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The aim was to evaluate the incidence, clinical course, and outcome of adenoviral infection (AdVI) in pediatric patients diagnosed and treated due to cancer and in pediatric recipients of hematopoietic stem cell. Over a 72‐month period, all‐in 5599 children with cancer: 2441 patients with hematological malignancy (HM) and 3158 with solid tumors (ST), and 971 patients after transplantation: 741 after allogeneic (allo‐HSCT) and 230 after autologous (auto‐HSCT) were enrolled into the study. Among cancer patients, 67 episodes of AdVI appeared in 63 (1.1%) children, including 45 (1.8%) with HM and 18 (0.6%; P <.001) with ST. Within transplanted patients, AdVIs were responsible for 88 episodes in 81 (8.3%) children (P <.001), including 78 (10.5%) patients after allo‐HSCT and 3 (1.3%) after auto‐HSCT. Time to develop AdVI was short, especially after allo‐HSCT. The most common clinical manifestation in cancer patients was enteritis diagnosed in 63 (94.0%) cases, while among HSCT recipient asymptomatic adenoviremia was found in 36 (40.9%) cases and the most common clinical manifestation was urinary tract infection. Cancer patients with disseminated disease, as well as HSCT recipients with either asymptomatic viremia or disseminated disease, received antiviral treatment. The most commonly used first‐line therapy was cidofovir. None of the cancer patients died due to AdVI, while within HSCT recipients three patients developed disseminated adenoviral disease and died despite antiviral treatment. In cancer patients, AdVIs are rare and associated with very good prognosis even without specific treatment. However, in allo‐HSCT recipients, disseminated disease with fatal outcome is more likely to occur. Highlights: Low incidence of HAdV causing less severe infections not requiring antiviral therapy but with satisfactory outcome within cancer patients.In allo‐HSCT recipients disseminated disease requiring antiviral drugs and at risk of fatal outcome is more likely to occur.Monitoring for HAdV in all patients, and especially in those who underwent AdVI prior to HSCT or had MD or MMUD‐HSCT, at early posttransplantation period should be recommended. [ABSTRACT FROM AUTHOR]

Angiosarcoma is a rare, highly malignant vascular neoplasm with little data available on its clinical course and management in children. Ten children with angiosarcoma (M/F: 6/4; aged 2, 3-16 years) registered in Polish Paediatric Rare Tumours and Soft Tissue Sarcomas Studies between 1992 and 2006. Primary tumour exceeded 5 cm in seven patients and affected mainly deep tissues (heart-2, head/neck, bladder, brain, liver and upper limb - one patient each). Four patients had regional and two metastatic diseases (lungs and bones). Three patients were initially misdiagnosed as haemangioma. Complete primary excision was unfeasible even in local stages. All patients received supplementing chemotherapy with no response in four. Radiotherapy was given to five children, including three after relapse. Three of five secondary tumour resections proved complete. Seven patients experienced relapses (mainly metastatic) and two continuous progression. Relapsed patients received chemotherapy ± radiotherapy and surgery (three). Nine patients died of disease (overall survival 6-66 months), and one child after mutilating secondary resection is alive. Angiosarcoma in children is highly aggressive with an extremely poor prognosis. Complete primary excision is unfeasible, even in seemingly local stages. The response to chemotherapy is poor and the large number of metastatic recurrences suggests a need for systemic therapy modifications. [ABSTRACT FROM AUTHOR]

Kazanowska B, Jelen M, Reich A, Tarnawski W&Chybicka A(2004)Histopathology45,352–359The role of nuclear morphometry in prediction of prognosis for rhabdomyosarcoma in children: To determine whether nuclear morphometry can be used in pretreatment diagnostic procedures to guide the therapy of childhood rhabdomyosarcoma (RMS).: Biopsy specimens obtained from 108 patients with rhabdomyosarcoma aged between 1 and 217 months treated in 12 paediatric oncology departments in Poland were evaluated. There were 65 (60.2%) specimens of embryonal rhabdomyosarcoma (RME), 32 (29.6%) of alveolar RMS (RMA) and 11 (10.2%) cases of undifferentiated RMS (RMU). The clinical data from all analysed patients were evaluated. Nuclear morphometry was performed semiautomatically on haematoxylin–eosin-stained sections using the MultiScan v.8.08 Computer Scanning System and an Olympus BX 50 microscope with a× 40 magnification lens.: In the RMA subtype cells with spindle-shape nuclei were less common (P = 0.013) and cell nuclei were generally more round in comparison with RME (P = 0.033). The clinical outcome was better if the nuclei seen in biopsies of RMS were more spindle-shaped (event-free survival 0.51 and 0.23, respectively;P = 0.04) or more cells with spindle-shaped nuclei were observed (event-free survival 0.5 and 0.28,P =0.035). RME patients with small nuclei had a better outcome then patients with large nuclei (P = 0.014). In the RMA/RMU group, patients with small tumour cell nuclei had a worse prognosis than patients with larg tumour cell nuclei (P = 0.046).: Nuclear morphometry is a useful tool in the assessment of children with RMS. Additionally, certain morphometric parameters could be easily applied in a selection of patients with good prognosis. [ABSTRACT FROM AUTHOR]

The strongest predictors of outcome in pediatric B-cell precursor acute lymphoblastic leukemia (BCP-ALL) are minimal residual disease (MRD) and specific molecular abnormalities. One unfavorable prognostic factor is the presence of IKZF1 gene aberrations, particularly when co-occurring with high MRD level at the end of induction treatment. The present study determines the predictive value of a recently-defined IKZF1-plus (IKZF1 plus ) microdeletion profile in 373 children with BCP-ALL treated according to the ALL-intercontinental Berlin-Frankfurt-Munster protocol 2009 protocol. IKZF1-wild type (IKZF1 wt ) patients demonstrated lower leukemic burden parameters than those carrying IKZF1 deletion (IKZF1 del [n = 26, 7.0%]) or IKZF1 plus pattern (n = 34, 9.1%): (i) median blast percentage at diagnosis (78.0% vs. 86.9% vs. 86.0%; p = 0.021); (ii) median MRD level at day 15 of induction protocol (0.3% vs. 2.1% vs. 0.8%; p = 0.011); (iii) poor steroid response (7.6% vs. 26.5% vs. 12.5%; p = 0.010). Minimal residual disease level at day 33 (MRD33) exceeding 10 -4 was more frequently observed in both the IKZF1 del and IKZF1 plus subgroups than in IKZF1 wt patients (n = 9 [36.0%] vs. n = 13 [41.9%] vs. n = 70 [24.0%], p = 0.051). IKZF1 plus individuals showed a tendency for a lower MRD reduction between day 15 and 33 compared to IKZF1 del patients (p = 0.124). IKZF1 del and IKZF1 plus patients showed decreased relapse-free survival (HR [95%CI] for IKZF1 wt as reference = 2.72 [1.21-6.11] and 2.00 [0.87-4.49], respectively, p = 0.023). Both genetic markers including IKZF1 del and IKZF1 plus microdeletion profile provide additional predictive value of treatment outcome in childhood BCP-ALL and may contribute to more efficient patient stratification; the same is true in MRD guided protocols, which are based on flow cytometric measurements on day 15 of induction protocol., (© 2022 John Wiley & Sons Ltd.)

Anti-cancer treatment in children can deteriorate gonadal function and affect future fertility. We analyzed the hormonal markers of gonadal function in adolescent leukemia survivors, treated in childhood with different levels of aggressiveness. We analyzed hormone levels in 69 adolescents and young adults, leukemia survivors stratified into standard (SR), intermediate (IR), and high (HR) risk groups, and in 80 healthy controls (38 men) at a similar age. We assessed follicular stimulating hormone (FSH), luteinizing hormone (LH), and inhibin B in the whole group, testosterone in males, and E2 and anti-Müllerian hormone (AMH) in females. Males classified into HR group presented, in comparison to control, higher levels of FSH, LH, lower inhibin B, and normal testosterone, whereas in SR and IR group, the hormonal values were comparable to the control. In females, in all risk groups, the levels of FSH, LH, E2, and inhibin B were comparable with the control, but the mean AMH levels were slightly lowered. We did not observe the effect of prophylactic cranial irradiation (12 or 18 Gy) or the time of treatment (before vs. during puberty) on hormone levels. In females, a positive correlation was found between the time interval after the end of treatment and AMH levels. Male leukemia survivors having undergone more intensive chemotherapy show the symptoms of disturbed spermatogenesis and need to be followed-up in the future. Women, irrespective of the risk group, can develop the signs of preterm ovarian insufficiency. They should be informed about the impact of the treatment on gonadal function., (©2019 John Wiley & Sons, Ltd.)