The Efficacy of Delayed Surgery in Children with High-Risk Neuroblastoma

Context: Surgery is an important part of treatment in children with neuroblastoma; however, exact timing is unclear. Both initial and delayed surgery was suggested as the best by numerous studies. Aims: Thus, we aimed to investigate the role of delayed surgery on 31 children with high-risk neuroblastoma. Materials and Methods: Thirty-one children with high-risk neuroblastoma were enrolled into the study. Statistical Analysis Used: Statistical analysis was performed using Statistical Package for the Social Sciences (SPSS) for windows 10.0. Results: There 'were 15 male and 16 female patients with a median age of 3.0 +/- 3.2 years. Primary tumor site was adrenal in 27, non-adrenal in two, pelvic in one, and mediastinal in one patient. MYCN gene was amplified in four and non-amplified in 11 children on totally 15 children with available data. Lactate dehydrogenase was elevated in 30 children. The tumor volumes at diagnosis and before surgery in the whole group were 154.3 and 12.5 mL, respectively. The decline in tumor volume was statistically significant (P < 0.0001). Initial surgery was performed in three and delayed in 20 children, and eight children were inoperable. Surgical complication rate was 66.6% (two out of three patients) in initial surgery group; however, the rate was 15% (3 out of 20 patients) in delayed surgery group. The 5-year event-free survival and overall survival rates in the whole group were 44.8% and 50.8%, respectively. Primary tumor area control rate was 95% Conclusions: In conclusion, the delayed surgery with intensive chemotherapy and radiotherapy has been successful for primary control in high-risk neuroblastoma patients.WoSScopu

Gluteal Congenital Fibrous Hamartoma of Infancy: A Case Report

Fibrous hamartoma of infancy (FHI) is a rare benign subcutaneous tumor which is commonly seen in the first year of life. Patients usually present with a single, painless, rapidly growing mass located mostly on the upper extremities. It can be difficult to distinguish this lesion from the malignant soft tissue sarcomas because of the rapid growth and radiological characteristics. Here we report a 5-month-old boy with a large gluteal mass and cutaneous findings which was initially thought to be an infiltrative malignant tumor and finally diagnosed as FHI. [Cukurova Med J 2013; 38(2.000): 333-337

Outcome Of 102 Patients Under 5 Years Of Age With Hodgkin Lymphoma

Background. Hodgkin's lymphoma (HL) is one of the most curable pediatric cancers, however it is rare among children under five years of age and prognostic factors for survival rate are still unknown due to low frequency in this age group. Objectives. The aim of this study was to evaluate clinical characteristics, treatment regimens, and outcome of patients under five years of age with HL. Methods. Patients diagnosed with HL between 1972 and 2013 were retrospectively evaluated. All patients were treated with chemotherapy with or without radiotherapy. Results. There were 102 patients with a median age of 4 years (range: 2 to 4.9). The median follow-up time was 13 years. Twenty-three patients had B symptoms, 15 patients had `bulky disease' and the most common stages were stage I and II. Overall survival (OS) rates were significantly different according to the stage of the cancer (p = 0.008). Although there were no statistically significant differences; the positivity of `bulky disease' and B symptoms were associated with poor prognosis. Conclusion. Our single-center study included the largest number of patients under five years of age with HL. The stage was the main predictor for OS; on the other hand, the presence of B symptoms and bulky disease has also affected the prognosis.WoSScopu

Primary Pleuropulmonary Neoplasms in Childhood: Fourteen Cases From a Single Center

Background: We aimed to review clinical characteristics, treatment results and outcome of pediatric patients with primary pleuropulmonary neoplasms. Methods: Medical records of 14 cases diagnosed between 1972-2009 were reviewed retrospectively. Results: The male/female ratio was 5/9 and the mean age at diagnosis was 9.1 years (2-16). All but one were symptomatic, presenting with fever, coughing, dyspnea, or weight loss. One patient presented with hemoptysis, and another with digital clubbing. One mesothelioma was diagnosed incidentally. Some 8/14 patients were initially diagnosed as having pneumonia (median delay in diagnosis of 2.5 months). Diagnoses included pleuropulmonary blastoma (PPB, n=5), inflammatory pseudotumor (n=3), mesothelioma (n=2), mucoepidermoid carcinoma (MEC, n=2), and carcinoid tumor (n=2). Patients with PPB underwent surgery and received chemotherapy +/- radiotherapy. Two carcinoid tumor cases underwent surgery, one further received chemotherapy. Patients with mesothelioma were treated with chemotherapy. Inflammatory pseudotumors were all resected. Two cases with MEC received chemotherapy, one after surgery. 2/5 PPB patients survived without recurrence, 3 died; all carcinoid tumors and inflammatory pseudotumors were alive; 1/2 MEC patients was alive after 252 months, the other one was lost without disease; 1/2 mesothelioma patients was alive without disease, the other was died. For all cases, median follow-up was 30.5 months (0.6-252). Conclusions: Primary pleuropulmonary tumors are rare but clinical presentation can be varied and delay in diagnosis is common. Children with persistent coughs, recurrent pneumonia or hemoptysis should be considered as indicators for early diagnosis, very important because the prognosis of these tumors varies with histology and stage.Wo

Surgical Treatment Results In Unilateral Wilms Tumor: Experience From A High-Volume Pediatric Oncology Center In Turkey

Objective: The aim of the present study was to evaluate the clinical characteristics, factors affecting treatment approach, and long-term outcome of patients with Wilms tumor. Methods: We identified the demographic features, mode of presentation, applied treatments, and long-term outcomes of 88 patients treated between 1990 and 2011 at Hacettepe University Ihsan Dogramaci Children's Hospital according to the Turkish Pediatric Oncology Group protocol. Data were analyzed using SPSS program, and chi-square test was used for statistical analysis. Results: The study included 88 patients (50 females and 38 males) with a mean age at presentation of 3 +/- 2.48 years. Patients were classified as stage 1 (n=35, 39.8%), stage 2 (n=16, 18.2%), stage 3 (n=17, 19.3%), and stage 4 (n=20, 22.7%). Pathological examination of tumors revealed favorable histology in 76 (86.4%) patients and unfavorable histology in 10 (11.4%) patients. Forty-nine (55.6%) patients received preoperative chemotherapy, and patient's age at diagnosis and physical examination findings influenced the decision of the administration of preoperative chemotherapy (p<0.05). Of the 88 patients, 25% aged <1 year and 75% aged between 3 and 5 years received preoperative chemotherapy. The palpated mass was crossing the midline in 20.5% of patients who were subjected to primary surgery. Tumor ruptured in 5.6% of patients intraoperatively. Long-term prognosis of patients was as follows: 68 (83.9%) children were cured and 13 (16%) children died due to recurrences and metastases. Survival rates reached 100% in stage 1 and 2 patients but decreased to 75% and 50% in stage 3 and 4 patients, respectively. Conclusion: Age at presentation and physical examination findings are significant in surgical planning. Stage is the most important prognostic factor. Patients with Wilms tumor are treated with low complication and high survival rates due to multidisciplinary treatment approach at our institution.Wo