A New Era of Organ-Preserving Treatment in Pediatric Intraocular Retinoblastoma in Russia: A Multicenter Cohort Study

2018 ◽  
Vol 5 (1) ◽  
pp. 51-69 ◽  
Author(s):  
Тatiana L. Ushakova ◽  
Igor A. Тrofimov ◽  
Оlga V. Gorovtsova ◽  
Аndrey A. Yarovoy ◽  
Svetlana V. Saakyan ◽  
...  

Background.Retinoblastoma (RB) is a life threatening cancer disease. A breakthrough in the treatment of children with RB is associated with the improvement of conservative treatment that was administered in at least one of the two tumor-affected eyes in most bilateral cases, that was chemotherapy both systemic and local (selective intra-arterial and intravitreal) in most cases combined with laser therapy, cryotherapy, or brachytherapy. The development of such techniques as local chemotherapy is focused on preservation of visual functions, reducing the number of enucleations and radiotherapy (RT) course. The success of the healing of RB is closely associated with a multidisciplinary approach to diagnosis and treatment, as well as specialized longterm follow-up clinical examination.Objective.eye and vision preservation against large intraocular tumors with different growth types and localization without the course of remote radiation therapy was the main purpose.Methods.In the period from September 2012 to January 2016, the study enrolled 45 patients with RB when at least one eye had intraocular tumor spread corresponding to the group C or D. According to the ABC international classification, patients have a relatively good prognosis for organ-preserving treatment. 4 of 18 children with bilateral RB had undergone primary enucleation of worse eye the worst eye, group E; 49 (77.8%) of the 63 affected eyes had features for groups C and D. In this study, no patient received local chemotherapy initially, only after prior systemic chemotherapy. Selective intra-arterial chemotherapy (SIAC) was applied to 41 patients (45 eyes; mean course number was 2), and 32 patients (34 eyes) had undergone intravitreal chemo therapy (IViC) (mean course number was 2). Focal therapy and local chemotherapy were the main methods of treatment for progression (new lesions on the retina) in 8 (16.3%) of 49 eyes with tumors of group C (n=1) and D (n=7); the relapse in 14 of 49 (new lesions on the retina) in eyes with tumors of group C (n=5) and D (n=6) and (new lesions on the retina and the vitreous) in eyes with tumors of group D (n=3) (28.5%), and stabilization of disease n=23 (46.9%). We should note that 2 patients underwent repeated course of in case of systemic chemotherapy, 1 patient — a Gamma Knife procedure due to registered disease stabilization, progression or relapse.Results.10 (20.4%) of 49 eyes saved due to the combined chemotherapy. In 45 patients diseasefree survival rate was 56.1±8.9 % (with mean follow-up period 26.9±2.5 months). 1 of 45 patients died from leukemia. 44 of 45 patients are alive without metastasis. The mean follow-up was 20 months (3 to 43 months). Eye salvage rate in group C — 14 (93.3%) of 15, in group D — 31 (91.2%) of 34.Conclusion.These methods: second line of systemic chemotherapy, RT, and a Gamma Knife procedure should be considered as a failure of primary treatment. Our study demonstrated a high efficacy of local chemotherapy with promissing techniques of conservation therapy, which safety increases due to experience.

2020 ◽  
pp. 112067212095758
Author(s):  
Ibadulla Mirzayev ◽  
Ahmet Kaan Gündüz ◽  
Kıvılcım Yavuz ◽  
Mehmet Zahid Şekkeli ◽  
Funda Seher Özalp Ateş ◽  
...  

Purpose: To evaluate the results of secondary intra-arterial chemotherapy (IAC) and/or intravitreal chemotherapy (IVC) as a salvage treatment for retinoblastoma (RB). Materials and Methods: The medical records of 31 (20 male, 11 female) cases (with 38 eyes) who underwent secondary IAC and/or IVC between February 2010 and June 2019 were retrospectively reviewed. Results: Thirteen (41.9%) cases had unilateral and 18 (58.1%) had bilateral RB. According to the International Classification of RB, 6 (15.8%) eyes had group B, 9 (23.7%) eyes had group C, 16 (42.1%) eyes had group D, and 7 (18.4%) eyes had group E RB at diagnosis. All patients underwent six-cycle intravenous chemotherapy as primary treatment and 8 eyes received external radiotherapy before IAC/IVC. Secondary IAC was performed in 21 (55.3%) eyes, IVC in 10 (26.3%) eyes, and IAC + IVC in 7 (18.4%) eyes. External radiotherapy was applied in 2 (5.3%) eyes after IAC/IVC, one of which was later enucleated. In total, 17 (44.7%) eyes undergoing secondary IAC/IVC treatments were enucleated. Metastasis and death were not observed in any case during the mean follow-up period of 59.3 (median 61, range: 10–98) months. Discussion: Although 60.5% of the eyes undergoing IAC/IVC consisted of groups D and E RB, globe salvage and survival rates were 55.3% and 100.0%, respectively. External radiotherapy was required in 5.3% of the eyes after IAC/IVC. In conclusion, IAC and IVC are safe and effective treatment methods in eyes with RB unresponsive to other eye-preserving treatments and those demonstrating recurrence.


2017 ◽  
Vol 102 (4) ◽  
pp. 490-495 ◽  
Author(s):  
Raksha Rao ◽  
Santosh G Honavar ◽  
Vishal Sharma ◽  
Vijay Anand P Reddy

Background/aimTo evaluate the efficacy of intravitreal topotecan for refractory or recurrent vitreous seeds in retinoblastoma.MethodsIntravitreal injection of topotecan hydrochloride (30 µg/0.15 mL) was provided every 3 weeks by the safety enhanced technique.ResultsThe study included 17 consecutive patients with retinoblastoma with refractory or recurrent vitreous seeds. Five eyes (29%) belonged to International Classification of Retinoblastoma group C and 12 eyes (71%) belonged to group D. Primary treatment included triple drug intravenous chemotherapy for a mean of 10 cycles (median, 9 cycles; range, 6–18 cycles). Fifteen patients (88%) had undergone 56 periocular carboplatin injections with a mean of 4 injections (median, 3 injections; range, 1–8 injections), concurrent with intravenous chemotherapy. A total of 53 intravitreal topotecan injections were performed in 17 eyes of 17 consecutive patients with refractory or recurrent vitreous seeds with a mean of 3 injections (median, 3 injections; range, 2–6 injections). Complete regression of vitreous seeds was achieved in 17 of 17 eyes (100%). At a mean follow-up of 23.8 months (median, 24 months; range, 15.1–34.1 months), one eye (6%) with a recurrent retinal tumour needed enucleation, and the rest of the 16 eyes (94%) maintained complete regression. Final visual acuity could be reliably assessed in all 16 eyes (100%), of whom 12 eyes (75%) had visual acuity ≥20/200. None of the patients developed ocular or systemic complications.ConclusionThree-weekly intravitreal topotecan appears effective and safe in controlling focal or diffuse refractory or recurrent vitreous seeds in retinoblastoma.


2017 ◽  
Vol 35 (6_suppl) ◽  
pp. e539-e539
Author(s):  
Igor Nunes-Silva ◽  
Eric Barret ◽  
Mohammed Baghdadi ◽  
Victor Srougi ◽  
Silvia Garcia Barreras ◽  
...  

e539 Background: Magnetic resonance imaging (MRI) plays an important role as a treatment-monitoring tool along focal therapy (FT) follow-up. This is the first study to assess MRI ability to correctly predict prostate cancer (PCa) local T-stage in the FT postoperative scenario. We aimed to describe MRI effectiveness in predicting upstaging in two groups of men: 1) men who failure after primary FT and then underwent salvage robotic-assisted radical prostatectomy (S-RARP) and 2) men who underwent RARP as primary treatment (P-RARP). Methods: Prospective data of 2775 men underwent RARP for localized PCa from 2000 to 2016 were reviewed. Twenty-two men underwent S-RARP after FT failure (S-RARP group). Total 2750 underwent RARP as first treatment. Matched-pair 1:2 selection of 44 out of 2750 patients by age defined primary RARP group (P-RARP). All patients underwent MRI immediately before RARP. MRI findings were confronted with final surgical pathology. Primary endpoint: sensitivity, specificity, positive and negative predictive values; positive (+LR) and negative (-LR) likelihood ratio regarding upstaging analysis on S-RARP. Secondary endpoint: same effectiveness analysis on P-RARP. Results: Preoperative MRI failed in predicting upstaging in 80% versus 91.7% of patients (p = 0.515) that presented final pathological status ≥ pT3a on S-RARP and P-RARP groups, respectively. On the other hand, when final pathology described a localized disease T2a-T2c, MRI correctly predicted the final pathological status in 81.8% versus 95.2% of patients (p = 0.27) on S-RARP and P-RARP groups, respectively. Between-group analysis, showed sensitivity and specificity rates of 20% versus 8.33% and 81.8% versus 95.23%, respectively; Positive and negative predictive values of 33.33% versus 50% and 69.23% versus 64.51%, respectively; Positive (+LR) and negative (-LR) likelihood ratio of 1.1 versus 1.74 and 0.98 versus 0.96, respectively. Conclusions: MRI has shown to be a weak diagnostic tool for predicting extra-prostatic disease along FT follow-up. Urologists may be warned about the risk of underdiagnosis and undertreatment in patients presenting failure after FT.


2019 ◽  
Vol 104 (2) ◽  
pp. 208-213 ◽  
Author(s):  
Jose J Echegaray ◽  
Yahya A Al-Zahrani ◽  
Arun Singh

Background/AimsTo report visual outcomes, survival outcomes and complications following episcleral brachytherapy (EB) for retinoblastoma.MethodsRetrospective review of retinoblastoma cases treated with EB in a single institution. Survival outcomes were analysed using the Kaplan-Meier method.ResultsEleven tumours of 11 eyes were treated with either iodine-125 or ruthenium-106 EB with a mean apical dose of 44 Gy. The tumours were classified as group B in 5 (46%), C in 3 (27%) or D in 3 (27%) eyes, respectively. Mean follow-up time was 75.4 months. EB served as primary treatment in 3 eyes (27%) and secondary treatment in 8 eyes (73%). Final visual acuity was better than 20/200 in 70% of cases. Globe preservation was achieved in 9 (82%) eyes. Local recurrence occurred in 18% of cases at a mean onset of 17.4 months after EB. Two group D tumours that recurred after secondary EB underwent enucleation. Mean onset of radiation retinopathy was 17.4 months following EB. No metastatic or fatal events were recorded. Kaplan-Meier analysis showed recurrence-free survival and ocular survival of 80% and overall survival of 100% at 5 years after EB.ConclusionEB is an effective primary or secondary treatment modality for selected retinoblastoma eyes (groups B and C). Advanced group D tumours may represent a risk factor for local recurrence. Visually significant complications such as radiation retinopathy should be anticipated.


2019 ◽  
Vol 104 (1) ◽  
pp. 17-22 ◽  
Author(s):  
Ido Didi Fabian ◽  
Vishal Shah ◽  
Noa Kapelushnik ◽  
Zishan Naeem ◽  
Zerrin Onadim ◽  
...  

BackgroundEarly diagnosis strategies and advances in retinoblastoma (Rb) management have resulted in nearly 100% survival. More attention should, therefore, be given to quality of life considerations. We aimed to quantify the number of examinations under anaesthesia (EUAs) in a cohort of patients with Rb, as a measure of disease burden.MethodsA retrospective analysis of patients with unilateral Rb that presented to the London Rb service from 2006 to 2013, were treated and had long-term follow-up. Correlations of clinical variables to number of EUAs were investigated.ResultsA total of 107 patients with Rb were included that presented at a mean age of 26.51 ± 22.68 months. The International Intraocular Retinoblastoma Classification (IIRC) was group B in 5 (5%), C in 13 (12%), D in 48 (45%) and E in 41 (38%) of the cases. Primary treatment was intravenous chemotherapy in 36 (34%) and enucleation in 71 (66%) of the cases. Mean number of EUAs was 20.67 ± 6.62, 12.52 ± 6.23 and 11.15 ± 6.91 for combined groups B/C, group D and group E patients (p < 0.001), respectively. On analysis, early age atpresentation and conservative treatments were found to significantly correlate with increased number of EUAs (p < 0.001). Mean follow-up time was 74.42 ± 25.16 months and no metastasis or death were reported.ConclusionFamilies should be counselled regarding the number of EUAs associated with the patient's IIRC group, with B/C eyes undergoing twice the number as compared with D/E eyes. For group D cases, where both enucleation and conservative therapy are valid options, treatment choice has a significant impact on the number of EUAs.


2020 ◽  
Vol 98 (6) ◽  
pp. 371-377
Author(s):  
Won Jae Lee ◽  
Kyung-Rae Cho ◽  
Jung-Won Choi ◽  
Doo-Sik Kong ◽  
Ho Jun Seol ◽  
...  

<b><i>Objectives:</i></b> Surgical resection of nonfunctioning pituitary adenoma (NFPA) invading the cavernous sinus (CS) remains a challenging and significant factor associated with incomplete resection. The residual tumor in CS is usually treated with adjuvant stereotactic radiosurgery (SRS), but there is little information concerning SRS as an initial treatment for CS-invading NFPA. In this study, we investigated the tumor control rate and clinical outcomes of the patients who received primary gamma knife radiosurgery (GKRS) for CS-invading NFPA. <b><i>Methods:</i></b> This was a single-institute retrospective analysis of 11 patients. CS invasion of tumor was categorized using the modified Knosp grading system. The median tumor volume and maximal diameter were 1.6 cm<sup>3</sup> (range 0.4–6.5) and 17.2 mm (range 11.6–23.3), respectively. The median clinical follow-up period was 48.5 months (range 16.4–177.8). The median prescription dose at tumor margin was 15 Gy (range 11–25) and median prescription isodose was 50% (range 45–50). The maximum radiation dose to optic chiasm and optic nerve were 7.2 Gy (range 3.4–9.2) and 7.5 Gy (range 4.5–11.5), respectively. <b><i>Results:</i></b> Tumor control was achieved in all patients. The median tumor volume and maximal diameter at last follow-up were 0.4 cm<sup>3</sup> (range 0.1–2.3) and 11.4 mm (range 4.7–19.5), respectively. The median volume reduction rate was 52% (range 33–88). Six patients showed downgrading of modified Knosp grade after GKRS. No patients developed GKRS-related complications such as hypopituitarism or visual disturbance. <b><i>Conclusions:</i></b> SRS may be an alternative primary treatment option for CS-invading NFPA if there is no urgent and absolute indication for surgery such as optic apparatus compression.


2021 ◽  
pp. 1-7
Author(s):  
Ruyi Yang ◽  
Xinjun Wang ◽  
Ziqiang Xv ◽  
Puxue Zhao ◽  
Junwu Li ◽  
...  

OBJECTIVE Cavernous sinus hemangiomas (CSHs) are rare benign tumors originating from the cavernous sinus. Gamma Knife radiosurgery (GKRS) has been recommended as a primary treatment for small- to medium-sized CSHs. The optimal treatment for giant CSHs is still controversial. In this study, the authors retrospectively reviewed the effectiveness and safety of staged GKRS treatment for giant CSHs. METHODS Twenty-two patients with giant CSH who received staged GKRS treatment in the Gamma Knife Treatment Center of Henan Province during the period from January 1, 2011, to December 31, 2018, were enrolled in this study. Six patients had received microsurgery before GKRS, the other 16 patients were diagnosed according to clinical symptoms and MR images. All of the enrolled patients received 2-stage GKRS, and the mean interval between the two GKRS treatments was 6.5 months (range 6–12 months). For the first GKRS, the median isodose line was 48% (range 45%–50%), the median marginal dose was 13 Gy (range 11.5–14 Gy), and the median coverage of CSHs was 80% (range 70%–88%). For the second GKRS treatment, the median isodose line was 50% (range 45%–55%), the median marginal dose to the CSHs was 10.5 Gy (range 9–12.5 Gy), and the median coverage of the CSHs was 88% (range 80%–94%). RESULTS All of the patients received an outpatient review of an enhanced MR image of the head and a clinical physical check every 6 months after the first GKRS treatment. The mean follow-up duration was 52 months (range 24–84 months). The tumor control rate was 100% 24 months after staged GKRS, and at the last follow-up the mean tumor shrinkage rate was 96.7% (range 90.6%–100%) and the mean residual CSH volume was 2.1 ml (range 0–8.5 ml). Twenty patients suffered central nervous system (CNS) injury symptoms to varying degrees before staged GKRS treatment. Complete symptom recovery was found in 11 (55%) patients, improved symptoms in 5 (25%) patients, and no change in 4 (20%) patients after treatment. Only 1 patient suffered temporary preexisting headache aggravation and 1 patient suffered temporary preexisting diplopia aggravation 1 week after receiving the first GKRS treatment. Subacute or chronic complications were not detected after staged GKRS. CONCLUSIONS Staged GKRS is an effective treatment for giant CSHs. Because of the impressively low incidence of adverse effects, staged GKRS may be considered as a primary treatment for giant CSHs.


2007 ◽  
Vol 23 (6) ◽  
pp. E11 ◽  
Author(s):  
Gregory P. Lekovic ◽  
L. Fernando Gonzalez ◽  
Andrew G. Shetter ◽  
Randall W. Porter ◽  
Kris A. Smith ◽  
...  

Object Increasingly, radiosurgery is used to treat pineal region tumors, either as a primary treatment or as an adjunct to conventional radiation therapy. The authors report their experience with Gamma Knife surgery (GKS) for the treatment of pineal region tumors. Methods The authors retrospectively reviewed the charts of all patients undergoing GKS at their institution between 1997 and 2005. Seventeen patients underwent GKS for nonmetastatic tumors of the pineal region. All patients were treated using Leksell Gamma Plan treatment planning software (versions 4.12::5.34). The mean treatment volume was 7.42 cm3 (range 1.2–32.5 cm3). Prescribed doses ranged from 12 to 18 Gy. All doses were prescribed to the 50% isodose line. Independent neuroradiologists reviewed all follow-up imaging studies for evidence of progression of disease. Results One patient (Case 10) died 6 days after GKS. Mean clinical and imaging follow-up in the remaining 16 cases was 31 months. Local control was established during a mean neuroimaging follow-up period of 31 months (range 1–95) in 16 patients (100%). In 2 of these 16 patients (one with an anaplastic astrocytoma, the other with a primitive neuroectodermal tumor), leptomeningeal and spinal spread of tumor developed despite control of the pineal lesions. There were no new neurological deficits attributable to GKS. Three patients died (including the one who died 6 days after GKS) during the follow-up period. Conclusions Excellent control of pineal region brain tumors can be obtained with GKS when it is used in conjunction with surgery, conventional radiation therapy, or both. Patient survival and quality of life can be optimized through the use of multimodal treatment, including surgery, conventional radiation therapy and/or radiosurgery, and chemotherapy, when applicable.


2010 ◽  
Vol 113 (Special_Supplement) ◽  
pp. 2-8 ◽  
Author(s):  
Hae Yu Kim ◽  
Won Seok Chang ◽  
Dong Joon Kim ◽  
Jae Whan Lee ◽  
Jin Woo Chang ◽  
...  

Object Treatment of arteriovenous malformations (AVMs) is problematic due to many factors, including lesion size, lesion location, unacceptable complications, and negative outcomes. To overcome the limitation imposed by a large nidus volume, neurosurgeons have used Gamma Knife surgery (GKS) in a variety of ways, including combined with other treatment modalities, as volume-staged radiosurgery, and as repeat radiosurgery. We performed repeat radiosurgeries in patients who harbored large AVMs (> 30 cm3) and analyzed the AVM obliteration rates and complications. Methods The authors reviewed the cases of 44 patients at a single institution who underwent GKS between 1992 and 2007 for treatment of an AVM whose nidus was 30 cm3 or larger. The mean age of the patients was 27 years (range 4.5–62.3 years), and the median duration of follow-up was 109.4 months (range 27–202 months). The mean AVM nidus volume was 48.8 cm3 (range 30.3–109.5 cm3), and the mean radiation dose delivered to the margin of the nidus was 13.9 Gy (range 8.4–17.5 Gy). The authors determined complete AVM nidus obliteration based on findings on both MR images and digital subtraction angiograms. When they did not detect complete obliteration after GKS, they performed 1 or more additional GKSs separated by a minimum interval of 3 years. Results The overall obliteration rate following repeat GKS was 34.1%, and the estimated obliteration rate at 120 months was 41.8%. Three patients (6.8%) experienced hemorrhages after GKS, and 2 patients (4.5%) developed cysts. One patient (2.3%) experienced a newly developed seizure following GKS, and another patient (2.3%) was found to have radiation necrosis. Conclusions Even though complete obliteration of the large AVMs after repeat GKS took a long time, the complication rate was quite acceptable. In addition, the estimated obliteration rate at long-term follow-up was respectable. Repeat GKS should be considered as a primary treatment option for symptomatic large AVMs to overcome the limitation imposed on successful obliteration by the large volume of the nidus.


2020 ◽  
pp. 1-7
Author(s):  
Chiman Jeon ◽  
Kyung Rae Cho ◽  
Jung Won Choi ◽  
Doo-Sik Kong ◽  
Ho Jun Seol ◽  
...  

OBJECTIVEThis study was performed to evaluate the role of Gamma Knife radiosurgery (GKRS) as a primary treatment for central neurocytomas (CNs).METHODSThe authors retrospectively assessed the treatment outcomes of patients who had undergone primary treatment with GKRS for CNs in the period between December 2001 and December 2018. The diagnosis of CN was based on findings on neuroimaging studies. The electronic medical records were retrospectively reviewed for additional relevant preoperative data, and clinical follow-up data had been obtained during office evaluations of the treated patients. All radiographic data were reviewed by a dedicated neuroradiologist.RESULTSFourteen patients were treated with GKRS as a primary treatment for CNs in the study period. Seven patients (50.0%) were asymptomatic at initial presentation, and 7 (50.0%) presented with headache. Ten patients (71.4%) were treated with GKRS after the diagnosis of CN based on characteristic MRI findings. Four patients (28.6%) initially underwent either stereotactic or endoscopic biopsy before GKRS. The median tumor volume was 3.9 cm3 (range 0.46–18.1 cm3). The median prescription dose delivered to the tumor margin was 15 Gy (range 5.5–18 Gy). The median maximum dose was 30 Gy (range 11–36 Gy). Two patients were treated with fractionated GKRS, one with a prescription dose of 21 Gy in 3 fractions and another with a dose of 22 Gy in 4 fractions. Control of tumor growth was achieved in all 14 patients. The median volume reduction was 26.4% (range 0%–78.3%). Transient adverse radiation effects were observed in 2 patients but resolved with improvement in symptoms. No recurrences were revealed during the follow-up period, which was a median of 25 months (range 12–89 months).CONCLUSIONSPrimary GKRS for CNs resulted in excellent tumor control rates without recurrences. These results suggest that GKRS may be a viable treatment option for patients with small- to medium-sized or incidental CNs.


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