Background: Pediatric B-Lymphoblastic lymphoma(pB-LBL) is a rare entity, and appropriate treatment for pB-LBL is not well defined. While intensive Acute Lymphoblastic leukemia(ALL) type regimens achieve long term event free survival of 90% across western co-operative group trials, published data from Asian studies on long term outcomes in pB-LBL are scarce. We evaluated the outcomes and prognostic factors of pediatric B-LBL patients treated at our center. Methods: We retrospectively analyzed the data of pediatric B-LBL patients treated between January 2010 and December 2017 on a uniform protocol(modified BFM 90). Patients were evaluated for early response post-induction and monitored for toxicity and long term outcomes. Kaplan-Meier method was used to estimate the event free survival(EFS) and overall survival(OS). Cox regression models were performed to identify prognostic factors. Results: Of 21 patients who received treatment on the modified BFM 90 protocol, 17(81%) were alive in remission, 3(14%) had relapse, and 1(4%) had treatment-related mortality(TRM) while in remission. Two of 3 relapsed patients subsequently expired. With a median follow-up of 66 months(range 6–114), 5-year Event free survival(EFS) and overall survival(OS) were 80%(95% CI:71–89%) and 91% (95% CI:85–97%), respectively. While delayed presentation (≥3 months) had inferior EFS(p-0.030), patients with elevated baseline Lactate Dehydrogenase(LDH) had a worse OS(p-0.037). Age, gender, site of origin, stage, and post-induction response had no bearing on outcome. Conclusions: Outcomes of pB-LBL patients treated on modified BFM 90 protocol are excellent. Higher disease burden manifested by elevated baseline LDH and delayed presentation(≥3 months) portend poorer survival.
1 Background Persisting residual mass at treatment completion are known in rhabdomyosarcoma(RMS) who have been treated with definitive radiotherapy to the primary site, but their prognostic significance is uncertain. Tumour response as assessed by anatomic imaging is not prognostic and there are only limited studies based on FDG-PET response. We report the prognostic significance of persistent FDG avidity in residual masses, assessed 3-months post completion of radiotherapy, in paediatric RMS who have undergone definitive RT as primary local therapy. 2 Materials and Methods Children≤15 years with Group 3 or 4 RMS treated on a uniform chemotherapy protocol, who received definitive radiotherapy for local control from June 2013-December 2018, and had FDG-PET CT at 3-months post radiotherapy were retrospectively analysed for outcomes. 3 Results Sixty-three children formed the study cohort, (55 Group3 and 8 Group4) FDG-PET CT scan done 3-months post-radiotherapy showed FDG-avid residual mass in 11 patients(17.5%), morphologic only (FDG negative) residual mass in 24 patients(38.1%) and no residual in 28 patients(44.4%). At a median follow-up of 41months (range,10-83months), 3-year Event Free Survival of patients with FDG-avid residual are 45.5% (95%CI:23.8%-86.8%) and for those with morphologic only or no residual are 71.4% (95%CI:59.6%-85.5%). Presence of FDG-avid residual on PET-CT scan 3-months post definitive RT [HR-2.92(95%CI:1.13-7.57),p=0.028] and regional lymph node involvement [HR-3.14(95%CI:1.26-7.78),p=0.014] affected outcomes, which retained significance on multivariate analysis too. 4 Conclusions Persistent metabolic activity in residual disease at the end of therapy in RMS may portend poorer prognosis, and help identify patients who would benefit from alternative treatment strategy.
Background: The purpose of this single-centre study was to analyse the outcomes of extracranial germ cell tumors (GCTs) in children treated on a multi-modality regimen at a single-centre. Methods: Retrospective study of children (<18 years) with a histopathologically confirmed diagnosis of extracranial GCT over a period of 10 years (January’09-December’18) treated on a uniform institution-based protocol. All completely excised teratomas and stage I gonadal tumors received no further therapy (low risk); Stage IV Ovarian, Stage III-IV extragonadal GCTs received 6 cycles of chemotherapy (high risk) and the remaining received 4 cycles of chemotherapy (intermediate risk). Results: A total of 336 kids were treated of which the analysable cohort comprised of 297with a boy-girl ratio of 1.72:1 and median age of 4 years. Gonadal GCTs(n-180) were commoner than extragonadal GCTs(n-117) with ovary as primary site in 128 children(43%) and sacrococcygeal site being the commonest extragonadal location(n-41;14%). LR, IR and HR disease were noted in 60(20.2%) patients, 125(42%)patients and 112(37.8%)patients respectively. Forty-one patients relapsed and 43 children expired (disease related-33; toxic deaths-9; unknown-1). The 5-year EFS/OS was 79.3%/84.4% respectively with gonadal site, low-risk and non-metastatic disease associated with statistically better EFS (median follow-up:52.1±37.3 months). Conclusion(s): Both cisplatin and carboplatin based regimens had comparable outcomes. The low and intermediate GCTs had an excellent outcome, thus warranting a gradual shift in the approach to these tumors by reducing therapy and decreasing late effects of therapy. In high risk GCTs however, intensifying therapies to improve outcomes must be balanced against the risk of cumulative toxicity.