IMPORTANCE: The current standard-of-care salvage therapy in relapsed/refractory classic Hodgkin lymphoma (cHL) includes consolidation high-dose chemotherapy (HDCT)/autologous stem cell transplant (aSCT). OBJECTIVE: To investigate whether presalvage risk factors and fludeoxyglucose-18 (FDG) positron emission tomography (PET) response to reinduction chemotherapy can guide escalation or de-escalation between HDCT/aSCT or transplant-free consolidation with radiotherapy to minimize toxic effects while maintaining high cure rates. DESIGN, SETTING, AND PARTICIPANTS: EuroNet-PHL-R1 was a nonrandomized clinical trial that enrolled patients younger than 18 years with first relapsed/refractory cHL across 68 sites in 13 countries in Europe between January 2007 and January 2013. Data were analyzed between September 2022 and July 2024. INTERVENTION: Reinduction chemotherapy consisted of alternating IEP (ifosfamide, etoposide, prednisolone) and ABVD (adriamycin, bleomycin, vinblastine, dacarbazine). Patients with low-risk disease (late relapse after 2 cycles of first-line chemotherapy and any relapse with an adequate response after 1 IEP/ABVD defined as complete metabolic response on FDG-PET and at least 50% volume reduction) received a second IEP/ABVD cycle and radiotherapy (RT) to all sites involved at relapse. Patients with high-risk disease (all primary progressions and relapses with inadequate response after 1 IEP/ABVD cycle) received a second IEP/ABVD cycle plus HDCT/aSCT with or without RT. MAIN OUTCOMES AND MEASURES: The primary end point was 5-year event-free survival. Secondary end points were overall survival (OS) and progression-free survival (PFS). PFS was identical to event-free survival because no secondary cancers were observed. PFS data alone are presented for simplicity. RESULTS: Of 118 patients analyzed, 58 (49.2%) were female, and the median (IQR) age was 16.3 (14.5-17.6) years. The median (IQR) follow-up was 67.5 (58.5-77.0) months. The overall 5-year PFS was 71.3% (95% CI, 63.5%-80.1%), and OS was 82.7% (95% CI, 75.8%-90.1%). For patients in the low-risk group (n = 59), 41 received nontransplant salvage with a 5-year PFS of 89.7% (95% CI, 80.7%-99.8%) and OS of 97.4% (95% CI, 92.6%-100%). In contrast, 18 received HDCT/aSCT off protocol, with a 5-year PFS of 88.9% (95% CI, 75.5%-100%) and OS of 100%. All 59 patients with high-risk disease received HDCT/aSCT (and 23 received post-HDCT/aSCT RT) with a 5-year PFS of 53.3% (95% CI, 41.8%-67.9%) and OS of 66.5% (95% CI, 54.9%-80.5%). CONCLUSION AND RELEVANCE: In this nonrandomized clinical trial, FDG-PET response-guided salvage in relapsed cHL may identify patients in whom transplant-free salvage achieves excellent outcomes. HDCT/aSCT may be reserved for primary progression and relapsed cHL with inadequate response. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT00433459.

• MeSH
• autologní transplantace MeSH
• dítě MeSH
• etoposid terapeutické užití   aplikace a dávkování   MeSH
• Hodgkinova nemoc * terapie   farmakoterapie   patologie   MeSH
• lidé MeSH
• lokální recidiva nádoru MeSH
• mladiství MeSH
• pozitronová emisní tomografie MeSH
• protokoly protinádorové kombinované chemoterapie terapeutické užití   MeSH
• transplantace hematopoetických kmenových buněk MeSH
• záchranná terapie * MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• klinické zkoušky, fáze III MeSH
• komentáře MeSH
• multicentrická studie MeSH

Hypodense volumes (HDV) in mediastinal masses can be visualized in a computed tomography scan in Hodgkin lymphoma. We analyzed staging CT scans of 1178 patients with mediastinal involvement from the EuroNet-PHL-C1 trial and explored correlations of HDV with patient characteristics, mediastinal tumor volume and progression-free survival. HDV occurred in 350 of 1178 patients (29.7%), typically in larger mediastinal volumes. There were different patterns in appearance with single lesions found in 243 patients (69.4%), multiple lesions in 107 patients (30.6%). Well delineated lesions were found in 248 cases (70.1%), diffuse lesions were seen in 102 cases (29.1%). Clinically, B symptoms occurred more often in patients with HDV (47.7% compared to 35.0% without HDV (p = 0.039)) and patients with HDV tended to be in higher risk groups. Inadequate overall early-18F-FDG-PET-response was strongly correlated with the occurrence of hypodense lesions (p < 0.001). Patients with total HDV > 40 ml (n = 80) had a 5 year PFS of 79.6% compared to 89.7% (p = 0.01) in patients with HDV < 40 ml or no HDV. This difference in PFS is not caused by treatment group alone. HDV is a common phenomenon in HL with mediastinal involvement.

• MeSH
• doba přežití bez progrese choroby MeSH
• dospělí MeSH
• fluorodeoxyglukosa F18 MeSH
• Hodgkinova nemoc * patologie   diagnostické zobrazování   MeSH
• lidé středního věku MeSH
• lidé MeSH
• mediastinum patologie   diagnostické zobrazování   MeSH
• mladiství MeSH
• mladý dospělý MeSH
• nádory mediastina * patologie   diagnostické zobrazování   MeSH
• počítačová rentgenová tomografie MeSH
• pozitronová emisní tomografie MeSH
• senioři MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

BACKGROUND: Disseminated pulmonary involvement in pediatric Hodgkin lymphoma (pHL) is indicative of Ann Arbor stage IV disease. During staging, it is necessary to assess for coexistence of non-malignant lung lesions due to infection representing background noise to avoid erroneously upstaging with therapy intensification. OBJECTIVE: This study attempts to describe new lung lesions detected on interim staging computed tomography (CT) scans after two cycles of vincristine, etoposide, prednisolone, doxorubicin in a prospective clinical trial. Based on the hypothesis that these new lung lesions are not part of the underlying malignancy but are epiphenomena, the aim is to analyze their size, number, and pattern to help distinguish true lung metastases from benign lung lesions on initial staging. MATERIALS AND METHODS: A retrospective analysis of the EuroNet-PHL-C1 trial re-evaluated the staging and interim lung CT scans of 1,300 pediatric patients with HL. Newly developed lung lesions during chemotherapy were classified according to the current Fleischner glossary of terms for thoracic imaging. Patients with new lung lesions found at early response assessment (ERA) were additionally assessed and compared to response seen in hilar and mediastinal lymph nodes. RESULTS: Of 1,300 patients at ERA, 119 (9.2%) had new pulmonary lesions not originally detectable at diagnosis. The phenomenon occurred regardless of initial lung involvement or whether a patient relapsed. In the latter group, new lung lesions on ERA regressed by the time of relapse staging. New lung lesions on ERA in patients without relapse were detected in 102 (7.8%) patients. Pulmonary nodules were recorded in 72 (5.5%) patients, the majority (97%) being<10 mm. Consolidations, ground-glass opacities, and parenchymal bands were less common. CONCLUSION: New nodules on interim staging are common, mostly measure less than 10 mm in diameter and usually require no further action because they are most likely non-malignant. Since it must be assumed that benign and malignant lung lesions coexist on initial staging, this benign background noise needs to be distinguished from lung metastases to avoid upstaging to stage IV disease. Raising the cut-off size for lung nodules to ≥ 10 mm might achieve the reduction of overtreatment but needs to be further evaluated with survival data. In contrast to the staging criteria of EuroNet-PHL-C1 and C2, our data suggest that the number of lesions present at initial staging may be less important.

• MeSH
• dítě MeSH
• doxorubicin terapeutické užití   MeSH
• etoposid terapeutické užití   aplikace a dávkování   MeSH
• Hodgkinova nemoc * diagnostické zobrazování   patologie   farmakoterapie   MeSH
• lidé MeSH
• mladiství MeSH
• nádory plic * diagnostické zobrazování   patologie   MeSH
• počítačová rentgenová tomografie * metody   MeSH
• předškolní dítě MeSH
• prevalence MeSH
• prospektivní studie MeSH
• protokoly protinádorové kombinované chemoterapie terapeutické užití   MeSH
• retrospektivní studie MeSH
• staging nádorů * MeSH
• vinkristin terapeutické užití   MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH

The clinical presentation of chronic myeloid leukemia (CML) at diagnosis differs in children compared to adults. At younger age, anemia appears to be frequent at diagnosis, but its prevalence and its impact on prognosis are not well known. In the International Registry of Childhood CML, we selected children and adolescents in chronic phase at diagnosis of CML and treated upfront with imatinib. We examined their hemoglobin level at diagnosis according to the WHO grades to assess the prevalence of anemia and its impact on response to tyrosine kinase inhibitors (TKIs). Data on 430 patients were included. Anemia at diagnosis was observed in 350 patients (81%), with a mean hemoglobin level of 96.4 g/l (SD 23.6). Among them, 182 patients (52%) presented with moderate anemia and 110 (31%) with severe anemia while 58 (17%) had mild anemia. Compared with mild and no anemia, moderate and severe forms were significantly associated with younger age at diagnosis, asthenia, splenomegaly, and increased leukocyte and basophil counts. Delays in achieving major and deep molecular responses were significantly increased for patients with moderate and severe anemia, and also failure of imatinib treatment was more frequent in these two sub-cohorts. However, hemoglobin level was not significantly associated with survival. Anemia at diagnosis of pediatric CML was frequent and may be considered as a prognostic factor.

• MeSH
• anemie * farmakoterapie   MeSH
• chronická myeloidní leukemie * farmakoterapie   MeSH
• dítě MeSH
• dospělí MeSH
• hemoglobiny MeSH
• imatinib mesylát terapeutické užití   MeSH
• inhibitory proteinkinas terapeutické užití   MeSH
• lidé MeSH
• mladiství MeSH
• prevalence MeSH
• prognóza MeSH
• protinádorové látky * terapeutické užití   MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• lidé MeSH
• mladiství MeSH
• Publikační typ
• časopisecké články MeSH

BACKGROUND: Rebound thymic hyperplasia (RTH) is a common phenomenon caused by stress factors such as chemotherapy (CTX) or radiotherapy, with an incidence between 44% and 67.7% in pediatric lymphoma. Misinterpretation of RTH and thymic lymphoma relapse (LR) may lead to unnecessary diagnostic procedures including invasive biopsies or treatment intensification. The aim of this study was to identify parameters that differentiate between RTH and thymic LR in the anterior mediastinum. METHODS: After completion of CTX, we analyzed computed tomographies (CTs) and magnetic resonance images (MRIs) of 291 patients with classical Hodgkin lymphoma (CHL) and adequate imaging available from the European Network for Pediatric Hodgkin lymphoma C1 trial. In all patients with biopsy-proven LR, an additional fluorodeoxyglucose (FDG)-positron emission tomography (PET)-CT was assessed. Structure and morphologic configuration in addition to calcifications and presence of multiple masses in the thymic region and signs of extrathymic LR were evaluated. RESULTS: After CTX, a significant volume increase of new or growing masses in the thymic space occurred in 133 of 291 patients. Without biopsy, only 98 patients could be identified as RTH or LR. No single finding related to thymic regrowth allowed differentiation between RTH and LR. However, the vast majority of cases with thymic LR presented with additional increasing tumor masses (33/34). All RTH patients (64/64) presented with isolated thymic growth. CONCLUSION: Isolated thymic LR is very uncommon. CHL relapse should be suspected when increasing tumor masses are present in distant sites outside of the thymic area. Conversely, if regrowth of lymphoma in other sites can be excluded, isolated thymic mass after CTX likely represents RTH.

• MeSH
• dítě MeSH
• fluorodeoxyglukosa F18 terapeutické užití   MeSH
• Hodgkinova nemoc * diagnostické zobrazování   farmakoterapie   komplikace   MeSH
• hyperplazie thymu * diagnostické zobrazování   etiologie   MeSH
• lidé MeSH
• lokální recidiva nádoru diagnostické zobrazování   farmakoterapie   MeSH
• lymfom * farmakoterapie   MeSH
• nádory brzlíku * diagnostické zobrazování   farmakoterapie   komplikace   MeSH
• počítačová rentgenová tomografie MeSH
• pozitronová emisní tomografie metody   MeSH
• radiofarmaka MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

BACKGROUND: Children and adolescents with early-stage classical Hodgkin lymphoma have a 5-year event-free survival of 90% or more with vincristine, etoposide, prednisone, and doxorubicin (OEPA) plus radiotherapy, but late complications of treatment affect survival and quality of life. We investigated whether radiotherapy can be omitted in patients with adequate morphological and metabolic responses to OEPA. METHODS: The EuroNet-PHL-C1 trial was designed as a titration study and recruited patients at 186 hospital sites across 16 European countries. Children and adolescents with newly diagnosed stage IA, IB, and IIA classical Hodgkin lymphoma younger than 18 years of age were assigned to treatment group 1 to be treated with two cycles of OEPA (vincristine 1·5 mg/m2 intravenously, capped at 2 mg, on days 1, 8, and 15; etoposide 125 mg/m2 intravenously, on days 1-5; prednisone 60 mg/m2 orally on days 1-15; and doxorubicin 40 mg/m2 intravenously on days 1 and 15). If no adequate response (a partial morphological remission or greater and PET negativity) had been achieved after two cycles of OEPA, involved-field radiotherapy was administered at a total dose of 19·8 Gy (usually in 11 fractions of 1·8 Gy per day). The primary endpoint was event-free survival. The primary objective was maintaining a 5-year event-free survival rate of 90% in patients with an adequate response to OEPA without radiotherapy. We performed intention-to-treat and per-protocol analyses. The trial was registered at ClinicalTrials.gov (NCT00433459) and with EUDRACT, (2006-000995-33) and is completed. FINDINGS: Between Jan 31, 2007, and Jan 30, 2013, 2131 patients were registered and 2102 patients were enrolled onto EuroNet-PHL-C1. Of these 2102 patients, 738 with early-stage disease were allocated to treatment group 1. Median follow-up was 63·3 months (IQR 60·1-69·8). We report on 714 patients assigned to and treated on treatment group 1; the intention-to-treat population comprised 713 patients with 323 (45%) male and 390 (55%) female patients. In 440 of 713 patients in the intention-to-treat group who had an adequate response and did not receive radiotherapy, 5-year event-free survival was 86·5% (95% CI 83·3-89·8), which was less than the 90% target rate. In 273 patients with an inadequate response who received radiotherapy, 5-year event-free survival was 88·6% (95% CI 84·8-92·5), for which the 95% CI included the 90% target rate. The most common grade 3-4 adverse events were neutropenia (in 597 [88%] of 680 patients) and leukopenia (437 [61%] of 712). There were no treatment-related deaths. INTERPRETATION: On the basis of all the evidence, radiotherapy could be omitted in patients with early-stage classical Hodgkin lymphoma and an adequate response to OEPA, but patients with risk factors might need more intensive treatment. FUNDING: Deutsche Krebshilfe, Elternverein für Krebs-und leukämiekranke Kinder, Gießen, Kinderkrebsstiftung Mainz of the Journal Oldtimer Markt, Tour der Hoffnung, Menschen für Kinder, Mitteldeutsche Kinderkrebsforschung, Programme Hospitalier de Recherche Clinique, and Cancer Research UK.

• MeSH
• dítě MeSH
• doxorubicin MeSH
• etoposid MeSH
• Hodgkinova nemoc * MeSH
• kvalita života MeSH
• lidé MeSH
• mladiství MeSH
• novorozenec MeSH
• prednison MeSH
• vinkristin MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• novorozenec MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• klinické zkoušky MeSH
• práce podpořená grantem MeSH

BACKGROUND: Children and adolescents with intermediate-stage and advanced-stage classical Hodgkin lymphoma achieve an event-free survival at 5 years of about 90% after treatment with vincristine, etoposide, prednisone, and doxorubicin (OEPA) followed by cyclophosphamide, vincristine, prednisone, and procarbazine (COPP) and radiotherapy, but long-term treatment effects affect survival and quality of life. We aimed to investigate whether radiotherapy can be omitted in patients with morphological and metabolic adequate response to OEPA and whether modified consolidation chemotherapy reduces gonadotoxicity. METHODS: Our study was designed as a titration study with an open-label, embedded, multinational, non-inferiority, randomised controlled trial, and was carried out at 186 hospital sites across 16 European countries. Children and adolescents with newly diagnosed intermediate-stage (treatment group 2) and advanced-stage (treatment group 3) classical Hodgkin lymphoma who were younger than 18 years and stratified according to risk using Ann Arbor disease stages IIAE, IIB, IIBE, IIIA, IIIAE, IIIB, IIIBE, and all stages IV (A, B, AE, and BE) were included in the study. Patients with early disease (treatment group 1) were excluded from this analysis. All patients were treated with two cycles of OEPA (1·5 mg/m2 vincristine taken intravenously capped at 2 mg, on days 1, 8, and 15; 125 mg/m2 etoposide taken intravenously on days 1-5; 60 mg/m2 prednisone taken orally on days 1-15; and 40 mg/m2 doxorubicin taken intravenously on days 1 and 15). Patients were randomly assigned to two (treatment group 2) or four (treatment group 3) cycles of COPP (500 mg/m2 cyclophosphamide taken intravenously on days 1 and 8; 1·5 mg/m2 vincristine taken intravenously capped at 2 mg, on days 1 and 8; 40 mg/m2 prednisone taken orally on days 1 to 15; and 100 mg/m2 procarbazine taken orally on days 1 to 15) or COPDAC, which was identical to COPP except that 250 mg/m2 dacarbazine administered intravenously on days 1 to 3 replaced procarbazine. The method of randomisation (1:1) was minimisation with stochastic component and was centrally stratified by treatment group, country, trial sites, and sex. The primary endpoint was event-free survival, defined as time from treatment start until the first of the following events: death from any cause, progression or relapse of classical Hodgkin lymphoma, or occurrence of secondary malignancy. The primary objectives were maintaining 90% event-free survival at 5 years in patients with adequate response to OEPA treated without radiotherapy and to exclude a decrease of 8% in event-free survival at 5 years in the embedded COPDAC versus COPP randomisation to show non-inferiority of COPDAC. Efficacy analyses are reported per protocol and safety in the intention-to-treat population. The trial is registered with ClinicalTrials.gov (trial number NCT00433459) and EUDRACT (trial number 2006-000995-33), and is closed to recruitment. FINDINGS: Between Jan 31, 2007, and Jan 30, 2013, 2102 patients were recruited. 737 (35%) of the 2102 recruited patients were in treatment group 1 (early-stage disease) and were not included in our analysis. 1365 (65%) of the 2102 patients were in treatment group 2 (intermediate-stage disease; n=455) and treatment group 3 (advanced-stage disease; n=910). Of these 1365, 1287 (94%) patients (435 [34%] of 1287 in treatment group 2 and 852 [66%] of 1287 in treatment group 3) were included in the titration trial per-protocol analysis. 937 (69%) of 1365 patients were randomly assigned to COPP (n=471) or COPDAC (n=466) in the embedded trial. Median follow-up was 66·5 months (IQR 62·7-71·7). Of 1287 patients in the per-protocol group, 514 (40%) had an adequate response to treatment and were not treated with radiotherapy (215 [49%] of 435 in treatment group 2 and 299 [35%] of 852 in treatment group 3). 773 (60%) of 1287 patients with inadequate response were scheduled for radiotherapy (220 [51%] of 435 in the treatment group 2 and 553 [65%] of 852 in treatment group 3. In patients who responded adequately, event-free survival rates at 5 years were 90·1% (95% CI 87·5-92·7). event-free survival rates at 5 years in 892 patients who were randomly assigned to treatment and analysed per protocol were 89·9% (95% CI 87·1-92·8) for COPP (n=444) versus 86·1% (82·9-89·4) for COPDAC (n=448). The COPDAC minus COPP difference in event-free survival at 5 years was -3·7% (-8·0 to 0·6). The most common grade 3-4 adverse events (intention-to-treat population) were decreased haemoglobin (205 [15%] of 1365 patients during OEPA vs 37 [7%] of 528 treated with COPP vs 20 [2%] of 819 treated with COPDAC), decreased white blood cells (815 [60%] vs 231 [44%] vs 84 [10%]), and decreased neutrophils (1160 [85%] vs 223 [42%] vs 174 [21%]). One patient in treatment group 2 died of sepsis after the first cycle of OEPA; no other treatment-related deaths occurred. INTERPRETATION: Our results show that radiotherapy can be omitted in patients who adequately respond to treatment, when consolidated with COPP or COPDAC. COPDAC might be less effective, but is substantially less gonadotoxic than COPP. A high proportion of patients could therefore be spared radiotherapy, eventually reducing the late effects of treatment. With more refined criteria for response assessment, the number of patients who receive radiotherapy will be further decreased. FUNDING: Deutsche Krebshilfe, Elternverein für Krebs-und leukämiekranke Kinder Gießen, Kinderkrebsstiftung Mainz, Tour der Hoffnung, Menschen für Kinder, Programme Hospitalier de Recherche Clinique, and Cancer Research UK.

• MeSH
• cyklofosfamid terapeutické užití   MeSH
• dítě MeSH
• folikuly stimulující hormon krev   MeSH
• Hodgkinova nemoc farmakoterapie   mortalita   radioterapie   MeSH
• lidé MeSH
• mladiství MeSH
• prednison terapeutické užití   MeSH
• prokarbazin terapeutické užití   MeSH
• protokoly protinádorové kombinované chemoterapie škodlivé účinky   terapeutické užití   MeSH
• staging nádorů MeSH
• vinkristin terapeutické užití   MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH
• práce podpořená grantem MeSH
• randomizované kontrolované studie MeSH
• srovnávací studie MeSH

BACKGROUND: In the EuroNet Pediatric Hodgkin Lymphoma (EuroNet-PHL) trials, decision on Waldeyer's ring (WR) involvement is usually based on clinical assessment, that is, physical examination and/or nasopharyngoscopy. However, clinical assessment only evaluates mucosal surface and is prone to interobserver variability. Modern cross-sectional imaging technology may provide valuable information beyond mucosal surface, which may lead to a more accurate WR staging. PATIENTS, MATERIALS, AND METHODS: The EuroNet-PHL-C1 trial recruited 2102 patients, of which 1752 underwent central review including reference reading of their cross-sectional imaging data. In 14 of 1752 patients, WR was considered involved according to clinical assessment. In these 14 patients, the WR was re-assessed by applying an imaging-based algorithm considering information from 18 F-fluorodeoxyglucose positron emission tomography, contrast-enhanced computed tomography, and/or magnetic resonance imaging. For verification purposes, the imaging-based algorithm was applied to 100 consecutive patients whose WR was inconspicuous on clinical assessment. RESULTS: The imaging-based algorithm confirmed WR involvement only in four of the 14 patients. Of the remaining 10 patients, four had retropharyngeal lymph node involvement and six an inconspicuous WR. Applying the imaging-based algorithm to 100 consecutive patients with physiological appearance of their WR on clinical assessment, absence of WR involvement could be confirmed in 99. However, suspicion of WR involvement was raised in one patient. CONCLUSIONS: The imaging-based algorithm was feasible and easily applicable at initial staging of young patients with Hodgkin lymphoma. It increased the accuracy of WR staging, which may contribute to a more individualized treatment in the future.

• MeSH
• dítě MeSH
• fluorodeoxyglukosa F18 analýza   MeSH
• Hodgkinova nemoc diagnostické zobrazování   MeSH
• lidé MeSH
• magnetická rezonanční tomografie MeSH
• mladiství MeSH
• multimodální zobrazování MeSH
• počítačová rentgenová tomografie MeSH
• pozitronová emisní tomografie MeSH
• předškolní dítě MeSH
• staging nádorů MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• klinické zkoušky MeSH
• práce podpořená grantem MeSH

Bolesti brucha patria medzi pomerne časté symptómy u adolescentov. Príčiny, ktoré sa podieľajú na bolestiach brucha, sú pestré a tým aj diferenciálna diagnostika je pomerne rozsiahla a zložitá. To súvisí pravdepodobne s tým, že brucho zaberá pomerne veľkú oblasť, obsahuje množstvo orgánov, štruktúr, systémov vrátane lymfatických uzlín, ktoré generalizovane reagujú na infekciu, či nádorové ochorenia. V našej kazuistike opisujeme prípad 17-ročného pacienta s náhlymi bolesťami brucha, chudnutím, vracaním a ikterom. V rámci diferenciálnej diagnostiky sa realizovali laboratórne a zobrazovacie vyšetrenia so zameraním na pankreas a hepatobiliárny trakt, kde sa zobrazila zväčšená hlava a chvost pankreasu, peripankreatická lymfadenopatia s vedľajším nálezom drobných ložísk na obličkách. V úvode sa suponovala skôr hereditárna pankreatitída a pseudotumory na obličkách, vzhľadom na vek pacienta bol menej pravdepodobný tumor pankreasu a metastázy. Definitívna diagnóza bola stanovená odberom bioptických vzoriek pankreasu pomocou endosonografického vyšetrenia. Histologický a cytologický nález preukázal prítomnosť lymfocytárnych blastických buniek, charakteru CD20+ B-NHL, konštatovaný difúzny veľkobunkový B-lymfóm pankreasu (DLBCL – diffuse large B cell lymphoma). Staging ochorenia nepreukázal inflitráciu malígnych buniek v kostnej dreni a centrálnej nervovej sústave. Promptne stanovená diagnóza a podaná príslušná onkologická liečba (chemoterapia v kombinácii s monoklonálnou protilátkou) zabezpečila pacientovi návrat do každodenného života a je nevyhnutným predpokladom priaznivej prognózy pacienta.

Abdominal pain is a relatively common symptom in adolescents. The causes that contribute to abdominal pain are varied and the differential diagnosis is therefore rather extensive and complex. This is probably related to the fact that the abdomen extends over a relatively large area containing numerous organs, structures, and systems, including the lymph nodes which respond to infection or cancer in a generalized way. In our case report we present the case of a 17-year-old patient with sudden abdominal pain, weight loss, vomiting and jaundice. Laboratory and imaging examinations were performed, focusing on the pancreas and the hepatobiliary tract and showing an enlarged head and tail of the pancreas, peripancreatic lymphadenopathy with a secondary finding of small lesions on the kidneys. Firstly, we suspected hereditary pancreatitis and pseudotumors in the kidney, while pancreatic tumor and metastases were considered less likely, given the patient’s age. The definitive diagnosis was established by collecting biopsy samples from the pancreas via endosonographic examination. Histological and cytological findings showed the presence of lymphocyte blast cells of the CD20 + B-NHL nature, diagnosed as diffuse large B cell lymphoma (DLBCL). Staging of the disease showed no infiltration of malignant cells in the bone marrow and central nervous system. The prompt diagnosis and administration of appropriate oncological treatment (chemotherapy in combination with a monoclonal antibody) ensured the patient’s return to daily life and they were a necessary prerequisite for a favorable prognosis of the patient.

• MeSH
• bolesti břicha etiologie   MeSH
• difúzní velkobuněčný B-lymfom * diagnóza   patologie   terapie   MeSH
• lidé MeSH
• mladiství MeSH
• nádory slinivky břišní * diagnóza   terapie   MeSH
• pankreas diagnostické zobrazování   patologie   MeSH
• PET/CT metody   MeSH
• protokoly protinádorové kombinované chemoterapie MeSH
• výsledek terapie MeSH
• Check Tag
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• Publikační typ
• kazuistiky MeSH

Východiská: Neuroblastóm je najčastejší solídny extrakraniálny nádor detského veku s extrémne heterogénnym bio­logickým a klinickým správaním. Napriek pokrokom v liečbe je dlhodobá prognóza pacientov s vysokorizikovým neuroblastómom a s relapsom ochorenia nepriaznivá. Potenciál zlepšiť ju prináša implementácia imunoterapie do liečebných protokolov. Dinutuximab, chimerická monoklonálna protilátka, vedie k apoptóze nádorových buniek prostredníctvom väzby na GD2 receptor. Cieľom článku je prezentácia prvých skúseností nášho centra s liečbou dinutuximabom. Súbor a metódy: V rokoch 2018–2019 sme podali 7 pacientom 31 cyklov dinutuximabu. Piatim pacientom s vysokorizikovým neuroblastómom bol dinutuximab podávaný v 1. línii, u dvoch pacientov s relapsom ochorenia bol dinutuximab podávaný v rámci 2. línie liečby. Na vyhodnotenie toxicity liečby boli retrospektívne analyzované ošetrovateľské záznamy pacientov počas podávania imunoterapie. Výsledky: U dvoch pacientov liečených dinutuximabom v 1. línii pretrváva po ukončení imunoterapie kompletná remisia, traja pacienti dosiahli parciálnu odpoveď. Obom pacientom s relapsom neuroblastómu bol po ukončení imunoterapie dia­gnostikovaný druhý relaps a exitovali na progresiu ochorenia. Tolerancia liečby bola u väčšiny pacientov primeraná – u 6 pacientov boli nežiaduce účinky zvládnuté podpornou liečbou. Išlo najmä o prejavy capillary leak syndrómu, bolesti a hypersenzitívne reakcie. U jedného pacienta bola liečba prerušená pre závažnú neurotoxicitu. Záver: Dinutuximab má preukázateľný benefit v eradikácii minimálnej reziduálnej choroby pri liečbe neuroblastómu. Imunoterapia je v súčasnosti štandardom prvolíniovej liečby pacientov s vysokorizikovým neuroblastómom. Jej úloha v liečbe pacientov s relapsom ochorenia je predmetom viacerých prebiehajúcich štúdií, rovnako ako aj optimalizácia terapeutických schém v oboch indikáciách. Liečba dinutuximabom je spojená s rizikom rozvoja širokej palety nežiaducich účinkov, preto jeho podávanie patrí do rúk skúseného centra detskej onkológie.

Background: Neuroblastoma is the most common extracranial solid tumour of childhood with extremely heterogeneous biological and clinical behaviour. Despite advances in its treatment, the long-term prognosis of patients with a high-risk and relapsed neuroblastoma remains poor. The implementation of immunotherapy into the treatment protocols has the potential to improve it. Dinutuximab, a chimeric monoclonal antibody, leads to the apoptosis of tumour cells through binding to the GD2 receptor. The article aim is to present the first experience of our centre with dinutuximab treatment. Patients and methods: In 2018–2019, we administered 31 cycles of dinutuximab to seven patients. Five patients with high-risk neuroblastoma received dinutuximab in the first line, in two patients with relapse, dinutuximab was administered in the second line of treatment. To evaluate the toxicity of the treatment, the nursing records of patients during immunotherapy were retrospectively analysed. Results: Two patients treated with dinutuximab in the first line are in complete remission, three patients achieved a partial response. Both patients with relapsed neuroblastoma were diagnosed with a second relapse after immunotherapy and died of disease progression. The treatment tolerance was acceptable in most patients – in six patients adverse events were managed with adequate supportive care. These were mainly symptoms of capillary leak syndrome, pain and hypersensitivity reactions. In one patient, the treatment was discontinued due to severe neurotoxicity. onclusion: Dinutuximab has a proven benefit in the eradication of the minimal residual disease in the treatment of neuroblastoma. Immunotherapy is currently the standard for first-line treatment of high-risk neuroblastoma. Its role in the treatment of relapsed neuroblastoma is a subject of several ongoing studies as well as the optimization of therapeutic regimens. Dinutuximab administration is associated with a considerable risk of severe adverse reactions, so the treatment belongs to the hands of an experienced paediatric oncology centre.

• Klíčová slova
• dinutuximab,
• MeSH
• dítě MeSH
• lidé MeSH
• monoklonální protilátky terapeutické užití   MeSH
• neuroblastom * farmakoterapie   MeSH
• protinádorové látky terapeutické užití   MeSH
• protokoly protinádorové léčby MeSH
• retrospektivní studie MeSH
• Check Tag
• dítě MeSH
• lidé MeSH