axial T1-weighted and T2-weighted imaging, DWI, and postcontrast axial, coronal, and oblique-sagittal T1-weighted imaging. ASSESSMENT PLONI was assessed using post-enucleation histology and preoperative MRI features (optic nerve signal intensity, enlargement, and enhancement on conventional MRI, and apparent diffusion coefficient [ADC] of the optic nerve on DWI) evaluated by three observers. STATISTICAL TESTS MRI features suggesting the presence of PLONI were identified using univariable and multivariable analyses. Receiver operating characteristic (ROC) curve and area under the curve (AUC) were used to analyze diagnostic performance. RESULTS Optic nerve enhancement and low ADC of the optic nerve were significant indicators of PLONI. ROC curve analysis showed that the AUC of the combination of these two features for detecting PLONI was 0.87 (95% confidence interval [CI]: 0.78-0.93). The diagnostic performance of this model was significantly superior to that of optic nerve enhancement alone (0.76, 95% CI: 0.65-0.85) and marginally superior to that of the ADC of the affected optic nerve (0.78, 95% CI: 0.68-0.87, P = 0.051). DATA CONCLUSION Conventional MRI combined with DWI can improve the detection of PLONI in RB patients over conventional MRI alone. EVIDENCE LEVEL 3 Technical Efficacy: Stage 2.
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Affiliation(s)
- Jing Liu
- Department of Radiology Beijing Tongren Hospital, Capital Medical University Beijing China
- Dongfang Hospital, Beijing University of Chinese Medicine Beijing China
| | - Xiaolin Xu
- Institute of Ophthalmology, Beijing Tongren Eye Center Beijing Tongren Hospital, Capital Medical University Beijing China
| | - Jing Yan
- Dongfang Hospital, Beijing University of Chinese Medicine Beijing China
| | - Jian Guo
- Department of Radiology Beijing Tongren Hospital, Capital Medical University Beijing China
| | - XinYan Wang
- Department of Radiology Beijing Tongren Hospital, Capital Medical University Beijing China
| | - Junfang Xian
- Department of Radiology Beijing Tongren Hospital, Capital Medical University Beijing China
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Li Z, Guo J, Xu X, Wei W, Xian J. MRI-based radiomics model can improve the predictive performance of postlaminar optic nerve invasion in retinoblastoma. Br J Radiol 2022; 95:20211027. [PMID: 34826253 PMCID: PMC8822570 DOI: 10.1259/bjr.20211027] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/03/2023] Open
Abstract
OBJECTIVES To develop an MRI-based radiomics model to predict postlaminar optic nerve invasion (PLONI) in retinoblastoma (RB) and compare its predictive performance with subjective radiologists' assessment. METHODS We retrospectively enrolled 124 patients with pathologically proven RB (90 in training set and 34 in validation set) who had MRI scans before surgery. A radiomics model for predicting PLONI was developed by extracting quantitative imaging features from axial T2W images and contrast-enhanced T1W images in the training set. The Kruskal-Wallis test, least absolute shrinkage and selection operator regression, and recursive feature elimination were used for feature selection, where upon a radiomics model was built with a logistic regression (LR) classifier. The area under the curve (AUC) of the receiver operating characteristic (ROC) curve and the accuracy were assessed to evaluate the predictive performance in the training and validation set. The performance of the radiomics model was compared to radiologists' assessment by DeLong test. RESULTS The AUC of the radiomics model for the prediction of PLONI was 0.928 in the training set and 0.841 in the validation set. Radiomics model produced better sensitivity than radiologists' assessment (81.1% vs 43.2% in training set, 82.4vs 52.9% in validation set). In all 124 patients, the AUC of the radiomics model was 0.897, while that of radiologists' assessment was 0.674 (p < 0.001, DeLong test). CONCLUSION MRI-based radiomics model to predict PLONI in RB patients was shown to be superior to visual assessment with improved sensitivity and AUC, and may serve as a potential tool to guide personalized treatment.
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Affiliation(s)
- Zhenzhen Li
- Department of Radiology, Beijing Tongren Hospital, Capital Medical University, No. 1 of Dongjiaominxiang, Dongcheng District, Beijing, China
- Clinical Center for Eye Tumors, Capital Medical University, Beijing, China
| | - Jian Guo
- Department of Radiology, Beijing Tongren Hospital, Capital Medical University, No. 1 of Dongjiaominxiang, Dongcheng District, Beijing, China
- Clinical Center for Eye Tumors, Capital Medical University, Beijing, China
| | - Xiaolin Xu
- Clinical Center for Eye Tumors, Capital Medical University, Beijing, China
- Institute of Ophthalmology, Beijing Tongren Eye Center, Beijing Key Laboratory of Intraocular Tumor Diagnosis and Treatment, Beijing Ophthalmology & Visual Sciences Key Lab, Beijing Tongren Hospital, Capital Medical University, Beijing, China
| | - Wenbin Wei
- Clinical Center for Eye Tumors, Capital Medical University, Beijing, China
- Institute of Ophthalmology, Beijing Tongren Eye Center, Beijing Key Laboratory of Intraocular Tumor Diagnosis and Treatment, Beijing Ophthalmology & Visual Sciences Key Lab, Beijing Tongren Hospital, Capital Medical University, Beijing, China
| | - Junfang Xian
- Department of Radiology, Beijing Tongren Hospital, Capital Medical University, No. 1 of Dongjiaominxiang, Dongcheng District, Beijing, China
- Clinical Center for Eye Tumors, Capital Medical University, Beijing, China
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7
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Deike-Hofmann K, von Lampe P, Eerikaeinen M, Ting S, Schlüter S, Schlemmer HP, Bechrakis NE, Forsting M, Radbruch A. Anterior chamber enhancement predicts optic nerve infiltration in retinoblastoma. Eur Radiol 2022; 32:7354-7364. [PMID: 35524782 PMCID: PMC9668776 DOI: 10.1007/s00330-022-08778-4] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/02/2021] [Revised: 03/15/2022] [Accepted: 03/26/2022] [Indexed: 01/03/2023]
Abstract
OBJECTIVES As described recently, intravenously injected gadolinium-based contrast agent (GBCA) penetrates into the anterior eye chamber (AC) and is drained from the retina to the distal optic nerve (ON) along perivascular spaces, which serves retinal homeostasis and was termed the orbital glymphatic system (GS). Independently, AC enhancement predicted ON infiltration, a major risk factor for advanced retinoblastoma (RB), in a small RB patient cohort. We aimed to review the supposed imaging biomarker for ON infiltration in a large RB cohort and with respect to the recently described orbital GS. METHODS This IRB-approved retrospective single-center study encompassed 539 orbital MRIs performed with an orbital coil and with the children under general anesthesia. Differences of signal intensity ratios (∆SIRs) of the AC to the lens were determined between non-contrast and GBCA-enhanced T1-weighted images and were correlated with histopathologic presence of ON infiltration. RESULTS ∆SIR of the RB eye was an independent, significant predictor for ON invasion in multivariate analysis with adjustment for tumor size (p < 0.05) and increased with infiltration level. CONCLUSIONS GBCA enhancement of the AC predicts ON infiltration. This might be caused by impairment of the orbital glymphatic system, which is supposed to clear toxic metabolites from the retina to the postlaminar ON. In RB with ON infiltration, this efflux path is likely to be inhibited, which is supposed to result in disturbed retinal homeostasis, release of vascular endothelial growth factor, and iris neovascularization, which increases penetration of GBCA into the AC. KEY POINTS • Infiltration of the optic nerve can be predicted by anterior chamber enhancement after intravenous MRI contrast agent administration. • Increased anterior chamber enhancement in retinoblastoma with optic nerve infiltration might result from dysfunction of the orbital glymphatic system with disturbance of retinal homeostasis and consecutive iris neovascularization.
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Affiliation(s)
- Katerina Deike-Hofmann
- Department of Radiology, German Cancer Research Center, DKFZ, Heidelberg, Germany ,Department of Neuroradiology, University Hospital Bonn, Bonn, Germany ,Clinical Neuroimaging Group, German Center for Neurodegenerative Diseases, DZNE, Bonn, Germany
| | - Paula von Lampe
- Department of Radiology, University Hospital Essen, Essen, Germany
| | | | - Saskia Ting
- Department of Pathology, University Hospital Essen, Essen, Germany
| | - Sabrina Schlüter
- Department of Ophthalmology, University Hospital Essen, Essen, Germany
| | | | | | - Michael Forsting
- Department of Radiology, University Hospital Essen, Essen, Germany
| | - Alexander Radbruch
- Department of Neuroradiology, University Hospital Bonn, Bonn, Germany ,Clinical Neuroimaging Group, German Center for Neurodegenerative Diseases, DZNE, Bonn, Germany
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8
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Silvera VM, Guerin JB, Brinjikji W, Dalvin LA. Retinoblastoma: What the Neuroradiologist Needs to Know. AJNR Am J Neuroradiol 2021; 42:618-626. [PMID: 33509920 DOI: 10.3174/ajnr.a6949] [Citation(s) in RCA: 20] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/09/2020] [Accepted: 10/14/2020] [Indexed: 12/12/2022]
Abstract
Retinoblastoma is the most common primary intraocular tumor of childhood. Accurate diagnosis at an early stage is important to maximize patient survival, globe salvage, and visual acuity. Management of retinoblastoma is individualized based on the presenting clinical and imaging features of the tumor, and a multidisciplinary team is required to optimize patient outcomes. The neuroradiologist is a key member of the retinoblastoma care team and should be familiar with characteristic diagnostic and prognostic imaging features of this disease. Furthermore, with the adoption of intra-arterial chemotherapy as a standard of care option for globe salvage therapy in many centers, the interventional neuroradiologist may play an active role in retinoblastoma treatment. In this review, we discuss the clinical presentation of retinoblastoma, ophthalmic imaging modalities, neuroradiology imaging features, and current treatment options.
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Affiliation(s)
- V M Silvera
- From the Departments of Neuroradiology (V.M.S., J.B.G., W.B.)
| | - J B Guerin
- From the Departments of Neuroradiology (V.M.S., J.B.G., W.B.)
| | - W Brinjikji
- From the Departments of Neuroradiology (V.M.S., J.B.G., W.B.).,Neurosurgery (W.B.)
| | - L A Dalvin
- Ophthalmology (L.A.D.), Mayo Clinic, Rochester, Minnesota
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Cho SJ, Kim JH, Baik SH, Sunwoo L, Bae YJ, Choi BS. Diagnostic performance of MRI of post-laminar optic nerve invasion detection in retinoblastoma: A systematic review and meta-analysis. Neuroradiology 2020; 63:499-509. [PMID: 32865636 DOI: 10.1007/s00234-020-02538-1] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/23/2020] [Accepted: 08/20/2020] [Indexed: 10/23/2022]
Abstract
PURPOSE Preoperative MRI detection of post-laminar optic nerve invasion (PLONI) offers guidance in assessing the probability of total tumor resection, an estimation of the extent of surgery, and screening of candidates for eye-preserving therapies or neoadjuvant chemotherapies in the patients with retinoblastoma (RB). The purpose of this systematic review and meta-analysis was to evaluate the diagnostic performance of MRI for detecting PLONI in patients with RB and to demonstrate the factors that may influence the diagnostic performance. METHODS Ovid-MEDLINE and EMBASE databases were searched up to January 11, 2020, for studies identifying the diagnostic performance of MRI for detecting PLONI in patients with RB. The pooled sensitivity and specificity of all studies were calculated followed by meta-regression analysis. RESULTS Twelve (1240 patients, 1255 enucleated globes) studies were included. The pooled sensitivity was 61%, and the pooled specificity was 88%. Higgins I2 statistic demonstrated moderate heterogeneity in the sensitivity (I2 = 72.23%) and specificity (I2 = 78.11%). Spearman correlation coefficient indicated the presence of a threshold effect. In the meta-regression, higher magnetic field strength (3 T than 1.5 T), performing fat suppression, and thinner slice thickness (< 3 mm) were factors causing heterogeneity and enhancing diagnostic power across the included studies. CONCLUSIONS MR imaging was demonstrated to have acceptable diagnostic performance in detecting PLONI in patients with RB. The variation in the magnetic field strength and protocols was the main factor behind the heterogeneity across the included studies. Therefore, there is room for developing and optimizing the MR protocols for patients with RB.
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Affiliation(s)
- Se Jin Cho
- Department of Radiology, Seoul National University Bundang Hospital, 82, Gumi-ro 173beon-gil, Bundang-gu, Seongnam, Gyeonggi, 13620, Republic of Korea
| | - Jae Hyoung Kim
- Department of Radiology, Seoul National University Bundang Hospital, 82, Gumi-ro 173beon-gil, Bundang-gu, Seongnam, Gyeonggi, 13620, Republic of Korea.
| | - Sung Hyun Baik
- Department of Radiology, Seoul National University Bundang Hospital, 82, Gumi-ro 173beon-gil, Bundang-gu, Seongnam, Gyeonggi, 13620, Republic of Korea
| | - Leonard Sunwoo
- Department of Radiology, Seoul National University Bundang Hospital, 82, Gumi-ro 173beon-gil, Bundang-gu, Seongnam, Gyeonggi, 13620, Republic of Korea
| | - Yun Jung Bae
- Department of Radiology, Seoul National University Bundang Hospital, 82, Gumi-ro 173beon-gil, Bundang-gu, Seongnam, Gyeonggi, 13620, Republic of Korea
| | - Byung Se Choi
- Department of Radiology, Seoul National University Bundang Hospital, 82, Gumi-ro 173beon-gil, Bundang-gu, Seongnam, Gyeonggi, 13620, Republic of Korea
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10
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Kim U, Rathi G, Chowdhary G, Srinavasan KG, Shanthi R, Krishna RSP. Accuracy of preoperative imaging in predicting optic nerve invasion in retinoblastoma: A retrospective study. Indian J Ophthalmol 2020; 67:2019-2022. [PMID: 31755442 PMCID: PMC6896533 DOI: 10.4103/ijo.ijo_1611_18] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022] Open
Abstract
Purpose: Optic nerve invasion is an important cause of mortality in retinoblastoma. We aimed correlate preoperative imaging and surgical histopathology findings in enucleated eyes with retinoblastoma to determine the efficacy of preoperative imaging in predicting optic nerve invasion in retinoblastoma. Methods: A retrospective review of records of all patients undergoing primary enucleation for retinoblastoma at a tertiary eyecare system between March 2013 and December 2017 with all patients having undergone preoperative imaging, either CT scan or MRI. Data was analyzed statistically to determine the correlation between preoperative CT scan/MRI and histopathology. Results: Totally, 97 eyes of 97 patients were included in the study who underwent primary enucleation for unilateral retinoblastoma. The average age at presentation was 27.8 months with the chief complaint being leukocoria in all the cases. 14 patients (14.43%) had evidence of optic nerve involvement in preoperative imaging. 30 patients had optic nerve invasion on histopathology (laminar and retrolaminar). Spearman's rank correlation test revealed a significant correlation between MRI findings and HP and an insignficant correlation between CT findings and HP. The CT scan had a sensitivity of 20%, specificity of 88.89%, 50% positive predictive value and 66.67% negative predictive value. MRI had a sensitivity of 40%, specificity of 93.55%, positive predictive value of 66.67% and a negative predictive value of 82.86%. Conclusion: MRI showed significant moderate correlation with surgical histopathology for predicting optic nerve invasion in retinoblastoma whereas CT shows insignificant correlation with HPE. Therefore, we recommend MRI for predicting optic nerve invasion in cases of retinoblastoma.
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Affiliation(s)
- Usha Kim
- Department of Orbit and Oculoplasty, Aravind Eye Hospital, Madurai, Tamil Nadu, India
| | - Gunjan Rathi
- Department of Orbit and Oculoplasty, Aravind Eye Hospital, Madurai, Tamil Nadu, India
| | - Gunja Chowdhary
- Department of Orbit and Oculoplasty, Aravind Eye Hospital, Madurai, Tamil Nadu, India
| | - K G Srinavasan
- Department of Orbit and Oculoplasty, Aravind Eye Hospital, Madurai, Tamil Nadu, India
| | - R Shanthi
- Department of Orbit and Oculoplasty, Aravind Eye Hospital, Madurai, Tamil Nadu, India
| | - R S Prabhu Krishna
- Department of Orbit and Oculoplasty, Aravind Eye Hospital, Madurai, Tamil Nadu, India
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11
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Li Z, Guo J, Xu X, Wang Y, Mukherji SK, Xian J. Diagnosis of Postlaminar Optic Nerve Invasion in Retinoblastoma With MRI Features. J Magn Reson Imaging 2019; 51:1045-1052. [PMID: 31617290 DOI: 10.1002/jmri.26961] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/15/2019] [Revised: 09/20/2019] [Accepted: 09/20/2019] [Indexed: 11/07/2022] Open
Abstract
BACKGROUND Retinoblastomas (RBs) with postlaminar optic nerve invasion (PLONI) increases the risk of local recurrence or systemic metastasis. Most MRI studies on PLONI focused on optic nerve enhancement and tumor size, with reported relatively high specificity (84-98%) but low sensitivity (37-78%), which shows room for improvement, especially with regard to sensitivity. PURPOSE To evaluate the diagnostic performance for detecting PLONI with RB using MRI features. STUDY TYPE Retrospective. POPULATION Fifty patients with histopathologic PLONI and 70 patients without PLONI of RB. FIELD STRENGTH/SEQUENCE 1.5T and 3.0T, precontrast axial T1 -weighted and T2 -weighted, postcontrast axial, coronal and oblique-sagittal T1 -weighted. ASSESSMENT The eyes were histopathologically analyzed and the preoperative MRI features of the eyes were independently evaluated by three observers. STATISTICAL TESTS MRI features suggesting the presence of PLONI were identified by univariate and multivariable analysis. Receiver operating characteristic curve (ROC) and the area under the curve (AUC) were used to analyze diagnostic performance. RESULTS Significant independent diagnostic factors for PLONI include: 1: Bilateral tumor (odds ratio [OR], 15.32; 95% confidence interval [CI]: 1.63-143.51); 2: Tumor with total coverage of the optic disk (OR, 6.43; 95% CI: 1.04-39.79); and 3: Optic nerve enhancement (OR, 8.43; 95% CI: 3.50-20.31). On the other hand, isointense signal of tumor on T2 WI (OR, 0.30; 95% CI: 0.12-0.75) was an independent diagnostic factor in excluding PLONI. ROC analysis showed AUC of 0.84 (95% CI: 0.77-0.91, P < 0.0001) for PLONI. Based on the cutoff of maximum Youden index, the sensitivity, specificity, and accuracy were 82%, 73%, and 77%, respectively. DATA CONCLUSION MRI features of RB showed a strong association with PLONI. The model of MRI features demonstrated promising diagnostic performance in detecting PLONI. LEVEL OF EVIDENCE 4 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2020;51:1045-1052.
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Affiliation(s)
- Zhenzhen Li
- Department of Radiology, Beijing Tongren Hospital, Capital Medical University, Beijing, China.,Clinical Center for Eye Tumors, Capital Medical University, Beijing, China
| | - Jian Guo
- Department of Radiology, Beijing Tongren Hospital, Capital Medical University, Beijing, China.,Clinical Center for Eye Tumors, Capital Medical University, Beijing, China
| | - Xiaolin Xu
- Clinical Center for Eye Tumors, Capital Medical University, Beijing, China.,Institute of Ophthalmology, Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China
| | - Yongzhe Wang
- Department of Radiology, Beijing Tongren Hospital, Capital Medical University, Beijing, China.,Clinical Center for Eye Tumors, Capital Medical University, Beijing, China
| | - Suresh Kumar Mukherji
- Department of Radiology, Michigan State University, Michigan State University Health Team, East Lansing, Michigan, USA
| | - Junfang Xian
- Department of Radiology, Beijing Tongren Hospital, Capital Medical University, Beijing, China.,Clinical Center for Eye Tumors, Capital Medical University, Beijing, China
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12
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Bonanomi MTBC, Saito OC, de Lima PP, Bonanomi RC, Chammas MC. Blood Flow in Monocular Retinoblastoma Assessed by Color Doppler and Correlations With High-Risk Pathologic Features. Invest Ophthalmol Vis Sci 2018; 59:5441-5446. [PMID: 30452597 DOI: 10.1167/iovs.18-24777] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/24/2022] Open
Abstract
Purpose To use color Doppler to analyze blood flow in the retrobulbar central retinal artery (CRA) and central retinal vein (CRV) in monocular retinoblastoma. Methods This prospective study included patients with group D and E retinoblastomas managed with only enucleation. Peak blood velocities were assessed in the CRA and CRV of tumor-containing eyes (CRAv and CRVv, respectively). The resistivity index in the CRA (RIa) and pulse index in the CRV (PIv) were calculated and related to optic nerve invasion (ONi), choroid invasion (mCHi), and tumor volume. RIa and PIv were also calculated for healthy eyes. Results In total, 25 patients with a mean age of 30.8-months old were included. The means (SD) for CRAv, CRVv, RIa, and PIv were 26.94 (12.32) cm/s, 16.2 (9.56) cm/s, 0.88 (0.12) and 0.79 (0.29), respectively. Tumor volume was significantly correlated with CRAv (P = 0.025) and RIa (P = 0.032). ONi was present in 19 eyes and correlated with a smaller PIv (P < 0.001). A PIv less than 0.935 had a sensitivity of 89.5% and specificity of 83.3% for predicting ONi. mCHi was not correlated with flow values. Healthy eyes had a significantly lower RIa (P < 0.001) and lower PIv than eyes with (P = 0.009) and without (P < 0.001) ONi. Conclusions In advanced-stage monocular retinoblastoma, tumor volume was directly correlated with CRAv and RIa, and lower PIv was correlated with optic nerve invasion when a predictive cut-off value of less than 0.935 was applied. Comparisons with healthy eyes showed that tumor-containing eyes were associated with higher RIa and PIv values.
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Affiliation(s)
| | - Osmar C Saito
- Department of Radiology and Ultrasound, University of São Paulo, São Paulo, São Paulo, Brazil
| | | | | | - Maria Cristina Chammas
- Department of Radiology and Ultrasound, University of São Paulo, São Paulo, São Paulo, Brazil
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Hiasat JG, Saleh A, Al-Hussaini M, Al Nawaiseh I, Mehyar M, Qandeel M, Mohammad M, Deebajah R, Sultan I, Jaradat I, Mansour A, Yousef YA. The predictive value of magnetic resonance imaging of retinoblastoma for the likelihood of high-risk pathologic features. Eur J Ophthalmol 2018; 29:262-268. [PMID: 29890860 DOI: 10.1177/1120672118781200] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
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.
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Affiliation(s)
- Jamila G Hiasat
- 1 Department of Surgery/Ophthalmology, King Hussein Cancer Center, Amman, Jordan
| | - Alaa Saleh
- 2 Department of Radiology, King Hussein Cancer Center, Amman, Jordan
| | - Maysa Al-Hussaini
- 3 Department of Pathology and Laboratory Medicine, King Hussein Cancer Center, Amman, Jordan
| | - Ibrahim Al Nawaiseh
- 1 Department of Surgery/Ophthalmology, King Hussein Cancer Center, Amman, Jordan
| | - Mustafa Mehyar
- 1 Department of Surgery/Ophthalmology, King Hussein Cancer Center, Amman, Jordan
| | - Monther Qandeel
- 2 Department of Radiology, King Hussein Cancer Center, Amman, Jordan
| | - Mona Mohammad
- 1 Department of Surgery/Ophthalmology, King Hussein Cancer Center, Amman, Jordan
| | - Rasha Deebajah
- 4 Department of Pediatric Oncology, King Hussein Cancer Center, Amman, Jordan
| | - Iyad Sultan
- 4 Department of Pediatric Oncology, King Hussein Cancer Center, Amman, Jordan
| | - Imad Jaradat
- 5 Department of Radiation Oncology, King Hussein Cancer Center, Amman, Jordan
| | - Asem Mansour
- 2 Department of Radiology, King Hussein Cancer Center, Amman, Jordan
| | - Yacoub A Yousef
- 1 Department of Surgery/Ophthalmology, King Hussein Cancer Center, Amman, Jordan
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The value of MRI in evaluating the efficacy and complications with the treatment of intra-arterial chemotherapy for retinoblastoma. Oncotarget 2018; 8:38413-38425. [PMID: 28415600 PMCID: PMC5503542 DOI: 10.18632/oncotarget.16423] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/23/2016] [Accepted: 03/14/2017] [Indexed: 01/10/2023] Open
Abstract
Retinoblastoma is the most common intraocular malignant tumor of childhood. Intra-arterial chemotherapy (IAC) is a recently popularized technique used for the treatment of retinoblastoma, to decrease mortality, increase preservation of the eye, and prevent blindness. Along with the extensive use of IAC, it is important to apply noninvasive examination methods to assess the activity of the tumor and the risk factors for disease dissemination without histopathological confirmation. There are few studies that have assessed the value of magnetic resonance imaging (MRI) in evaluating the efficacy and complications of IAC for retinoblastoma. We retrospectively analyzed the MRI features of 60 patients with unilateral retinoblastoma given the primary treatment of IAC from January 2014 to February 2016 in our hospital. Our study showed that MRI could well assess the decreased activity of the tumor after IAC, presenting with diminished tumor size, increased apparent diffusion coefficient (ADC) values (from 0.94 ± 0.24 × 10-3 mm2/s to 2.24 ± 0.40 × 10-3 mm2/s), and a reduced degree of enhancement of the tumor. Our study also showed that MRI can monitor the risk factors of abnormal enhancement of the postlaminar optic nerve, to avoid unnecessary enucleation. Meanwhile, the results showed that the main late complications after IAC included affected eyeball volume reduction, subretinal hemorrhage, vitreous hemorrhage, vitreous opacity, cataractous len, and choroidal vascular ischemia.
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Correa-Acosta A, González-Alviar ME, Gaviria-Bravo ML. Retinoblastoma and optic nerve enhancement in a brain magnetic resonance scan: is it always a metastasis? ACTA ACUST UNITED AC 2017; 93:251-254. [PMID: 29277434 DOI: 10.1016/j.oftal.2017.10.010] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/24/2017] [Revised: 10/27/2017] [Accepted: 10/28/2017] [Indexed: 12/31/2022]
Abstract
CASE REPORT The case is presented on a girl with a unilateral retinoblastoma that required treatment with intra-arterial chemotherapy. In the nuclear magnetic resonance imaging of the brain performed 1 month after intra-arterial chemotherapy treatment, post-laminar optic nerve (ON) enhancement was observed, leading to the suspicion of an ON tumour infiltration. Additional examinations were requested by which a probable optic neuropathy was diagnosed. DISCUSSION The ON enhancement in magnetic resonance imaging of the brain in retinoblastoma generally corresponds to tumour invasion of the ON. However, other diagnostic alternatives associated with the use of new treatments, such as intra-arterial chemotherapy, should be considered.
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Affiliation(s)
- A Correa-Acosta
- Servicio de Oftalmología, Hospital Universitario San Vicente Fundación, Universidad de Antioquia, Medellín, Colombia.
| | - M E González-Alviar
- Servicio de Oftalmología, Hospital Universitario San Vicente Fundación, Universidad de Antioquia, Medellín, Colombia
| | - M L Gaviria-Bravo
- Servicio de Oftalmología, Hospital Universitario San Vicente Fundación, Universidad de Antioquia, Medellín, Colombia
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Cui Y, Luo R, Wang R, Liu H, Zhang C, Zhang Z, Wang D. Correlation between conventional MR imaging combined with diffusion-weighted imaging and histopathologic findings in eyes primarily enucleated for advanced retinoblastoma: a retrospective study. Eur Radiol 2017; 28:620-629. [PMID: 28786011 DOI: 10.1007/s00330-017-4993-7] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/11/2017] [Revised: 07/10/2017] [Accepted: 07/13/2017] [Indexed: 11/29/2022]
Abstract
OBJECTIVES To determine the diagnostic accuracy of conventional MRI in detecting tumour invasion of advanced intraocular retinoblastoma and to correlate ADC values with high-risk prognostic parameters. METHOD The sensitivities, specificities, positive predictive values (PPV), negative predictive values (NPV) and accuracies of MRI in detecting tumour-extent parameters of 63 retinoblastomas were determined. Furthermore, ADC values were correlated with high-risk prognostic parameters. RESULTS MRI detected postlaminar optic nerve with a sensitivity of 73.3% (95% CI 44.9-92.2%) and a specificity of 89.6% (77.3-96.5%), while the specificity for choroidal invasion was only 31.8% (13.9-54.9%). Likewise, MRI failed to predicted early optic nerve invasion in terms of low sensitivity and PPV. In contrast, scleral and ciliary body invasion could be correctly excluded with high NPV. ADC values were significantly lower in patients with undifferentiated tumours, large tumour size, as with optic nerve and scleral invasion (all p < 0.05). However, no correlation was found between ADC values and the degree of choroidal or ciliary body infiltration. Additionally, ADC values were negatively correlated with Ki-67 index (r = -0.62, P = 0.002). CONCLUSIONS Conventional MRI has some limitations in reliably predicting microscopic infiltration, with the diagnostic efficiency showing room for improvement, whereas ADC values correlated well with certain high-risk prognostic parameters for retinoblastoma. KEY POINTS • Conventional MRI failed to predicted microscopic infiltration of the retinoblastoma. • Scleral and ciliary body invasion could be excluded with high NPV. • ADC values correlated well with some high-risk pathological prognostic parameters.
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Affiliation(s)
- Yanfen Cui
- Department of Radiology, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200092, China.,Department of Radiology, Shanxi Province Cancer Hospital, Shanxi Medical University, Taiyuan, 030013, China
| | - Ran Luo
- Department of Radiology, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200092, China
| | - Ruifen Wang
- Department of Pathology, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200092, China
| | - Huanhuan Liu
- Department of Radiology, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200092, China
| | - Caiyuan Zhang
- Department of Radiology, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200092, China
| | - Zhongyang Zhang
- Department of Radiology, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200092, China
| | - Dengbin Wang
- Department of Radiology, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200092, China.
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Bonanomi MTBC, Saito OC, de Lima PP, Bonanomi RC, Chammas MC. Blood flow velocity in monocular retinoblastoma assessed by color Doppler. Clinics (Sao Paulo) 2015; 70:797-803. [PMID: 26735219 PMCID: PMC4676317 DOI: 10.6061/clinics/2015(12)06] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/14/2015] [Accepted: 09/22/2015] [Indexed: 11/18/2022] Open
Abstract
OBJECTIVE To analyze the flow of retrobulbar vessels in retinoblastoma by color Doppler imaging. METHODS A prospective study of monocular retinoblastoma treated by enucleation between 2010 and 2014. The examination comprised fundoscopy, magnetic resonance imaging, ultrasonography and color Doppler imaging. The peak blood velocities in the central retinal artery and central retinal vein of tumor-containing eyes (tuCRAv and tuCRVv, respectively) were assessed. The velocities were compared with those for normal eyes (nlCRAv and nlCRVv) and correlated with clinical and pathological findings. Tumor dimensions in the pathological sections were compared with those in magnetic resonance imaging and ultrasonography and were correlated with tuCRAv and tuCRVv. In tumor-containing eyes, the resistivity index in the central retinal artery and the pulse index in the central retinal vein were studied in relation to all variables. RESULTS Eighteen patients were included. Comparisons between tuCRAv and nlCRAv and between tuCRVv and nlCRVv revealed higher velocities in tumor-containing eyes (p < 0.001 for both), with a greater effect in the central retinal artery than in the central retinal vein (p = 0.024). Magnetic resonance imaging and ultrasonography measurements were as reliable as pathology assessments (p = 0.675 and p = 0.375, respectively). A positive relationship was found between tuCRAv and the tumor volume (p = 0.027). The pulse index in the central retinal vein was lower in male patients (p = 0.017) and in eyes with optic nerve invasion (p = 0.0088). CONCLUSIONS TuCRAv and tuCRVv are higher in tumor-containing eyes than in normal eyes. Magnetic resonance imaging and ultrasonography measurements are reliable. The tumor volume is correlated with a higher tuCRAv and a reduced pulse in the central retinal vein is correlated with male sex and optic nerve invasion.
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Affiliation(s)
- Maria Teresa B C Bonanomi
- Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, Departamento de Oftalmologia, São Paulo/SP, Brazil
- Corresponding author: E-mail:
| | - Osmar C Saito
- Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, Departamento de Radiologia e Ultrassom, São Paulo/SP, Brazil
| | - Patricia Picciarelli de Lima
- Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, Departamento de Patologia, São Paulo/SP, Brazil
| | | | - Maria Cristina Chammas
- Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, Departamento de Radiologia e Ultrassom, São Paulo/SP, Brazil
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High-resolution MRI using orbit surface coils for the evaluation of metastatic risk factors in 143 children with retinoblastoma. Neuroradiology 2015; 57:805-14. [DOI: 10.1007/s00234-015-1544-2] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/20/2015] [Accepted: 05/18/2015] [Indexed: 10/23/2022]
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Sirin S, Schlamann M, Metz KA, Bornfeld N, Schweiger B, Holdt M, Temming P, Schuendeln MM, Goericke SL. High-resolution MRI using orbit surface coils for the evaluation of metastatic risk factors in 143 children with retinoblastoma. Neuroradiology 2015; 57:815-24. [DOI: 10.1007/s00234-015-1538-0] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/20/2015] [Accepted: 04/20/2015] [Indexed: 11/24/2022]
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Chawla B, Lokdarshi G, Pathy S. Recent advances in management of retinoblastoma: A review. World J Ophthalmol 2015; 5:31-35. [DOI: 10.5318/wjo.v5.i1.31] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/11/2014] [Revised: 10/27/2014] [Accepted: 11/03/2014] [Indexed: 02/06/2023] Open
Abstract
The management of retinoblastoma has evolved significantly over recent years. Current treatment options aim to preserve the globe as well as vision with minimum morbidity. High resolution imaging has improved tumor detection and is useful for prognosticating cases and monitoring response to treatment. Targeted chemotherapy such as intra-arterial and intra-vitreal chemotherapy has shown promising results and these routes are being increasingly employed world-wide for globe preservation. The advent of new radiotherapy techniques has led to improved radiation delivery to the target and more conformal treatment plans with better normal tissue sparing. This review aims to highlight newer advancements in the field of diagnosis and management of retinoblastoma that have been introduced in recent times, with a special emphasis on globe-preserving therapy.
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Abstract
Retinoblastoma is the most common neoplasm of the eye in childhood, and represents 3% of all childhood malignancies. Retinoblastoma is a cancer of the very young; two-thirds are diagnosed before 2 years of age and 95% before 5 years. Retinoblastoma presents in 2 distinct clinical forms: (1) a bilateral or multifocal, heritable form (25% of all cases), characterized by the presence of germline mutations of the RB1 gene; and (2) a unilateral or unifocal form (75% of all cases), 90% of which are nonhereditary. The treatment of retinoblastoma is multidisciplinary and is designed primarily to save life and preserve vision.
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Affiliation(s)
- Carlos Rodriguez-Galindo
- Department of Pediatric Oncology, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Harvard Medical School, 450 Brookline Avenue, D3-133, Boston, MA 02215, USA.
| | - Darren B Orbach
- Department of Radiology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02215, USA
| | - Deborah VanderVeen
- Department of Ophthalmology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02215, USA
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Brisse HJ, de Graaf P, Galluzzi P, Cosker K, Maeder P, Göricke S, Rodjan F, de Jong MC, Savignoni A, Aerts I, Desjardins L, Moll AC, Hadjistilianou T, Toti P, van der Valk P, Castelijns JA, Sastre-Garau X. Assessment of early-stage optic nerve invasion in retinoblastoma using high-resolution 1.5 Tesla MRI with surface coils: a multicentre, prospective accuracy study with histopathological correlation. Eur Radiol 2014; 25:1443-52. [PMID: 25433413 DOI: 10.1007/s00330-014-3514-1] [Citation(s) in RCA: 23] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/29/2014] [Revised: 09/20/2014] [Accepted: 11/17/2014] [Indexed: 11/25/2022]
Abstract
OBJECTIVES To assess the accuracy of high-resolution (HR) magnetic resonance imaging (MRI) in diagnosing early-stage optic nerve (ON) invasion in a retinoblastoma cohort. METHODS This IRB-approved, prospective multicenter study included 95 patients (55 boys, 40 girls; mean age, 29 months). 1.5-T MRI was performed using surface coils before enucleation, including spin-echo unenhanced and contrast-enhanced (CE) T1-weighted sequences (slice thickness, 2 mm; pixel size <0.3 × 0.3 mm(2)). Images were read by five neuroradiologists blinded to histopathologic findings. ROC curves were constructed with AUC assessment using a bootstrap method. RESULTS Histopathology identified 41 eyes without ON invasion and 25 with prelaminar, 18 with intralaminar and 12 with postlaminar invasion. All but one were postoperatively classified as stage I by the International Retinoblastoma Staging System. The accuracy of CE-T1 sequences in identifying ON invasion was limited (AUC = 0.64; 95 % CI, 0.55 - 0.72) and not confirmed for postlaminar invasion diagnosis (AUC = 0.64; 95 % CI, 0.47 - 0.82); high specificities (range, 0.64 - 1) and negative predictive values (range, 0.81 - 0.97) were confirmed. CONCLUSION HR-MRI with surface coils is recommended to appropriately select retinoblastoma patients eligible for primary enucleation without the risk of IRSS stage II but cannot substitute for pathology in differentiating the first degrees of ON invasion. KEY POINTS • HR-MRI excludes advanced optic nerve invasion with high negative predictive value. • HR-MRI accurately selects patients eligible for primary enucleation. • Diagnosis of early stages of optic nerve invasion still relies on pathology. • Several physiological MR patterns may mimic optic nerve invasion.
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de Jong MC, de Graaf P, Noij DP, Göricke S, Maeder P, Galluzzi P, Brisse HJ, Moll AC, Castelijns JA. Diagnostic Performance of Magnetic Resonance Imaging and Computed Tomography for Advanced Retinoblastoma. Ophthalmology 2014; 121:1109-18. [DOI: 10.1016/j.ophtha.2013.11.021] [Citation(s) in RCA: 50] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/17/2013] [Revised: 11/05/2013] [Accepted: 11/06/2013] [Indexed: 11/26/2022] Open
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