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

Author(s):  
Sabine Dittner-Moormann ◽  
Madlen Reschke ◽  
Floor Abbink ◽  
Isabelle Aerts ◽  
Hatice Atalay ◽  
...  

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.

2018 ◽  
Vol 29 (2) ◽  
pp. 262-268 ◽  
Author(s):  
Jamila G Hiasat ◽  
Alaa Saleh ◽  
Maysa Al-Hussaini ◽  
Ibrahim Al Nawaiseh ◽  
Mustafa Mehyar ◽  
...  

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.


2013 ◽  
Vol 31 (11) ◽  
pp. 1458-1463 ◽  
Author(s):  
Isabelle Aerts ◽  
Xavier Sastre-Garau ◽  
Alexia Savignoni ◽  
Livia Lumbroso-Le Rouic ◽  
Estelle Thebaud-Leculée ◽  
...  

Purpose The objective of this prospective study was to assess overall survival and event-free survival in patients with intraocular unilateral retinoblastoma (Reese-Ellsworth group V) treated by primary enucleation with or without adjuvant therapy depending on histopathologic risk factors. Patients and Methods Patients (n = 123) were divided into three groups on the basis of risk factors for extraocular relapse and metastasis assessed on centralized histologic examination of enucleated eyes. Group 1 (n = 70) had minimal or no choroidal involvement and/or prelaminar or no optic nerve involvement and received no adjuvant therapy. Group 2 (n = 52) had massive choroidal involvement and/or intra- or retrolaminar optic nerve involvement and/or anterior segment involvement and received four courses of adjuvant chemotherapy. Group 3 (n = 1) had invasion of the surgical margin of the optic nerve and/or microscopic extrascleral involvement and received six courses of adjuvant chemotherapy with intrathecal thiotepa, consolidation chemotherapy, and autologous stem-cell rescue. Genetic testing was also performed. Results Median follow-up for the 123 patients was 71 months. No disease progression, relapse, or distant metastasis occurred during follow-up. No second malignancies occurred. This requires confirmation with longer follow-up. Secondary bilateralization occurred in two patients with identified RB1 germline mutation. Adjuvant chemotherapy was well tolerated, with limited toxicity. Molecular testing found constitutional RB1 gene mutations in only nine of 100 evaluated patients. Conclusion The survival rate of 100% was excellent, including 57% of patients who received no adjuvant therapy, suggesting that chemotherapy could be de-escalated in some patients, especially those with massive choroidal involvement.


Cancer ◽  
1994 ◽  
Vol 73 (3) ◽  
pp. 692-698 ◽  
Author(s):  
Carol L. Shields ◽  
Jerry A. Shields ◽  
Karin Baez ◽  
Jacqueline R. Cater ◽  
Patrick de Potter

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 ◽  
...  

2015 ◽  
Vol 5 (9) ◽  
pp. 723-726
Author(s):  
S Karki ◽  
P Upadhyaya ◽  
M Agarwal ◽  
KK Maharjan ◽  
P Lavaju

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


2006 ◽  
Vol 130 (11) ◽  
pp. 1669-1672 ◽  
Author(s):  
Eva-Marie Chong ◽  
Robert E. Coffee ◽  
Murali Chintagumpala ◽  
Richard L. Hurwitz ◽  
Mary Y. Hurwitz ◽  
...  

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 &lt; .001), post-lamina-cribrosal invasion (P &lt; .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.


2021 ◽  
pp. 1-12
Author(s):  
Chenghua Yuan ◽  
Qingyu Yao ◽  
Lei Cheng ◽  
Can Zhang ◽  
Longbing Ma ◽  
...  

OBJECTIVE Knowledge on the management of spinal cord astrocytoma (SCA) remains scarce. Here, the authors constructed and validated a predictive nomogram, often used for individualized prognosis and evaluations, to estimate cancer-specific survival (CSS) and overall survival (OS) for patients with SCA. METHODS Epidemiological characteristics were compared between low-grade SCA (LGSCA) and high-grade SCA (HGSCA) patients from the Surveillance, Epidemiology, and End Results (SEER) database. Risk factors for CSS and OS were determined using univariate and multivariate analyses and Kaplan-Meier curves. A nomogram was developed to individually predict the 3-, 5-, and 10-year CSS and OS rates. The clinical usefulness of the nomogram was assessed using calibration plots, the concordance index (C-index), and time-dependent receiver operating characteristic curves. RESULTS A total of 468 LGSCA and 165 HGSCA patients were eligible for inclusion. LGSCA and HGSCA patients demonstrated differences in age, tumor extension, insurance status, adjuvant treatment, and survival. Multivariate analysis demonstrated that in the LGSCA group, tumor extension, surgery type, and adjuvant therapy were individually associated with CSS. The distance of tumor extension and WHO grade were individually associated with CSS in the HGSCA group. The prognostic variables were further demonstrated using the Kaplan-Meier method, which also suggested that adjuvant treatment provided no advantage to HGSCA patients. A nomogram was constructed, and the C-index for CSS was 0.84 by internal validation (95% CI 0.79–0.90). CONCLUSIONS This research suggests that the distance of tumor extension, type of surgery, and adjuvant therapy are significant risk factors for CSS using multivariate analysis in the LGSCA group. Adjuvant treatment provided no advantages for CSS or OS in patients with HGSCAs. The nomogram may be clinically useful to healthcare providers.


2011 ◽  
Vol 135 (8) ◽  
pp. 1017-1023 ◽  
Author(s):  
Pallavi Suryawanshi ◽  
Mukta Ramadwar ◽  
Rajesh Dikshit ◽  
Shubhada V Kane ◽  
Purna Kurkure ◽  
...  

Context.—Advanced cases of retinoblastoma are treated with chemoreduction followed by enucleation. Further adjuvant therapy is recommended in patients with known pathologic risk factors (PRFs). Objectives.—To determine the PRFs in enucleated specimens after chemoreduction and their association for adverse events of recurrence, metastasis, or death. Design.—This was a retrospective study of 77 enucleation specimens from patients treated between January 2000 and September 2008 with prior chemoreduction that were accessioned in the pathology department of a tertiary referral cancer center with an average follow-up of 24 months. Various PRFs were noted and their association with the development of an adverse event was recorded. Results.—Of 77 patients, (male to female ratio, 51∶26), the incidence of overall PRF was 51.9%, and retrolaminar optic nerve invasion (32.5%), optic nerve cut margin (12.9%), massive choroidal invasion (26%), scleral invasion (23.4%), vitreous seedings (44.2%), and anterior segment invasion (20.8%). Undifferentiated tumor (&gt;60%) was seen in 60.3% of cases (41 of 68 patients with differentiation available). Adverse event occurred in 18 of 72 patients with available follow-up (25%). Retrolaminar optic nerve invasion, optic nerve cut margin involvement, and scleral invasion were independent prognostic factors predicting the occurrence of an adverse event. Undifferentiated tumor (&gt;60%) was a significant risk factor in univariate analysis, which is the unique feature in this study. Conclusions.—Classic PRF with the addition of a predominant presence from the undifferentiated component were associated with adverse outcomes in retinoblastoma treated with anterior chemotherapy. The latter may represent chemoresistant clones and more intensive adjuvant chemotherapy may be warranted in these patients.


2012 ◽  
Vol 32 (S 01) ◽  
pp. S39-S42 ◽  
Author(s):  
S. Kocher ◽  
G. Asmelash ◽  
V. Makki ◽  
S. Müller ◽  
S. Krekeler ◽  
...  

SummaryThe retrospective observational study surveys the relationship between development of inhibitors in the treatment of haemophilia patients and risk factors such as changing FVIII products. A total of 119 patients were included in this study, 198 changes of FVIII products were evaluated. Results: During the observation period of 12 months none of the patients developed an inhibitor, which was temporally associated with a change of FVIII products. A frequent change of FVIII products didn’t lead to an increase in inhibitor risk. The change between plasmatic and recombinant preparations could not be confirmed as a risk factor. Furthermore, no correlation between treatment regimens, severity, patient age and comorbidities of the patients could be found.


2020 ◽  
Vol 91 (12) ◽  
pp. 940-947
Author(s):  
Matthias Albermann ◽  
Maria Lehmann ◽  
Christian Eiche ◽  
Joachim Schmidt ◽  
Johannes Prottengeier

BACKGROUND: In their working life, airline pilots are exposed to particular risk factors that promote nonspecific low back pain (LBP). Because of the varying incidence internationally, we evaluated the point prevalences of acute, subacute, and chronic nonspecific LBP, as well as the current prevalences in German airline pilots. Furthermore, we compared the prevalence to the general German population and to European counterparts.METHODS: An anonymous online survey of 698 participating German airline pilots was evaluated. The impairment between groups was analyzed. Prevalences from our data were compared to existing data.RESULTS: The following point prevalences were found: 8.2% acute, 2.4% subacute, 82.7% chronic LBP; 74.1% of all individuals were suffering from current LBP when answered the questionnaire. A total time spent flying greater than 600 h within the last 12 mo was significantly related to acute nonspecific LBP. Individuals with any type of LBP were significantly impaired compared to those unaffected. It was found that German airline pilots suffer more often from current LBP than the general population and have a higher point prevalence of total LBP than their European counterparts.CONCLUSIONS: The evaluation showed a surprisingly high, previously unidentified, prevalence of nonspecific LBP in German airline pilots. Why German airline pilots suffer more often from LBP remains uncertain. The number of flying hours appears to have a negative effect on developing acute low back pain, but causation cannot be concluded. Other risk factors could not be confirmed.Albermann M, Lehmann M, Eiche C, Schmidt J, Prottengeier J. Low back pain in commercial airline pilots. Aerosp Med Hum Perform. 2020; 91(12):940947.


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