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1. Features of regression of different stages of retinoblastoma after primary combined (intravitreal plus systemic) polychemotherapy and additional consolidation therapy

Author

Nadiya Bobrova, Svitlana Tronina, Tetyana Sorochynska, Tetyana Romanova, Ganna Dembovetska, A.V. Shylyk, and O.D. Dovgan

Subjects
Internal medicine, RC31-1245
Abstract

Background: Complete retinoblastoma (RB) regression is defined as final changes undergone by the tumor in the course of eye-salvage treatment. Studies of RB regression patterns following various types of eye-salvage treatment are important for assessing the outcomes of this treatment. Purpose: To study the regression patterns in different stages of RB after primary combined polychemotherapy (PCPC) and additional consolidation therapy. Material and Methods: We reviewed RB regression patterns in 89 children (119 eyes) aged 1.5 to 77 months after PCPC and various types of consolidation therapy. Of these children, 37 had unilateral retinoblastoma, and 52, bilateral retinoblastoma. At presentation, T3 RB was the most prevalent (67.2%), followed by T2 (23.6%). T1 stage was observed only in 9.2% - most often it was diagnosed on a fellow "healthy" eye at a spreading T3 stage of the countralateral eye. Sixteen eyes (13%) had multifocal tumors type of tumor growth. Because the number of tumor foci per eye ranged from one to three, the total number of foci (124) exceeded the total number of affected eyes. Treatment was carried out according to the developed PСPС method, which invсcluded the intravitral injection of melfalan at the dose of 10-30 mcg, depending on the tumor stage, followed by intravenous systemic therapy (VEC protocol). Results: We found different RB regression patterns after PCPC. After the first cycle of PCPC, type 2 regression pattern was typical for small T1 tumors, whereas type 3 regression pattern was most prevalent (60%) for T2 and T3 tumors. This is likely to indicate tumor mosaicism with the presence of less differentiated and, consequently, more malignant cell types, which faster reacted to PCPC by calcification than surrounding more differentiated and, consequently, less malignant cells which reacted weaker. After the completion of PCPC, type 1 regression pattern was seen in 29%, which indicated complete regression, whereas type 3 regression pattern persisted in 33% foci. The features of tumor regression following PCPC included (a) fragmentation of a large RB (59.3%) after first 1-2 PCPC cycles with appearance of necrotic foci which resolved or calcified finally; (b) presence of various types of regression in one eye in the multifocal growth; and (c) transformation of a regression type into another regression type: most commonly, transformation of type 2 into type 3 and type 3 into type 1.

Published
2023
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2. Incidence of Retinoblastoma Has Increased: Results from 40 European Countries

Author

Andrew W. Stacey, Richard Bowman, Allen Foster, Tero T. Kivelä, Francis L. Munier, Nathalie Cassoux, Ido Didi Fabian, Lamis Al Harby, Silvia Alarcón Portabella, Donjeta B. Alia, Charlotta All-Eriksson, Romanzo Antonino, Nicholas J. Astbury, Julia Balaguer, Walentyna Balwierz, Honorio Barranco, Covadonga Bascaran, Maja Beck Popovic, Eva M. Biewald, Nadia Bobrova, Norbert Bornfeld, Bénédicte G. Brichard, Sharon Blum, Michael Capra, Guilherme Castela, Jaume Català-Mora, Guillermo L. Chantada, Violeta S. Chernodrinska, Krzysztof Cieslik, Codruta Comsa, Maria G. Correa Llano, Monika Csóka, Patrick De Potter, Laurence Desjardins, Monica D. Dragomir, Ana Fernández-Teijeiro, David García Aldana, Pernille A. Gregersen, Nir Gomel, Theodora Hadjistilianou, Stanislava Hederova, Marlies Hummlen, Kristina Husakova, Russo Ida, Vesna R. Ilic, Helen Jenkinson, Noa Kapelushnik, Tamar Kardava, Naama Keren-Froim, Tomas Kepak, Zaza Khotenashvili, Artur Klett, Dalia Krivaitiene, Slobodanka Latinović, Livia Lumbroso, Lesia Lysytsia, Erika Maka, Nieves Martín Begue, Edoardo Midena, Annette C. Moll, Gabriela Murgoi, Larisa Naumenko, Vladimir Neroev, Marina Nikitovic, Andrzej Olechowski, Ruzanna Papyan, Raffaele Parrozzani, Manoj V. Parulekar, Katarzyna Pawinska-Wasikowska, Sanja Perić, Pavel Pochop, Vladimir G. Polyakov, M. Ashwin Reddy, Petra Ritter-Sovinz, Svetlana Saakyan, Mandeep S. Sagoo, Sonsoles San Román Pacheco, Stefan Seregard, Sónia Silva, Tetyana Sorochynska, Christina Stathopoulos, Branka Stirn Kranjc, Karel Svojgr, Gevorg Tamamyan, Alketa Tandili, Bekim Tateshi, Manca Tekavcic Pompe, Steen F. Urbak, Tatiana L. Ushakova, Sandra Valeina, Milo van Hoefen Wijsard, Nevyana V. Veleva-Krasteva, Maris Viksnins, Werner Wackernagel, Charlotte Wolley Dod, Vera A. Yarovaya, Andrey A. Yarovoy, Katsiaryna Zhilyaeva, Marcia Zondervan, Ophthalmology, ACS - Diabetes & metabolism, APH - Health Behaviors & Chronic Diseases, APH - Quality of Care, CCA - Cancer Treatment and quality of life, CCA - Imaging and biomarkers, and Amsterdam Reproduction & Development (AR&D)

Subjects
Male, Pediatrics, medicine.medical_specialty, Adolescent, Retinal Neoplasms, MEDLINE, 03 medical and health sciences, 0302 clinical medicine, Familial, Genetic, Fitness, Medicine, Humans, Child, business.industry, Retinoblastoma, Incidence (epidemiology), Incidence, Infant, Newborn, Infant, medicine.disease, Europe, Ophthalmology, Cross-Sectional Studies, 030220 oncology & carcinogenesis, Child, Preschool, 030221 ophthalmology & optometry, Female, business
Published
2021

3. Travel burden and clinical presentation of retinoblastoma: analysis of 1024 patients from 43 African countries and 518 patients from 40 European countries

Author

Tero Kivelä, Marina Nikitovic, Dalia Krivaitiene, Tamar Kardava, Stefan Seregard, Kristína Husáková, Fousseyni Traore, Claude Moreira, Vera Yarovaya, Donjeta B. Alia, Chatonda Manda, Mariana Kruger, Ghislaine Obono-Obiang, Argentino A. Almeida, Nevyana V. Veleva-Krasteva, Petra Ritter-Sovinz, Bekim Tateshi, Dupe S Ademola-Popoola, Sanja Perić, Oluyemi Fasina, Jennifer Geel, Steen F Urbak, Grace Ssali, Katsiaryna Zhilyaeva, Nieves Martín Begue, Aemero A. Mengesha, Gerald Msukwa, Larisa Naumenko, Monica D. Dragomir, Gabrielle C. Bouda, P. Kemilev, Henry Nkumbe, Amany M. Ali, Slobodanka Latinović, Amaria Boumedane, Ido Didi Fabian, Jaume Català-Mora, Walentyna Balwierz, Theodora Hadjistilianou, Marlies Hummlen, Laila Hessissen, Shelley J. Biddulph, Vesna R. Ilic, Okwen M. Muyen, Michael Capra l, Edoardo Midena, Shehu U. Abdullahi, Ruzanna Papyan, Allen Foster, Annette C. Moll, Laurence Desjardins, Covadonga Bascaran, Sónia Silva, Ahmed A. Mohamedani, Krzysztof Cieslik, Naama Keren-Froim, Milo van Hoefen Wijsard, Mahmoud M. Elzembely, Marchelo Matua, Sidi Sidi Cheikh, Charlotta All-Eriksson, Ana Fernández-Teijeiro, Furahini G. Mndeme, Karin Lecuona, David Garcia Aldana, Katarzyna Pawinska-Wasikowska, Maja Beck Popovic, Léa Raobela, Vladimir Neroev, Remezo Philbert, Kareem Olatunbosun Musa, Gita Naidu, Mutale Nyaywa, Rolande L. Kabore, Shirley Sharabi, Erika Maka, Abubakar Kalinaki, Alaa Elhaddad, Charlotte Wolley Dod, Stanislava Hederová, Jenny M. Yanga, Nissrine Bennani Guebessi, Helen Jenkinson, Sally Painter, Russo Ida, Werner Wackernagel, Harba Tyau-Tyau, Honorio Barranco, Sadik Taju Sherief, Diriba F. Hordofa, Jonathan J. Pons, Bénédicte Brichard, David K Stones, Guilherme Castela, S.V. Saakyan, Peter Osei-Bonsu, Amal M. Elbahi, Zelalem Gizachew, Codruta Comsa, Ali Bala Umar, Mussagy M. Riheia, Manca Tekavčič Pompe, Elhassan Abdallah, Noa Kapelushnik, Sadiq Hassan, Magritha Du Bruyn, Rose Atsiaya, Robert M Lukamba, Tomáš Kepák, Freddy B. Mbumba, Karel Svojgr, Asmaa El Kettani, Branka Stirn Kranjc, Monika Csóka, Norbert Bornfeld, Pavel Pochop, Sonsoles San Roman Pacheco, Silvia Alarcón Portabella, Amelia D.C. Wime, Ngozi C Oguego, Keith M. Waddell, Nathalie Cassoux, Dina Elgalaly, Eva Biewald, Fatoumata Sylla, Pernille Axel Gregersen, Hamzah Mustak, Lamis Al Harby, Ada Aghaji, Othman Ao Ziko, Sharon Blum, Theophile B Amani Kabesha, Nick Astbury, Rokia C. Berete, Andrew W. Stacey, V.G. Polyakov, Kangwa I. Muma, Marianna Boehme, Vivian Paintsil, Richard Bowman, Vera Adobea Essuman, Koffi M. Guedenon, Amina H Wali Nigeria, Julia Balaguer, Maris Viksnins, Ismail Mayet, Tetyana Sorochynska, Patrick De Potter, Alketa Tandili, Lesia Lysytsia, Tatiana L Ushakova, Judy Schoeman, Sandra Valeina, Moawia Mohammed Ali Elhassan, Moira Gandiwa, Alice Kyara, M. Ashwin Reddy, Livia Lumbroso, Isac V. Da Gama, Marcia Zondervan, Zaza Khotenashvili, Hédi Bouguila, Azza Ma Said, Nadia Bobrova, Faraja S. Chiwanga, Christina Stathopoulos, Alan Davidson, Sarra Benmiloud, Francis L. Munier, Mayuri Makan, Eva S Lachmann, Gevorg Tamamyan, Romanzo Antonino, Ibrahim O. Matende, Guillermo Chantada, AO Adio, Jess Elio Kosh Komba Palet, Manoj Parulekar, Luisa Paiva, Trish A. Scanlan, Nir Gomel, Khalifa M. Alsawidi, Artur Klett, Violeta Chernodrinska, Ekhtelbenina Zein, Mandeep S. Sagoo, Sahadatou Adamou Boubacar, A. Oscar, Raffaele Parrozzani, McHikirwa S. Msina, David Reynders, Elizabeth D Nkanga, Gabriela Murgoi, Andrey A. Yarovoy, Dahiru Ribadu, PD Wade, Amadou I. Alfa Bio, Lorna Renner, Ted Grimbert A. Evina, Paule Aïda Ndoye Roth, Maria G. Correa Llano, Tuyisabe Theophile, and Andrzej Olechowski

Subjects
medicine.medical_specialty, Referral, Epidemiology, Retinal Neoplasms, Medizin, Disease, 03 medical and health sciences, Cellular and Molecular Neuroscience, 0302 clinical medicine, Risk Factors, Neoplasia, Medicine, Humans, business.industry, Retinoblastoma, Extraocular Retinoblastoma, medicine.disease, eye diseases, Sensory Systems, 3. Good health, Ophthalmology, Cross-Sectional Studies, 030220 oncology & carcinogenesis, Africa, 030221 ophthalmology & optometry, Health education, Residence, Presentation (obstetrics), Epidemiology, Neoplasia, business, human activities, Demography
Abstract

BackgroundThe travel distance from home to a treatment centre, which may impact the stage at diagnosis, has not been investigated for retinoblastoma, the most common childhood eye cancer. We aimed to investigate the travel burden and its impact on clinical presentation in a large sample of patients with retinoblastoma from Africa and Europe.MethodsA cross-sectional analysis including 518 treatment-naïve patients with retinoblastoma residing in 40 European countries and 1024 treatment-naïve patients with retinoblastoma residing in 43 African countries.ResultsCapture rate was 42.2% of expected patients from Africa and 108.8% from Europe. African patients were older (95% CI −12.4 to −5.4, pConclusionsFewer than half the expected number of patients with retinoblastoma presented to African referral centres in 2017, suggesting poor awareness or other barriers to access. Despite the relatively shorter distance travelled by African patients, they presented with later-stage disease. Health education about retinoblastoma is needed for carers and health workers in Africa in order to increase capture rate and promote early referral.

Published
2020

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