A multicentre, prospective, risk and response adapted treatment for newly diagnosed paediatric classical Hodgkin Lymphoma was carried out to avoid radiotherapy (RT) when complete remission (CR) was achieved after chemotherapy. Three risk groups were initially defined. Chemotherapy combined ABVD and ESHAP regimes. CR was defined by Deauville Score <1-2 or >80% CT volume reduction. Patients in CR after completing chemotherapy did not receive RT, those in partial remission received involved site RT (30 Gy). Refractory disease was considered a trial failure. From October 2017 to November 2024, 109 assessable patients were recruited, M/F = 1.8, median age 11.9 (r = 3-16.8) years. Low risk = 18%, Intermediate risk = 30%, High risk = 51%. CR after chemotherapy was obtained in 94 patients (86.2%). Radiotherapy was delivered in 19.3% of patients, including cases in partial remission, relapse, and refractory disease. With a median follow up of 58 months, at 5 years, EFSp was 88.5% (SE = 3%) and OS, 100%.

BACKGROUND:Racotumomab is a murine anti-idiotype monoclonal antibody targeting N-glycolylated (NeuGc) glycoconjugates like NeuGcGM3 ganglioside, expressed in nearly 85% of neuroblastoma tumors. AIMS:To report the immune response, safety, and clinical outcomes in subjects with high-risk neuroblastoma (HR-NB) treated with racotumomab in a Phase 2 study (NCT02998983). METHODS:Subjects were assigned to two strata. Stratum 1 included those in first or second very good partial remission (VGPR) or complete remission (CR) after first- or second-line conventional treatments for HR-NB. Stratum 2 included those in partial remission (PR) or stable disease (SD) after first- or second-line treatment. Racotumomab was administered as five biweekly doses followed by 10 monthly doses (1 year total). Serum anti-vaccine and anti-NeuGc glycoconjugates antibody titers were quantified by enzyme-linked immunosorbent assay, and antibody-dependent cell-mediated cytotoxicity (ADCC) was assessed using a luminescence-based assay. Adverse events were graded per CTCAE v4.0. RESULTS:Thirty-nine patients met eligibility criteria, with 35 (89.7%) having Stage M disease at diagnosis. Twelve (30.8%) had N-myc amplification, 27 (69.2%) prior ASCT, and two (5.1%) prior anti-GD2 immunotherapy. Twenty-six were assigned to Stratum 1 (median age: 5.5 years) and 13 to Stratum 2 (median age: 6.3 years). Immune responses included human anti-mouse antibodies in 37 (97%), NeuGc glycoconjugate-specific IgM/IgG in 16 (42%), and ADCC in eight (21%) at 3 and/or 6 months. No serious treatment-related adverse events occurred. In patients treated in VGPR/CR (Stratum 1), the 3-year progression-free survival was 0.48 (95% confidence interval [CI]: 0.28-0.66). Subjects with IgM responses had improved progression-free survival (p = 0.046). CONCLUSIONS:Racotumomab elicited immune responses without serious related adverse events in HR-NB patients.

Les gliomes de bas grade representent la premiere cause de tumeurs cerebrales chez l'enfant. L'exerese chirurgicale complete doit toujours etre envisagee, mais est parfois irrealisable en raison de la localisation tumorale ou de l'infiltration du cerveau sain. Le role des traitements complementaires, particulierement important a envisager en fonction du comportement tumoral a long terme et des sequelles iatrogenes, n'est pas encore clairement etabli. Les auteurs ont realise une etude retrospective unicentrique sur 51 enfants, traites de 1986 a 1995, afin de correler leur evolution aux parametres cliniques, histologiques et therapeutiques. L'âge median etait de 7 ans. Les localisations etaient sustentorielles, infra-tentotrielles, et spinales dans respectivement 63, 31, et 6 % des cas. Le diagnostic histologique correspondait essentiellement a un astrocytome pilocytique (53%). L'exerese chirurgicale a ete consideree comme complete ou subtotale, partielle, ou reduite a une biopsie dans respectivement 37, 30, et 37 % des cas. Aucun des 17 enfants dont l'exerese tumorale etait complete ou subtotale, n'a rechute. Une rechute est survenue chez 3 enfants parmi les 14 dont l'exerese etait partielle, et 2 sont decedes. Parmi les 15 enfants n'ayant beneficie que d'une biopsie, 5 ont rechute malgre un traitement associant le plus souvent la radiotherapie et la chimiotherapie. Le taux de reponse a la chimiotherapie a ete de 39 %. Les taux de survie et de survie sans progression sont respectivement de 86 et de 82 %, avec un recul median de 84 mois. En analyse univariee, seule l'exerese chirurgicale complete apparait comme un facteur pronostique. Le role de la radiotherapie initiale reste a demontrer. La chimiotherapie seule a permis de differer la radiotherapie chez 2 enfants sur 4. En cas d'exerese partielle, le taux de survie, dans notre serie, n'est pas different de celui des series ou le traitement complementaire est realise au moment de la rechute. Notre etude confirme (i) le role de l'exerese chirurgicale complete, qui doit etre systematiquement envisagee, et qui permet d'eviter tout traitement complementaire; (ii) l'efficacite de la chimiotherapie qui permet d'obtenir des remissions completes et de differer la radiotherapie.

Low-grade gliomas are the most common brain tumors in children. Total surgical excision should always be sought but may be unfeasible if the tumor is in a critical site or has ill-defined margins. The role of adjunctive treatments needs to be clarified based on long-term tumoral behavior and on adverse treatment effects. Fifty-one cases of pediatric low-grade glioma treated in a single center between 1986 and 1995 were reviewed with the goal of looking for correlations between outcomes and clinical, histological, and therapeutic parameters. Median age was seven years, Supratentorial, infratentorial, and spinal tumors contributed 63%, 31%, and 6% of cases, respectively. Juvenile pilocytic astrocytoma was the most common histologic type (53%). Surgical excision was considered total or subtotal, partial, or limited to a simple biopsy in 37%, 30%, and 37% of cases, respectively. There were no recurrences in the 17 patients who had total or subtotal excision. Of the 14 patients who had partial excision, three developed a recurrence and two died. Five of the 15 biopsied patients had a recurrence despite nonsurgical treatment that usually combined chemotherapy and radiation therapy. The chemotherapy response rate was 39%. Survival and progression-free survival rates were 86% and 82%, respectively, after a median follow-up of 84 months. Total surgical excision was the only significant prognostic factor in the univariate analysis. The role of initial radiation therapy remains to be determined. Chemotherapy allowed to postpone radiation therapy in two of four patients. Survival rates in patients who had partial excision were similar to those reported in studies of patients who received adjunctive therapy only at the time of the recurrence. Our data confirm the importance of total surgical excision, which should be the goal in every case and obviates the need for adjunctive therapy. They also demonstrate that chemotherapy can provide a complete remission and/or allow to postpone radiation therapy.