Can CD34+CD38− lymphoblasts, as likely leukemia stem cells, be a prognostic factor in B-cell precursor acute lymphoblastic leukemia in children?

BackgroundCD34+CD38− lymphoblasts as likely leukemia stem cells (LSCs) may be responsible for a worse response to treatment and may be a risk factor for recurrence in B-cell precursor acute lymphoblastic leukemia (BCP-ALL).ObjectiveThe study objective was to assess the prognostic role of CD34+CD38− lymphoblasts in bone marrow on the day of BCP-ALL diagnosis.Methods115 patients with BCP-ALL, the median age of 4.5 years (range 1.5–17.9 years), gender: female 63 (54.8%) with BCP-ALL were enrolled; Group I (n = 90)—patients with CD34+CD38+ antigens and Group II (n = 20)—patients with CD34+CD38− antigens on the lymphoblast surface.ResultsA worse response on Days 8, 15, and 33 of therapy and at the end of treatment in Group II (CD34+CD38−) was more often observed but these differences were not statistically significant. A significantly higher incidence of BCP-ALL recurrence was in Group II.Conclusions1.In BCP-ALL in children, the presence of CD34+CD38− lymphoblasts at the diagnosis does not affect the first remission.2.In BCP-ALL in children, the presence of CD34+CD38− lymphoblasts at the diagnosis may be considered an unfavorable prognostic factor for disease recurrence.3.It is necessary to further search for prognostic factors in BCP-ALL in children

Relapses Children’s Acute Lymphoblastic Leukemia, Single Center Experience

The prognosis in children and adolescents with relapsed ALL, despite intensive therapy, including hematopoietic stem cell transplantation, is still challenging. This study aims to analyze the incidence of relapsed ALL and survival rates in correlation to the risk factors. Materials and methods: 125 pediatric patients with ALL diagnosed in our department between 2000-2018; age 1–18 years old (median 6.4); female 53.6% vs. male 46.4%. Results: 19 pts (15.2%) were diagnosed with a relapse. Three pts (15.8%) had been diagnosed with very early relapses (2/3 T-ALL), 12 pts (63.1%) as an early relapse, and 4 pts (21.1%) as a late relapse. Bone marrow was the most frequent relapses localization. The five-year survival has been achieved by six patients (31.6%). A significant difference was found in regard to the five-year overall survival and relapse type (p < 0.05). The group with very early relapses (3/3; 100%) has not reached the five-year survival. Conclusions: 1. The main prognostic factor in children’s ALL relapses is still the time of the onset of the relapse. 2. The T lineage acute lymphoblastic leukemia is a worse prognostic factor. 3. The analysis of the above relapse risk factors alongside cytogenethic markers and flow cytometry or polymerase chain reaction minimal residual disease is very important for first-line chemotherapy improvement and a more personalized choice of therapy for ALL patients

Clear Cell Renal Cell Carcinoma, Diagnostic and Therapeutic Difficulties, Case Report and Literature Review

Nephroblastoma is the most common kidney tumour in children, constitutes about 85% of cases. Although renal cell carcinoma (RCC) is the second-most common kidney malignancy in children, it constitutes only about 2&ndash;6% of all cases. Currently, the basis of children&rsquo;s RCC treatment is Umbrella Protocol of SIOP-RTSG, but, due to the rare diagnosis of this neoplasm in children, in difficult cases, treatment is based on the experience in adult patients with RCC. Nephrectomy improves prognosis and is usually performed at the first step of treatment. Acute kidney injury secondary to urolithiasis in a patient after nephrectomy due to RCC is a unique, very serious complication. Study design: We present a case of a 10-year-old boy with metastatic clear cell renal cell carcinoma (ccRCC) of the right kidney and an acute renal failure of the left kidney secondary to uric acid nephrolithiasis. Partial regression of the spread of ccRCC after 12.5-month treatment with sunitinib, followed by progression being observed and satisfactory effects and tolerance of nivolumab were observed later. Comorbidity of acute kidney injury during nephrolithiasis and ccRCC after nephrectomy in children is unique. Drugs used in the treatment clear cell carcinoma in adults (sunitinib and nivolumab), are also used in children with ccRCC

Polish Multi-Institutional Study of Children with Ependymoma—Clinical Practice Outcomes in the Light of Prospective Trials

We performed a multi-institutional analysis of 74 children with ependymoma to evaluate to what extent the clinical outcome of prospective trials could be reproduced in routine practice. The evaluation of factors that correlated with outcome was performed with a log rank test and a Cox proportional-hazard model. Survival was estimated with the Kaplan–Meier method. The majority of patients had brain tumours (89%). All had surgery as primary treatment, with adjuvant radiotherapy (RTH) and chemotherapy (CTH) applied in 78% and 57%, respectively. Median follow-up was 80 months and 18 patients died. Five- and 10-year overall survival (OS) was 83% and 73%. Progression was observed in 32 patients, with local recurrence in 28 cases. The presence of metastases was a negative prognostic factor for OS. Five- and 10-year progression-free survival (PFS) was 55% and 40%, respectively. The best outcome in patients with non-disseminated brain tumours was observed when surgery was followed by RTH (+/−CTH afterwards; p = 0.0001). Children under 3 years old who received RTH in primary therapy had better PFS (p = 0.010). The best outcome of children with ependymoma is observed in patients who received radical surgery followed by RTH, and irradiation should not be omitted in younger patients. The role of CTH remains debatable