Results of a Multicenter Prospective Study on the Postoperative Treatment of Unilateral Retinoblastoma After Primary Enucleation

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.

2019 ◽  
Vol 37 (31) ◽  
pp. 2883-2891 ◽  
Author(s):  
Patricia Chévez-Barrios ◽  
Ralph C. Eagle ◽  
Mark Krailo ◽  
Jin Piao ◽  
Daniel M. Albert ◽  
...  

PURPOSE To prospectively determine the prevalence of high-risk histopathologic features (HRFs) in patients with unilateral retinoblastoma who undergo enucleation and to evaluate the role of chemotherapy in preventing recurrences. PATIENTS AND METHODS Children newly diagnosed with enucleated unilateral retinoblastoma were enrolled prospectively. After central histopathology review, patients with specific HRFs received chemotherapy; others were observed. Primary end points were event-free survivals (EFS). RESULTS Of the 331 patients enrolled during 2005 to 2010, 321 eligible patients had central histopathologic review. Discordance between central review and contributing institutions occurred in 23% of patients with HRFs and in 17% of patients without HRFs. Postlaminar optic nerve involvement was present in 53 patients; 42 had massive posterior uveal invasion (≥ 3 mm); 15 had concomitant peripapillary 3 mm or greater choroid and postlaminar optic nerve involvement; and 15 had focal (< 3 mm) choroidal concomitant with lamina or prelamina optic nerve involvement. Two-year EFS for patients with HRFs requiring adjuvant chemotherapy was 0.96 (95% CI, 0.89 to 0.98), and 2-year EFS for patients without HRFs for which observation was indicated was 0.99 (95% CI, 0.96 to 1.0). The 2-year EFS for all patients was 0.98 (95% CI, 0.96 to 0.99). CONCLUSION Adequate handling and interpretation of histopathology of eyes with retinoblastoma is necessary to assign metastatic risk. Concomitant less than 3 mm choroidal and any prelaminar/laminar optic nerve invasion show no recurrence and may warrant no adjuvant chemotherapy. In contrast, concomitant greater than 3 mm peripapillary choroidal invasion and 1.5 mm or greater of postlaminar optic nerve invasion have the poorest outcomes, supporting the need for a more intensive adjuvant chemotherapy regimen for this subgroup. Strict criteria for adjuvant therapy may improve outcomes of children who undergo enucleation at diagnosis and may avoid unnecessary adjuvant chemotherapy for those who are not at risk for recurrence.


1992 ◽  
Vol 16 (4) ◽  
pp. 411-413 ◽  
Author(s):  
Ciro Costagliola ◽  
Mario Rinaldi ◽  
Luigi Cotticelli ◽  
Sandro Sbordone ◽  
Giacomo Nastri

2020 ◽  
pp. 1-4
Author(s):  
Layla Maris Areas ◽  
Fabio Lavinsky ◽  
Daniel Lavinsky ◽  
Rodrigo Leivas Lindenmeyer ◽  
Helena Messinger Pakter ◽  
...  

2019 ◽  
Vol 39 (3) ◽  
pp. 391-393
Author(s):  
Abdullah S. Alamri ◽  
Daniah A. Alshowaeir ◽  
Ali A. AlFaiz ◽  
Fatimah H. Al Mousawi ◽  
Adel A. Mahmoud ◽  
...  

2010 ◽  
Vol 51 (12) ◽  
pp. 1676 ◽  
Author(s):  
In Ki Park ◽  
Sang Woong Moon ◽  
Ji Sang Han ◽  
Jae Ho Shin

2011 ◽  
Vol 29 (35) ◽  
pp. 4677-4681 ◽  
Author(s):  
Jorge Aparicio ◽  
Pablo Maroto ◽  
Xavier García del Muro ◽  
Josep Gumà ◽  
Alfonso Sánchez-Muñoz ◽  
...  

Purpose To confirm the efficacy of a risk-adapted treatment approach for patients with clinical stage I seminoma. The aim was to reduce both the risk of relapse and the proportion of patients receiving adjuvant chemotherapy while maintaining a high cure rate. Patients and Methods From 2004 to 2008, 227 patients were included after orchiectomy in a multicenter study. Eighty-four patients (37%) presented no local risk factors, 44 patients (19%) had tumors larger than 4 cm, 25 patients (11%) had rete testis involvement, and 74 patients (33%) had both criteria. Only the latter group received two courses of adjuvant carboplatin, whereas the rest were managed by surveillance. Results After a median follow-up time of 34 months, 16 relapses (7%) have been documented (15 [9.8%] among patients on surveillance and one [1.4%] among those treated with carboplatin). All relapses occurred in retroperitoneal lymph nodes, except for one case in pelvic nodes. Median node size was 25 mm, and median time to recurrence was 14 months. All patients were rendered disease-free with chemotherapy. The actuarial 3-year disease-free survival rate was 88.1% (95% CI, 82.3% to 93.9%) for patients on surveillance and 98.0% (95% CI, 94.0% to 100%) for those treated with adjuvant chemotherapy. Overall 3-year survival was 100%. Conclusion With the limitations of the short follow-up duration, we confirm that a risk-adapted approach is effective for stage I seminoma. Adjuvant carboplatin seems adequate treatment for patients with 2 risk criteria, as is active surveillance for those with 0 to one risk factors. More reliable predictive factors are needed to improve the applicability of this model.


2012 ◽  
Vol 30 (15_suppl) ◽  
pp. e14658-e14658
Author(s):  
Xuezhong Yang ◽  
Benjamin Weinberg ◽  
Jimmy J. Hwang ◽  
Christina Sing-Ying Wu ◽  
Madeeha Akram ◽  
...  

e14658 Background: The 5-year survival of PAC with surgery alone is below 10%, and with adjuvant chemotherapy increases to about 20%. The original GITSG adjuvant study demonstrating a survival benefit compared to surgery could be attributed to the use of 2-years of weekly IV bolus 5FU, and not only chemoradiation. In theory, the prolonged exposure to therapy could maintain pressure on dormant cancer cells that may remain in G0 arrest, by attacking them as they infrequently enter G1/S phase. To evaluate this hypothesis, we retrospectively evaluated our pts who were treated with or without maintenance Cape. Methods: Pts in the Georgetown/Lombardi Cancer Center EMR since Oct 2007 were sought for PAC that was resected with curative intent, received standard adjuvant chemotherapy with or without chemoradiation. The study group received maintenance cape for at least 2 months, and the control group was monitored until disease recurrence. Only pts with complete follow-up survival data were analyzed. Results: 20 pts met the criteria as study group, and 58 pts as the control group. In the study group, cape was usually given 1000mg orally twice a day, Monday through Friday following adjuvant therapy, for an indefinite period, up to 2 years. Pts received cape for median duration of 12.5 months (2 to 24 months), and the median follow-up duration was 33 months (16 to 78 months). The median overall survival (OS) for the study group was 48 months. The 2 year OS was 94%, and 5 year OS was 40%. The median recurrence free survival (RFS) was 39 months. The 2 year RFS was 67%, and the 5 year RFS was 25%. Common toxicities were mild hand-and-foot syndrome and fatigue. 4 pts discontinued cape due to toxicities: febrile neutropenia, severe fatigue, weight loss and diarrhea. The control group was of comparable staging, and the median OS was 22 months, 5 year OS rate was 16%, median RFS was 13 months, 2 year RFS was 19%. Conclusions: In this single institute retrospective controlled cohort study, Cape maintenance therapy following adjuvant therapy in resected PAC is associated with a significantly (p<0.05) higher OS and PFS compared to the control group. This approach should be studied in a RCT.


2020 ◽  
Vol 38 (4_suppl) ◽  
pp. 332-332
Author(s):  
Meena Sadaps ◽  
Neal Mehta ◽  
Michael J. McNamara ◽  
Alok A. Khorana ◽  
Amit Bhatt

332 Background: Adjuvant therapy after endoscopic resection (ER) of T1 EAC in non-surgical candidates is largely based on the risk of LNM. Risk factors for LNM in T1 EAC are not clearly defined. Our aim is to evaluate risk factors for LNM in T1 EAC patients following esophagectomy or ER with ≥ 5 years of follow-up. Methods: This is a retrospective analysis at a large tertiary referral center. Our pathology database identified patients who underwent esophagectomy or ER with ≥ 5 years follow-up, with histologically proven T1 EAC from 2010-2017. Patients were excluded if they (a) received chemoradiation prior to esophagectomy or before/after ER (b) had any other primary cancer treated within the previous 5 years. Specimens were reviewed by an expert GI pathologist for accuracy. Results: Of 80 patients [85% males], 61 (76%) underwent esophagectomy and 19 (24%) underwent ER. Twelve (15%) developed LNM per study criteria. Tumor size was significantly (p-value 0.014) associated with risk of LNM (Table). No other factors including lymphovascular invasion, differentiation on pathology, macroscopic appearance, infiltration growth pattern, or tumor distance from the gastroesophageal junction were significant risk factors for LNM. Conclusions: In T1 EAC, tumor size appears to be a significant risk factor for LNM at five years following surgical or endoscopic resection. Adjuvant therapy should be considered in patients with larger tumor size. [Table: see text]


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