Today, treatment results for acute lymphoblastic leukemia (ALL) look encouraging, yet 10–15% patients still end up relapsing. The success of relapse treatment is directly dependent on whether or not a tumor clone has been completely eradicated before hematopoietic stem cell transplantation (HSCT). Immunotherapy made it possible to achieve minimal residual disease (MRD) – negative remission even in refractory patients. One example of such immunotherapeutic agents is inotuzumab ozogamicin (InO), an anti-CD22 monoclonal antibody conjugated to the cytotoxic agent calicheamicin. We included 17 patients under the age of 18 with relapsed or refractory precursor B-cell ALL (pre-B ALL) who had been treated with InO at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of Russia from 01.10.2016 to 01.09.2022. The study was approved by the Independent Ethics Committee and the Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. The efficacy of the therapy was assessed based on the patients’ morphological response, MRD negativity and overall survival. Treatment toxicity was assessed according to CTCAE 5.0 (Common Terminology Criteria for Adverse Events). Statistical analysis was performed using the XLSTAT 2016 software. The majority of the patients (75%) responded to the therapy. MRD negativity was achieved in 41.2% of the study patients. The one-year overall survival rate was 40.3% (95% confidence interval 14.8–65.7). The treatment was well tolerated but 33% of the patients treated with standard-dose InO and subsequent HSCT developed veno-occlusive disease/sinusoidal obstruction syndrome. In our study, we demonstrated the high efficacy of InO both when used as a rescue therapy in patients with relapsed/refractory pre-B ALL and as a bridging therapy in patients before HSCT.
Introduction. Standard eye enucleation (EE) may not always guarantee a sufficient length of resection of the optic nerve (ON) so that the tumor cells do not spread along the optic nerve at the intersection line. Surgical access and the scope of surgical intervention are determined by the spread and localization of the tumor, as well as the qualification of the operating team.Purpose — to evaluate the role of extended surgical interventions in the spread of extraocular tumors on the ON.Materials and methods. The study included 9 patients with retinoblastoma (RB) and macroinvasion of the ON during primary magnetic resonance imaging (MRI) and/or micromorphological invasion of the ON resection line after EE. 4 of the 9 patients were treated with primary/secondary EE, induction chemotherapy (CT) and high-dose CT (HDCT), radiation therapy (RT). 5 out of 9 patients underwent secondary extended surgical interventions: exenteration of the orbit (n = 1), osteoplastic lateral orbitotomy with precanal resection of the ON (n = 2) in combination with EE in one case, subfrontal craniotomy (n = 1) and orbitozygomatic craniotomy (n = 1) with prechiasmal resection of the ON and EE with adjuvant RT (excluding the latter case) and CT, without HDCT.Results. It should be noted that the overall survival (OS) of 5 patients with complete microscopic resection (R0) after extended secondary operations was 75 ± 0.217 % with an average follow-up period of 77.25 ± 18.8 months, while in 4 patients with R1 (n = 4) without secondary extended operations with HDCT reached only 50 ± 0.25 % with an average follow-up period of 57 ± 24.8 months.Conclusion. MRI is mandatory for the primary diagnosis of RB, especially when there is a risk of the tumor spreading through the ON. Secondary surgery with R0-resection has a positive effect on survival. The need for adjuvant RT and CT after surgery should be discussed.
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