A proposal for an international retinoblastoma staging system

Chantada, Guillermo; Doz, François; Antoneli, Célia Beatriz Gianotti; Grundy, Richard G.; Stannard, F.F. Clare; Dunkel, Ira J.; Grabowski, Eríc F.; Leal-Leal, Carlos; Rodríguez‐Galindo, Carlos; Schvartzman, Enrique; Popović, Maja; Kremens, Bernhard; Meadows, Anna T.; Zucker, Jean‐Michel

doi:10.1002/pbc.20606

Cited by 239 publications

(150 citation statements)

References 22 publications

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“…Most of these results were generated in large referral centers, where multidisciplinary groups that treat retinoblastoma have been established but national cooperative groups have been recently established in Brazil, Mexico, Central America, and Turkey in an attempt to generalize these successful results [24,25,38]. However, until recently, a consensus staging system, common definitions for invasion of the ocular coats and pathology guidelines for the evaluation of enucleated eyes were not available [47,48]. The agreement of such guidelines was a major step toward harmonizing the diagnostic and staging procedures used by different centers in developing countries that would make possible the comparison of treatment results and the design of future prospective studies.…”

Section: Treating Patients With High-risk Pathology Featuresmentioning

confidence: 99%

Strategies to manage retinoblastoma in developing countries

Chantada

Qaddoumi

Canturk³

et al. 2010

Pediatric Blood & Cancer

127

109

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Survival of retinoblastoma is >90% in developed countries but there are significant differences with developing countries in stage at presentation, available treatment options, family compliance, and survival. In low‐income countries (LICs), children present with advanced disease, and the reasons are socioeconomic and cultural. In middle‐income countries (MICs), survival rates are better (>70%), but there is a high prevalence of microscopically disseminated extraocular disease. Programs for eye preservation have been developed, but toxicity‐related mortality is higher. Although effective treatment of microscopically extraocular disease improved the outcome, worldwide survival will be increased only by earlier diagnosis and better treatment adherence. Pediatr Blood Cancer 2011;56:341–348. © 2010 Wiley‐Liss, Inc.

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Section: Treating Patients With High-risk Pathology Featuresmentioning

confidence: 99%

Strategies to manage retinoblastoma in developing countries

Chantada

Qaddoumi

Canturk³

et al. 2010

Pediatric Blood & Cancer

127

109

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“…An independent International Staging is in use, however, which divides patients into stage 0 (treated conservatively), stage I (enucleated, without residual tumour), stage II (enucleated, microscopic residual tumour), stage III (orbital or regional lymph node extension), and stage IV (systemic metastasis or central nervous system extension). 57,58 Children with hereditary retinoblastoma have a 4% risk of developing an ectopic primitive intracranial neuroblastic tumour, known as trilateral retinoblastoma, 59 and are also prone to second cancers later in life. Currently, it is not possible to predict whether such tumours will develop, even when the exact mutation of the retinoblastoma gene is known.…”

Section: Prognosis For Survivalmentioning

confidence: 99%

Prognostication in eye cancer: the latest tumor, node, metastasis classification and beyond

Kivelä

Kujala

2012

Eye

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The tumour, node, metastasis (TNM) classification is a universal cancer staging system, which has been used for five decades. The current seventh edition became effective in 2010 and covers six ophthalmic sites: eyelids, conjunctiva, uvea, retina, orbit, and lacrimal gland; and five cancer types: carcinoma, sarcoma, melanoma, retinoblastoma, and lymphoma. The TNM categories are based on the anatomic extent of the primary tumour (T), regional lymph node metastases (N), and systemic metastases (M). The T categories of ophthalmic cancers are based on the size of the primary tumour and any invasion of periocular structures. The anatomic category is used to determine the TNM stage that correlates with survival. Such staging is currently implemented only for carcinoma of the eyelid and melanoma of the uvea. The classification of ciliary body and choroidal melanoma is the only one based on clinical evidence so far: a database of 7369 patients analysed by the European Ophthalmic Oncology Group. It spans a prognosis from 96% 5-year survival for stage I to 97% 5-year mortality for stage IV. The most accurate criterion for prognostication in uveal melanoma is, however, analysis of chromosomal alterations and gene expression. When such data are available, the TNM stage may be used for further stratification. Prognosis in retinoblastoma is frequently assigned by using an international classification, which predicts conservation of the eye and vision, and an international staging separate from the TNM system, which predicts survival. The TNM cancer staging manual is a useful tool for all ophthalmologists managing eye cancer. Keywords: conjunctival malignancies; eyelid malignancies; orbital malignances; retinoblastoma; uveal melanomaThe tumour, node, metastasis (TNM) classification is a cancer staging system, which has been in worldwide use across all medical specialties for five decades. 1 It emerged in 1968 when the International Union Against Cancer published the first edition of its classification based on the anatomic extent of the tumour after testing a set of draft classifications for 23 cancer sites. None of these sites related to the eye.Indeed, eye cancer is a latecomer in the TNM classification, appearing for the first time in the fourth and fifth editions. 2 The first ophthalmic section covered six sites and five cancer types-carcinoma and melanoma of the eyelid and conjunctiva, melanoma of the uvea, retinoblastoma, sarcoma of the orbit, and carcinoma of the lacrimal gland-but these classifications were untested and were rarely used outside cancer registries.The ophthalmic section was revised for the sixth edition of TNM in 2002. 3 This edition was based on the published literature, but the classifications were not tested against clinical data. The revision received publicity 4 but did not make a breakthrough. Meanwhile, data were collected of other cancers to improve staging. For the updated TNM classification of cutaneous melanoma, data from over 20 000

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“…Patients with bone marrow metastasis, positive cerebrospinal fluid examination, MRI showing involvement of the brain or optic nerve beyond the optic canal, and FNAC-positive lymph nodes other than cervical or preauricular were diagnosed as having metastatic disease (IRSS stage IV) and excluded from the present study. Patients with clinically overt or MRI evidence of extraocular extension without preauricular or cervical lymph node involvement were diagnosed as having IRSS stage IIIA disease, and patients with FNAC-positive cervical or preauricular lymph node were diagnosed as having IRSS stage IIIB disease (4).…”

Section: Study Populationmentioning

confidence: 99%

“…Patients with locally advanced retinoblastoma are at high risk for developing metastatic disease; this risk is evident from the reported overall survival (OS) rates of 20%-70% (2,3). In the recently proposed International Retinoblastoma Staging System (IRSS), microscopic locally advanced disease detected in primarily enucleated specimens is classified as stage II (4). Overt clinical or radiologically diagnosed extraocular disease is classified as stage III, and patients with metastatic disease are classified as stage IV (4).…”

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confidence: 99%

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Role of PET/CT in Staging and Evaluation of Treatment Response After 3 Cycles of Chemotherapy in Locally Advanced Retinoblastoma: A Prospective Study

Radhakrishnan¹,

Kumar²,

Malhotra³

et al. 2012

J Nucl Med

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The present study prospectively evaluated the role of 18 F-FDG PET/CT for staging, neoadjuvant chemotherapy response evaluation, and final outcome assessment in International Retinoblastoma Staging System (IRSS) stage III retinoblastoma. Methods: Twenty-five consecutive IRSS stage III patients, with a median age of 3 y, were prospectively enrolled after ethics approval. All patients received neoadjuvant chemotherapy followed by enucleation, radiotherapy, and adjuvant chemotherapy. PET/CT was performed at baseline (PET/CT-1) and after 3 cycles of neoadjuvant chemotherapy (PET/CT-2). All 25 patients underwent PET/CT-1, and 21 of 25 patients underwent PET/CT-2. PET/CT-1 was compared with routine staging, and response on PET/CT-2 was assessed by criteria from the European Organization for Research and Treatment of Cancer response. Eventfree survival (EFS) and overall survival (OS) were calculated using Kaplan-Meier survival analysis, and differences between the groups were compared using log-rank test. A P value of 0.05 or less was considered significant. Results: Increased 18 F-FDG uptake was noted in primary extraocular tumor in all patients, except 5 with bilateral retinoblastoma (one eye with advanced and the other eye with intraocular disease) in whom the intraocular tumor did not show 18 F-FDG uptake. Five of 22 IRSS stage IIIA patients with clinically negative cervical lymph node involvement were found to have uptake in cervical lymph nodes on PET/CT-1, and 2 of 3 IRSS stage IIIB patients with pathologically confirmed cervical lymph node involvement did not show any uptake in the involved lymph nodes. No significant difference in EFS and OS was seen between IRSS stage IIIA and IIIB patients using routine staging or PET/CT staging (P $ 0.05); however, there was a trend toward better OS in patients with IRSS stage IIIB disease on PET/CT (P 5 0.065). There was no significant discordance between routine staging and PET/CT staging (P 5 0.256). The 8 patients with optic nerve uptake had lower EFS (P 5 0.0001) and OS (P 5 0.0009) than did 17 patients without optic nerve uptake on PET/CT-1. The 17 patients with complete response or partial response had better EFS (P 5 0.042) and OS (P 5 0.026) than did the 4 patients with progressive disease on PET/CT-2. Conclusion: Optic nerve uptake at baseline on PET/CT and response after neoadjuvant chemotherapy according to criteria from the European Organization for Research and Treatment of Cancer are strong predictors of EFS and OS in IRSS stage III retinoblastoma.

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A proposal for an international retinoblastoma staging system

Abstract: The proposed staging system is the product of an international effort to adopt a uniform staging system for patients with retinoblastoma to cover the whole spectrum of the disease.

Cited by 239 publications

References 22 publications

Strategies to manage retinoblastoma in developing countries

Strategies to manage retinoblastoma in developing countries

Prognostication in eye cancer: the latest tumor, node, metastasis classification and beyond

Role of PET/CT in Staging and Evaluation of Treatment Response After 3 Cycles of Chemotherapy in Locally Advanced Retinoblastoma: A Prospective Study

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