Ultrasonografia dopplerowska w diagnostyce nowotworów jąder u dzieci

Malignant testicular tumors account for approximately 1-2% of all pediatric malignancies. We evaluated the usefulness of Color Doppler and contrast-enhanced Color Doppler ultrasonography in the detection and diagnostic differentiation of testicular tumors in children. Thirty boys were treated for testicular tumors. They ranged in age from 4 months to 16 years (mean: 4.6 years). All were staged according to the Pediatric Oncology Group/ Children Cancer Study Group staging system. Diagnostic assessment included in all cases conventional ultrasonography (US), Color Doppler (CD), and Power Doppler (PD) before and after the administration of contrast agent. Germinal tumors and tumor arising from the endodermal sinus (yolk sac tumors) were the most common. Eight patients had stage III, four stage IV, and eighteen stages I and II. Histologically, 13 patients had pure endodermal sinus tumor (EST), 4 had EST with embryonal carcinoma, 5 had embryonal carcinoma alone, 8 had teratoma, and 1 had non-germinal tumor. In the group of EST patients, the largest number of lesions were enhanced by 2-3 points, embryonal carcinoma by 4-5 points, and teratoma immatrum by over 4 points. EST and embryonal carcinoma presented peak enhancement in a time under 120 sec., and teratomas in a time under 180 sec. Teratomas and embryonal carcinoma had the longest enhancement times. Three patients with teratoma in stage 1 had only the tumor removed, sparing the testis. Twenty patients had unilateral orchiectomy and three had lymphadenectomy. All children received chemotherapy depending on the character of the tumors. We have observed much better prognosis in children with testicular tumors after surgery of the tumor and subsequent intensive chemotherapy. US examination with unenhanced and contrastenhanced Color Doppler is important in achieving the correct diagnosis and better treatment results. US contrast agent (Levovist) is very helpful in precise visualization of pathological vessels inside the tumor mass, and can be considered useful in the initial differentiation between testicular tumor types

Tumor expression of survivin, p53, cyclin D1, osteopontin and fibronectin in predicting the response to neo-adjuvant chemotherapy in children with advanced malignant peripheral nerve sheath tumor

Purpose Selected cell-cycle regulators and extracellular matrix proteins were found to play roles in malignant peripheral nerve sheath tumor (MPNST) biology. We aimed to analyze whether initial tumor tissue expressions of survivin, p53, cyclin D1, osteopontin (OPN) and fibronectin (FN) correlate with the response to neo-adjuvant CHT (naCHT) in children with advanced inoperable MPNST. Methods The study included 26 children with MPNST (M/F 14/12, median age 130 months) treated in Polish centers of pediatric oncology between 1992 and 2013. Tissue expression of markers was studied immunohistochemically in the manually performed tissue microarrays and assessed semi-quantitatively as low and high, based on the rate of positive cells and staining intensity. Results Good response to naCHT was noted in 47.6%, while poor-in 52.4% of patients. The response to naCHT was influenced negatively by the presence of neurofibromatosis NF1 and high initial tumor tissue expression of OPN, survivin, p53 and cyclin D1. Patients with high tumor expression of either OPN, survivin or p53 and those with simultaneous high expression of ≥ 3 of the markers, responded significantly worse to naCHT, than patients, in whom expression of ≤ 2 markers were detected at diagnosis. Nearly, 85% of patients expressing ≥ 3 markers, responded poor to CHT; while 87.5% of children, expressing ≤ 2 markers, were good responders. Conclusion The initial tumor tissue expression of OPN, survivin, p53 and cyclin D1 may serve as markers to predict response to naCHT in pediatric advanced MPNST. Future studies in more numerous group of patients are needed to confirm these preliminary results

Wskazania do transplantacji komórek krwiotwórczych u dzieci i młodzieży – rekomendacje Polskiej Pediatrycznej Grupy ds. Transplantacji Komórek Krwiotwórczych – 2014

This article presents the current recommendations from the Polish Pediatric Group for Hematopoietic Stem Cell Transplantation concerning the indications for hematopoietic stem cell transplantation (HSCT) in children and adolescents suffering from hematological malignancies, solid tumours, and congenital or acquired non-malignant disorders. Indications for HSCT are established in context of the recent results of conventional treatment, i.e. obtained with non-HSCT strategies; it means transplantation is justifiable exclusively, when it significantly increases individual patient's chances to be cured, despite of the risk of HSCT-related mortality. Hence, due to the advances of non-transplant treatment strategies as well as progress in the field of HSCT the indications for HSCT require to be regularly up-dated. The recommendations presented in this article are based on the current guidelines from the European Group for Blood and Bone Marrow Transplantation (EBMT), including those from the EBMT Pediatric Diseases Working Party and the EBMT Inborn Errors Working Party, and from the international treatment protocols currently applied in the centers of the Polish Pediatric Leukemia/Lymphoma Study Group and Polish Pediatric Solid Tumours Study Group. The recommendations are addressed not only to the Polish pediatric transplant centers, but first of all to the Polish pediatric centers involved in diagnostics and treatment of the malignancies and non-malignant disorders in children and adolescents with non-transplant strategies, because it is their responsibility to identify as soon as possible indications for HSCT and refer patient at the appropriate time to pediatric transplant center

Pre-operative radiotherapy is associated with superior local relapse-free survival in advanced synovial sarcoma

PURPOSE: Optimization of local therapies in synovial sarcoma (SS) considered unresectable at diagnosis is needed. We evaluated the effects of neoadjuvant versus adjuvant radiation versus surgery only on long-term outcomes. METHODS: Patients with macroscopic SS tumors before chemotherapy (IRS-group-III) in the trials CWS-81, CWS-86, CWS-91, CWS-96, CWS-2002-P and SoTiSaR-registry were analyzed. Local therapies were scheduled after 3 neoadjuvant chemotherapy cycles. RESULTS: Median age of 145 patients was 14.5 years. 106 survivors had median follow-up of 7.0 years. Tumor site was 96 extremities, 19 head-neck, 16 shoulder/hip, 14 trunk. Tumors were  10 cm in 34 patients. In a secondary resection during chemotherapy, R0-status was accomplished in 82, R1 in 30, R2 in 21 (12 missing). Radiotherapy was administered to 115 (R0 61, R1 29, R2 20, missing 5), thereof 57 before and 52 after tumor resection. 23 were treated with surgery only. For all patients, 5 year event-free (EFS) and overall survival (OS) was 68.9% ± 7.6 (95%CI) and 79.1% ± 6.9. To establish independent significance, tumor site, size, surgical results and sequencing of local therapies were analyzed in a Cox regression analysis. Variables associated with EFS and OS are site, size and sequencing of local therapies. Variables associated with local recurrence are site, surgical results and sequencing of local therapies. The only variable associated with suffering metastatic recurrence is tumor size. CONCLUSION: Differences in sequencing of local therapy procedures are independently associated with outcomes. Best local control is achieved when tumors are irradiated pre-operatively and undergo R0 or R1 resection thereafter