Search

Hodgkin's lymphoma (HL) is highly curable disease in its early stages, but in advanced stages, it presents a dilemma when it becomes refractory or relapses after several rounds of chemotherapy. Brentuximab vedotin (BV) is an antibody-drug conjugate that targets the tumor necrosis receptor family protein member CD30 positive malignancies via an anti-CD30 monoclonal antibody linked to monomethyl auristatin-E. In adult and pediatric studies, it has been shown to be an effective salvage therapy for primary refractory HL or relapse after autologous stem cell transplant (ASCT). Between July 2012 and August 2017, we administered BV (1.8 mg/m2 every three weeks; 12 cycles totally) with doxorubucin, vinblastin, dacarbazine (AVD), rituximab + ifosfamide + carboplatin + etoposide (RICE), or bendamustine combination treatment in pediatric HL patients, who were previosuly treated for refractory or relapsed advanced stage HL before (seven patients) or after (one patient) ASCT in our center. After eight BV courses, one patient was able to undergo match unrelated donor (MUD) SCT. Another seven pediatric HL patients, who were not able to go into remission with any other classical HL chemotherapy protocols, received 4-6 courses of BV-AVD and/or RICE/bendamustine. All were able to undergo ASCT after negative positron emission tomography (PET) imaging results. After ASCT, we switched to BV as consolidation therapy until a total of 12 cycles was completed. Patients went into remission after a median 34 (range: 12-42) months from the start of BV treatment. BV is an encouraging, well- tolerated, and effective targeted therapy especially when combined with AVD or when alternated with another targeted therapy combination, including RICE, when needed. [ABSTRACT FROM AUTHOR]

Respiratory tract viruses have an important effect on morbidity and mortality in patients with febrile neutropenia (FN). The aim of this study was to determine frequency and clinical influence of viral respiratory viruses as potential etiologic agents in episodes of FN in children. A total of 100 children (62 boys, 38 girls) with 166 FN episodes were included in this prospective study. Nasopharyngeal aspirate samples were analyzed for respiratory viral agents using multiplex real-time polymerase chain reaction. The origin of the fever could be defined in 111 (67%) of the episodes. We detected viral agents in 86 (51.8%), bacterial agents in 19 (11.4%), and fungal agents in 5 (3%) of the episodes. The most common detected viruses were rhinovirus (n= 27), respiratory syncytial virus (n=17), and coronavirus (n=16). Parainfluenza virus, influenza A and B, adenovirus, human metapneumovirus, enterovirus, bocavirus and parechovirus were the remaining detected agents. More than one virus positivity occurred in 13 FN episodes. Forty-three patients had multiple FN episodes. Only four patients had the same viral agent in consecutive attacks. Respiratory symptoms (cough, nasal discharge and congestion, sneezing, wheezing), physical examination signs (rales and rhonchi) and radiological findings were significantly more common in viral agent positive patients (p < 0.05). This study showed that respiratory viruses make a substantial contribution on the etiology of FN episodes in children. Identifying viral agents may help to constitute individualized infection-management algorithms in these patients. [ABSTRACT FROM AUTHOR]

Interleukin (IL)-13 has been reported to have a role in the pathogenesis of lymphoma through recent molecular studies predominantly in adult patients. As malignant lymphomas in children differ from adult counterparts in terms of histology and response to treatment, we aimed to determine the serum IL-13 levels of patients with lymphoma; its relation with clinical-laboratory parameters and to look for any correlation of serum IL-13 levels with different prognostic factors in children. Twenty-eight patients with malignant lymphoma and 20 age-matched healthy controls were included in the study. The median serum IL-13 level at diagnosis (range 0.59-68 pg/ml, median 3.40 pg/ml) was higher than that in remission (range 0.14-12.2 pg/ml, median 1.60 pg/ml) in the HL group (p<0.05). Remarkably, median serum IL-13 level of patients with nodular sclerosis at diagnosis was higher than those with mixed-cellularity (p<0.05) and declined to normal limits during remission (p<0.05). In Burkitt's lymphoma (BL) subgroup, the median (range 2.94-154 pg/ml, median 4.5 pg/ml) was high and declined to normal levels during remission (range 0.55-11.30 pg/ml, median 1.57 pg/ml) and the difference was significant (p<0.05). In terms of prognostic factors, serum IL-13 levels were found to be associated with white blood cells counts only in HL group. Although the number of patients is limited in our study, we found that the serum IL-13 levels exhibit variances in different histopathologic groups. IL- 13 might have a role in histopathogenesis of lymphoma, but seems to have no prognostic significance. Nevertheless, more molecular studies are needed to evaluate the pathogenesis of HL. [ABSTRACT FROM AUTHOR]

Neuroblastoma (NB) is the most frequently diagnosed neoplasm during infancy and its incidence declines within the first 3-5 years of life. It can be rarely diagnosed in adolescents and young adults. Adolescents have advanced stage of disease, higher frequency of uncommon metastatic sites such as lungs, and worse outcomes. Herein, we describe an unusual case of NB in a 17-yearold adolescent presented with lung metastasis at diagnosis. The patient was diagnosed with stage IV NB. Thorax high-resolution computed tomography (HRCT) scan revealed irregular septal thickening with ground glass opacity consistent with pulmonary parenchymal metastases. After the first cycle of chemotherapy he developed pulmonary hemorrhage and respiratory distress. He required ventilation support and mechanical ventilation was started. Metastatic nodules were determined on second thorax HRCT. We lost the patient due to septic shock and multiple organ failure 2 months after diagnosis. In conclusion, adolescents with NB have unfavorable prognosis. These patients may have lung metastases at diagnosis. Therefore, detailed chest imaging at initial diagnosis is crucial. [ABSTRACT FROM AUTHOR]

Background: It has been estimated that rare tumor rate is about 15% of all childhood cancer in United States. According to Turkish Pediatric Oncology Group (TPOG) datas, 8889 children were diagnosed between 2002 and 2008 in our country and 3.7% of them were diagnosed as rare tumors. Aim: To investigate the frequency and clinical features of rare tumors in our pediatric oncology center. Materials and Methods: A total of 43 cases that have diagnosed as rare tumor in 574 cancer patients between the yaer 2002 and 2012 were reviewed retrospectively. All cases definitive diagnosis were established by histopathological and immunohistochemical studies. Results: Frequency of rare tumors was 7.4% in our center. Benign and border line rare tumors were 27 (62.7%) cases, malignant rare tumor were 16 (37.2%) cases. Median follow-up period was 48 months (between 1 and 110 months). Six of the malignant rare tumors were died with progressive disease (synovial sarcoma, mixed malignant mesenchymal tumor, undifferentiated sarcoma, plexus choroideus carcinoma, renal peripheral primitive neuroectodermal tumor, adrenocortical carcinoma). Malignant rare tumor mortality rate was found 37.5% in our clinic. Conclusion: We have found that our rare tumor rate (7.4%) was higher than Turkish rare tumor rate (3.7%) according to TPOG's datas. However, it was still lower than rare tumor rates of western countries (15%), probably due to difficulties of diagnosis and referral problems. [ABSTRACT FROM AUTHOR]

Central venous catheter (CVC) fracture with embolization is a serious and rare complication, and few cases have been reported in children. Catheter fragments may cause cardiac perforation, arrythmias, pulmonary embolism, and sepsis. We report the successful retrieval of intracardiac CVC fragments by percutaneous transcatheter technique in two infants, aged 8 and 15 months. Double-lumen 7 French Hickman CVCs were accidentally fractured during their removal. Chest radiographs of the two patients revealed migrated intracardiac catheter fragments extending from the superior vena cava (SVC) to the right atrium and from the SVC to the right ventricle, respectively. The procedure was performed under ketamine anesthesia and fluoroscopic guidance using a percutaneous femoral vein approach. Nitinol Amplatz Goose Neck™ snares (10 mm in diameter) were used to successfully retrieve the catheter fragments without any complication. Percutaneous transcatheter retrieval of catheter fragments using Goose Neck snare is a safe and successful technique and can be chosen before resorting to surgery, which has potential risks related to general anesthesia, thoracotomy and cardiopulmonary bypass. [ABSTRACT FROM AUTHOR]

Background: Stenotrophomonas maltophilia is an important cause of life-threatening nosocomial infection. Aim: To evaluate the clinical features, antibiotic treatment and prognosis of S. maltophilia bacteraemia. Methods: Patients with blood cultures positive for S. maltophilia at the Children's Hospital, Ankara University Medical School between 1995 and 2005 were evaluated retrospectively. The results were compared with those of a case–control group of patients with Pseudomonas aeruginosa bacteraemia (n=33). Antibiotic susceptibilities of S. maltophilia strains were determined by disc diffusion. Susceptibility to ciprofloxacin was also determined by broth dilution. Results: Thirty-six (2.2%) blood cultures were positive for S. maltophilia. Neutropenia was more common in the P. aeruginosa group (p=0.001). Breakthrough bacteraemia developed more commonly during carbapenem treatment in the S. maltophilia group (p=0.02). Ciprofloxacin and trimethoprim–sulfamethoxazole in combination with/without an aminoglycoside were the antibiotics most commonly selected to treat S. maltophilia bacteraemia. Mortality was more common in the P. aeruginosa (13/33) than in the S. maltophilia (2/33) group (p=0.001). According to susceptibility, determination by the disk diffusion method, beta-lactam antibiotics, aminoglycosides and chloramphenicol had little or no effect, whereas trimethoprim–sulfamethoxazole, doxycycline and fluoroquinolones were more active against S. maltophilia strains. However, ciprofloxacin susceptibility results were quite different when determined by disk diffusion (97% isolates susceptible) and broth dilution (49% isolates susceptible). Conclusions: Although S. maltophilia bacteraemia is rare in children, antibiotic resistance to these strains is an important problem. Tetracyclines, trimethoprim–sulfamethoxazole and fluoroquinolones are the most active agents against S. maltophilia strains. [ABSTRACT FROM AUTHOR]

The objective of this study is to present an unusual case of Fanconi anemia (FA) with an initial presentation of abdominal rhabdomyosarcoma followed by acute myeloid leukemia (AML). Although epithelial cancers are more often seen in FA patients, rarities such as osteosarcomas, soft-tissue tumors and brain cancers have been reported in the literature. The present case was treated with VACA treatment (vincristine, actinomycin D, cyclophosphamide and adriamycin) and radiotherapy following initial assessment through surgery and histopathological evaluation. A second malignancy AML developed 17 months after completion of initial treatment and the patient died at 2 months remission and 6 months survival following the diagnosis of AML. [ABSTRACT FROM AUTHOR]

Although both Hodgkin lymphoma and cyst hydatic disease in children have been seen with an increased frequency, there is no previously reported case of Hodgkin lymphoma associated with cyst hydatic disease from Turkey. The authors report such a case of Hodgkin lymphoma. Intrahepatic cystic masses were diagnosed during ultrasound examination for clinical staging on admission. The diagnosis of cyst hydatic of the liver was confirmed by surgery. Although there was no residual and/or new cyst formation on radiologic follow-up, elevated antibody titers (indirect hemagglutination test) persisted following surgical excision at least for 2 years of follow-up. [ABSTRACT FROM AUTHOR]

Extraocular retinoblastoma occurs more frequently in developing countries as a delayed diagnosis and prognosis of patients with conventional therapy is very poor. Metastatic retinoblastoma, especially in the central nervous system (CNS), is a highly lethal disease. Tamoxifen has been used in some previous studies with variety of responses to therapy in patients with unresectable recurrent brain tumors. A 7-year-old girl with recurrent metastatic retinoblastoma received 60 mg/m 2 tamoxifen in addition to chemotherapy and CNS radiotherapy. She was in remission until she has died in a traffic accident at week 114. The authors think that tamoxifen can be added to treatment protocols of metastatic retinoblastoma to provide longer and at least higher quality of life for these patients. [ABSTRACT FROM AUTHOR]

Ninety-six untreated patients with malignant lymphoma (ML), 81 Hodgkin's disease, and 15 Burkitt's lymphoma were studied for zinc (Zn) status, and 21 patients also had selenium (Se) status analysis. Plasma and hair Zn and Se levels were measured by atomic absorption spectrophotometry. Chronic Zn and Se deficiencies (low plasma and low hair Zn and Se levels together) were found to be associated with ML in Turkish children. This was most likely due to the poor “nutritional environment” of the patients because majority of the ML patients were from families of low socioeconomic status. Supplementation of pediatric ML patients with Zn and Se, in addition to standard chemotherapy and radiotherapy regimen, is recommended. [ABSTRACT FROM AUTHOR]

The multidrug-resistant bacterial infections cause high mortality in immunocompromised patients because of the limited antibacterial choices. Tigecycline, first member of the glycylcyclines, has in vitro activity against a wide variety of organisms, including multidrug-resistant pathogens; however, it has not yet been approved for use in children. Herein, we report a nine-year-old girl with acute myeloid leukemia who was treated successfully with tigecycline due to multidrug-resistant Escherichia coli bacteremia. [ABSTRACT FROM AUTHOR]

Patients who survive Hodgkin lymphoma (HL) are at increased risk of secondary neoplasms (SNs). A wide variety of SNs have been reported, including leukemias, non-Hodgkin's lymphomas, and solid tumors, specifically breast and thyroid cancers. Herein we report subsequent neoplasms in four patients with HL receiving chemoradiotherapy. It is interesting that three SNs, fibrosarcoma, thyroid carcinoma, and retrobulbar meningioma, were observed in the radiation area in one of our patients. A hypopharyngeal epithelioid malignant peripheral nerve sheath tumor as an unusual secondary malignant neoplasm developed in another patient, while a benign thyroid nodule and invasive ductal breast carcinoma were observed at different times in the female patient. Follicular adenoma of the thyroid gland developed in one of our patients.