Retinoblastoma Outcomes Based on the 8th Edition American Joint Committee on Cancer Pathological Classification in 1411 Patients

Vijitha S Vempuluru; Carol L Shields; Jesse L Berry; Swathi Kaliki; High-Risk Retinoblastoma Collaborative Study Group

doi:10.1016/j.ophtha.2024.08.037

Retinoblastoma Outcomes Based on the 8th Edition American Joint Committee on Cancer Pathological Classification in 1411 Patients

Ophthalmology. 2024 Sep 7:S0161-6420(24)00538-4. doi: 10.1016/j.ophtha.2024.08.037. Online ahead of print.

Authors

Vijitha S Vempuluru¹, Carol L Shields², Jesse L Berry³, Swathi Kaliki⁴; High-Risk Retinoblastoma Collaborative Study Group

Collaborators

High-Risk Retinoblastoma Collaborative Study Group:
Alia Ahmad⁵, Kavya Madhuri Bejjanki⁶, Rosdali Diaz-Coronado⁷, Maya Eiger-Moscovich⁸, James E Elder⁹, Ido Didi Fabian¹⁰, Shahar Frenkel⁸, Hans Grossniklaus¹¹, G Baker Hubbard 3rd¹¹, Swathi Kaliki¹², Anasua Ganguly Kapoor⁶, Mona Mohammad¹³, John D McKenzie¹⁴, Jacob Pe'er⁸, Suryasnata Rath¹⁵, M Ashwin Reddy¹⁶, Olivia Rolfe¹⁴, Soma Rani Roy¹⁷, Mandeep S Sagoo¹⁸, Carol L Shields², Sandra E Staffieri¹⁹, Mika Tanabe²⁰, Ushakova Tatiana²¹, Devjyoti Tripathy¹⁵, Vicktoria Vishnevskia-Dai¹⁰, Vijitha S Vempuluru¹², Polyakov Vladimir²², Yacoub A Yousef¹³

Affiliations

¹ The Operation Eyesight Institute for Eye Cancer, LV Prasad Eye Institute, Hyderabad, India.
² Ocular Oncology Service, Wills Eye Hospital, Philadelphia, Pennsylvania.
³ Children's Hospital Los Angeles & USC Roski Eye Institute, Los Angeles, California; Keck School of Medicine, Los Angeles, California.
⁴ The Operation Eyesight Institute for Eye Cancer, LV Prasad Eye Institute, Hyderabad, India. Electronic address: [email protected].
⁵ University of Children Health Sciences, Children's Hospital, Lahore, Pakistan.
⁶ LV Prasad Eye Institute, Vijayawada, India.
⁷ Pediatric Oncology Department, Instituto Nacional de Enfermedades Neoplásicas, Lima, Peru; Universidad Peruana Cayetano Heredia, Lima, Peru.
⁸ Hadassah Medical Center, Jerusalem, Israel.
⁹ Department of Ophthalmology, Royal Children's Hospital, Victoria, Australia; University of Melbourne, Melbourne, Australia.
¹⁰ Goldschleger Eye Institute, Sheba Medical Center, Tel Hashomer, Faculty of Medicine, Tel-Aviv University, Tel Aviv, Israel.
¹¹ Emory University School of Medicine, Atlanta, Georgia.
¹² The Operation Eyesight Universal Institute for Eye Cancer, LV Prasad Eye Institute, Hyderabad, India.
¹³ King Hussein Cancer Center, Amman, Jordan.
¹⁴ Department of Ophthalmology, Royal Children's Hospital, Victoria, Australia.
¹⁵ LV Prasad Eye Institute, Bhubaneswar, India.
¹⁶ Royal London Hospital, Barts Health NHS Trust, London, United Kingdom.
¹⁷ Chittagong Eye Infirmary and Training Complex, Chittagong, Bangladesh.
¹⁸ Royal London Hospital, Barts Health NHS Trust, London, United Kingdom; Moorfields Eye Hospital NHS Foundation Trust, London, United Kingdom.
¹⁹ Department of Ophthalmology, Royal Children's Hospital, Victoria, Australia; University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia; Centre for Eye Research Australia, Royal Victorian Eye and Ear Hospital, Melbourne, Australia.
²⁰ Kyushu University, Fukuoka, Japan.
²¹ Department of Surgical Methods of Treatment with Chemotherapy No. 1 (Head and Neck Tumors), N. N. Blokhin National Medical Research Center of Oncology, Moscow, Russian Federation; L.A. Durnov Department of Pediatric Oncology of the Russian Medical Academy of Continuing Professional Education, Moscow, Russian Federation.
²² Department of Surgical Methods of Treatment with Chemotherapy No. 1 (Head and Neck Tumors), N. N. Blokhin National Medical Research Center of Oncology, Moscow, Russian Federation; L.A. Durnov Department of Pediatric Oncology of the Russian Medical Academy of Continuing Professional Education, Moscow, Russian Federation; Department of ENT Diseases of the Faculty of Pediatrics of the Federal State Educational Institution of Higher Education of N.I. Pirogov Russian National Research Medical University, Moscow, Russian Federation.

PMID: 39245078
DOI: 10.1016/j.ophtha.2024.08.037

Abstract

Purpose: To evaluate the outcomes of retinoblastoma (RB) based on the 8^th edition of the American Joint Committee on Cancer (AJCC) pathological classification in a global cohort of patients.

Design: Retrospective, multicenter, intercontinental, collaborative study.

Participants: A total of 1411 patients.

Intervention: Primary enucleation with or without adjuvant chemotherapy or radiotherapy.

Main outcome measures: Orbital tumor recurrence, tumor-related metastasis, and tumor-related death.

Results: According to the 8^th edition AJCC pathological classification, 645 eyes (46%) belonged to pathological T (pT)1, 164 (11%) to pT2, 493 (35%) to pT3, and 109 (8%) to pT4 categories. At a mean follow-up of 38 months (median, 35 months; < 1-149 months), orbital tumor recurrence was seen in 8 (1%), 5 (3%), 22 (4%), and 25 (23%) of pT1, pT2, pT3, and pT4 (P < 0.001) categories, respectively; tumor-related metastasis was seen in 7 (1%), 5 (3%), 40 (8%), and 46 (43%) of pT1, pT2, pT3, and pT4 (P < 0.001) categories, respectively; tumor-related death was seen in 12 (2%), 7 (4%), 64 (13%), and 64 (59%) of pT1, pT2, pT3, and pT4 (P < 0.001) categories, respectively. Multivariate Cox proportional hazards analysis of outcomes revealed pT category and adjuvant therapy as independent predictors of outcomes. Categories pT3b (P = 0.005), pT3c (P < 0.001), pT3d (P < 0.001), and pT4 (P < 0.001) had a greater hazard for orbital recurrence; categories pT2a (P = 0.015), pT3a (P < 0.001), pT3b (P < 0.001), pT3c (P < 0.001), pT3d (P < 0.001), and pT4 (P < 0.001) had a greater hazard for tumor-related metastasis; and categories pT2a (P = 0.068), pT2b (P = 0.004), pT3a (P < 0.001), pT3b (P < 0.001), pT3c (P < 0.001), pT3d (P < 0.001), and pT4 (P < 0.001) had a greater hazard for tumor-related death when compared with the pT1 category. Patients who did not receive adjuvant therapy had greater hazards of orbital tumor recurrence in categories pT3b (P = 0.005), pT3c (P = 0.003), and pT4 (P = 0.002); greater hazards of tumor-related metastasis in categories pT3a (P = 0.001), pT3b (P = 0.01), pT3c (P = 0.001), and pT4 (P = 0.007); and tumor-related death in categories pT3a (P < 0.001), pT3b (P = 0.009), pT3c (P = 0.018), and pT4 (P < 0.001) when compared with those who received adjuvant therapy.

Conclusions: The 8^th edition AJCC pathological classification predicts outcomes in patients undergoing primary enucleation for RB, and adjuvant therapy is associated with a lower risk of orbital recurrence, tumor-related metastasis, and tumor-related death in the pT3 and pT4 categories.

Financial disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Keywords: AJCC; American Joint Committee on Cancer Classification; Global Study; High-risk histopathological features; Retinoblastoma.