Assessment of nutritional status in children with cancer: Significance of arm anthropometry and serum visceral proteins.

BACKGROUND: Arm anthropometry is a better indicator of nutritional status in children with cancer. The value of serum albumin and prealbumin in nutritional assesment is debatable. We investigated the nutritional status of children with cancer and their serum albumin and prealbumin levels. PATIENTS AND METHODS: At diagnosis and following induction therapies, weight, height, body mass index (BMI), mid-upper arm circumference (MUAC), and triceps skin-fold thickness (TSFT) were measured; serum albumin and prealbumin levels were determined. Prevalences of malnutrition defined by anthropometric indices were calculated. Correlations of anthropometric indices with each other, with serum albumin/prealbumin levels, and clinicopathological parameters were analyzed. RESULTS: In 81 patients, median age was 7.5 years (males/females = 50/31), tumors were located mostly in the abdomen, and abdominal tumors were more common under 5 years. Prevalence of malnutrition according to weight for age, BMI, MUAC, TSFT z scores were 14.8%, 23.5%, 27.2%, 21%, respectively. Defined by combined BMI/MUAC/TSFT measurements, 33/81 cases (40.7%) had malnutrition (z scores < -1, 23 mild; z scores < -2, 10 moderate). Malnutrition was more prevalent under 5 years (P = .03), also in abdominal tumors (P = .03) and advanced disease (P < .001). Younger age and advanced disease were risk factors for malnutrition. At diagnosis, prevalences of low serum albumin and prealbumin levels were 7.4% and 54%, respectively. Cases with malnutrition had significantly lower survival rates. CONCLUSIONS: Nutritional status is assessed best by MUAC and TSFT measurements. Serum prealbumin levels can be used to identify patients at risk of undernutrition. Presence of malnutrition is a significant poor prognostic factor. All children with cancer should undergo nutritional evaluation and active nutritional support.

Desenlace de 102 pacientes con linfoma de Hodgkin menores de 5 años / Outcome of 102 patients under 5 years of age with Hodgkin lymphoma

Antecedentes. El linfoma de Hodgkin (LH) es uno de los tipos de cáncer pediátrico con más posibilidades de curación. Sin embargo, es raro entre los niños menores de 5 años, y aún se desconocen los factores pronósticos de la supervivencia debido a su baja frecuencia en este grupo etario. Objetivos. El objetivo de este estudio fue evaluar las características clínicas, los esquemas de tratamiento y el desenlace de los pacientes con LH menores de 5 años. Métodos: se realizó una evaluación retrospectiva de los pacientes con diagnóstico de LH entre 1972 y 2013. Todos los pacientes recibieron quimioterapia, con o sin radioterapia. Resultados. Participaron 102 pacientes; la mediana de edad fue de 4 años (intervalo: 2-4,9). La mediana de seguimiento fue de 13 años. Veintitrés pacientes tenían síntomas B, 15 pacientes tenían neoplasia maligna con gran masa tumoral, y los estadios I y II fueron los más frecuentes. Las tasas de supervivencia global (SG) fueron significativamente diferentes según el estadio del cáncer (p = 0,008). Si bien no se observaron diferencias estadísticamente significativas, la presencia de neoplasia maligna con gran masa tumoral y de síntomas B estuvo asociada con un mal pronóstico. Conclusión. En este estudio unicéntrico se incluyó la mayor cantidad de pacientes con LH menores de 5 años. El estadio fue el principal factor de predicción de SG; por otro lado, la presencia de síntomas B y neoplasia maligna con gran masa tumoral también afectó el pronóstico.

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 followup 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.

Bilateral Wilms tumors: Treatment results from a single center.

Aydin B, Akyüz C, Yalçin B, Ekinci S, Oguz B, Akçören Z, Yildiz F, Varan A, Kurucu N, Büyükpamukçu M, Kutluk T. Bilateral Wilms tumors: Treatment results from a single center. Turk J Pediatr 2019; 61: 44-51. The management of bilateral Wilms tumor (BWT) is challenging, particularly due to its presentation at a younger age, rarity, and difficulty for treatment decisions and surgical evaluation comparing to unilateral WT. In this study, the outcome of BWT patients from a single center who were treated by the Turkish Pediatric Oncology Group (TPOG) Wilms Tumor Regimen were retrospectively reviewed. From 1990 to 2016, 30 patients with synchronous BWT were treated with a preoperative chemotherapy of vincristine and actinomycin-D (VA). Chemotherapy was continued until safe nephron sparing surgery (NSS) could be performed for as long as radiological tumor response continued; otherwise, the chemotherapy was intensified by adding doxorubicin (D) alternating with VA every 6 weeks. The median followup of patients was 59 months (4-297 months). The median duration of preoperative chemotherapy was 81 days and ranged between 14 days and 198 days. Preoperative chemotherapy was modified in seven patients (23%) to the VAD regimen. Twenty-two patients (73%) had a radical nephrectomy on the larger tumor and NSS on the contralateral kidney, and 6 patients (20%) had bilateral NSS. Postoperative tumor stages for stage I, II and III were 60%, 22% and 14%, respectively. The 5-year event free survival (EFS) rates were 100%, 90% and 51% for stages I, II and III (p=0.02), respectively. Unfavorable histology and nephrogenic rests were reported in 20% and 20% of patients, respectively. The 5-year overall survival (OS) and EFS rates were 50% and 25%, respectively, in patients with anaplasia, while the same rates were 96% and 96% in patients with favorable histology tumors (p=0.05 and p < 0.001). The 10-year EFS and OS rates for all patients were 82% and 86%, respectively. Our results are comparable with the literature. VA is effective as initial preoperative treatment of BWT and allows for safe resection.

Outcome of 102 patients under 5 years of age with Hodgkin lymphoma. / Desenlace de 102 pacientes con linfoma de Hodgkin menores de 5 años.

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 followup 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.

Antecedentes. El linfoma de Hodgkin (LH) es uno de los tipos de cáncer pediátrico con más posibilidades de curación. Sin embargo, es raro entre los niños menores de 5 años, y aún se desconocen los factores pronósticos de la supervivencia debido a su baja frecuencia en este grupo etario. Objetivos. El objetivo de este estudio fue evaluar las características clínicas, los esquemas de tratamiento y el desenlace de los pacientes con LH menores de 5 años. Métodos: se realizó una evaluación retrospectiva de los pacientes con diagnóstico de LH entre 1972 y 2013. Todos los pacientes recibieron quimioterapia, con o sin radioterapia. Resultados. Participaron 102 pacientes; la mediana de edad fue de 4 años (intervalo: 2-4,9). La mediana de seguimiento fue de 13 años. Veintitrés pacientes tenían síntomas B, 15 pacientes tenían neoplasia maligna con gran masa tumoral, y los estadios I y II fueron los más frecuentes. Las tasas de supervivencia global (SG) fueron significativamente diferentes según el estadio del cáncer (p = 0,008). Si bien no se observaron diferencias estadísticamente significativas, la presencia de neoplasia maligna con gran masa tumoral y de síntomas B estuvo asociada con un mal pronóstico. Conclusión. En este estudio unicéntrico se incluyó la mayor cantidad de pacientes con LH menores de 5 años. El estadio fue el principal factor de predicción de SG; por otro lado, la presencia de síntomas B y neoplasia maligna con gran masa tumoral también afectó el pronóstico.

Single institution experience on cancer among adolescents 15-19 years of age.

Yagci-Küpeli B, Akyüz C, Yalçin B, Varan A, Kutluk T, Büyükpamukçu M. Single institution experience on cancer among adolescents 15-19 years of age. Turk J Pediatr 2017; 59: 1-5. Adolescent cancers differ from other age groups in terms of cancer types, treatment and outcome. We aimed to present our institutional data on survival of certain types of cancer in adolescents. Hospital files were retrospectively evaluated for distribution of tumor types, clinical features, and overall and event-free survival (OS and EFS) rates in adolescents with malignant tumors. Two hundred ninety-three cases between 15-19 years who were diagnosed with malignant tumor at our department in the last 38 years were included in the study. Mean age was 15.3 and median age was 15 years at time of diagnosis. Male/female ratio was found to be 1.8/1. The most common cancers were non-Hodgkin lymphoma (NHL) (20.5%), Hodgkin's lymphoma (HL) (19.8%), central nervous system (CNS) tumors (10.9%), osteosarcoma (10.6%), Ewing sarcoma/primitive neuroectodermal tumors (EWN/PNET (9.9%) nasopharyngeal carcinoma (NPC) (9.6%). Non-compliance to medical treatment was observed in 10.9% of cases with significantly high non-compliance in NHL and osteosarcoma (p=0.02). Overall survival was 57.6% and EFS was 45.7% at a median follow-up time of 8.75 years. In adolescents, lower OS and EFS rates than the younger age group are observed. The most appropriate management plan should be made according to the physical and psychosocial needs of patients in this age group.

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.

Pediatric intramedullary spinal cord tumors: a single center experience.

AIM: To evaluate clinical and radiological findings, pathological features and treatment modalities in pediatric patients with intramedullary spinal cord tumors. PATIENTS AND METHODS: The medical records of 36 patients with intramedullary spinal tumors were reviewed for clinical, radiological and histopathological data, chemotherapy, radiotherapy, surgical resection, treatment responses, events, and final outcome. Survival analyses were performed. RESULTS: The median age was 7.9 years (range: 1-16 years; male/female ratio:1.4). Majority of the tumors were histopathologically diagnosed as astrocytomas (n = 16, 44.4%) and ependymomas (n = 19, 52.8%); whereas one was unclassified glioma. Overall, 94% of the astrocytomas and 84% of the ependymomas were low-grade, only three tumors were high-grade. In one patient with ependymoma, histopathological grade was undetermined. The primary tumor was commonly located in thoracic (47%) and cervical segments (28%). All patients had undergone surgery (gross-total resection, 33%; subtotal resection, 45%; biopsy, 22%). Radiotherapy was administered to 26 patients (72%) and chemotherapy to 15 patients (42%). The 3-, 5- and 10-year overall survival rates were 72%, 63% and 56%, respectively; and event-free survival rates were 43%, 40% and 40%. Survival did not significantly differ with gender, age groups, lag-time, neurologic status, histopathological tumor type, tumor location, extent of resection, treatment, or treatment responses in univariate survival analyses. Survival rates were significantly higher in patients with low-grade tumors and in ependymoma patients with resected tumors. CONCLUSIONS: Patients with low-grade tumors and those who underwent gross-total tumor resection had better prognosis. Surgery remains the main treatment in intramedullary spinal tumors. The role of radiotherapy and chemotherapy is limited and even controversial in low-grade tumors.

Clinical features and treatment results in children with anaplastic large cell lymphoma.

Anaplastic large cell lymphoma (ALCL) tends to have frequent relapse and good response to salvage chemotherapy. The frequency of ALCL among 1486 Non-Hodgkin's lymphoma (NHL) cases followed-up since 1972 was 1.5%, however, the percentage was 9.3% in cases diagnosed after 2000. Event-free survival (EFS) and overall survival (OS) rates for 23 children were 32.2% and 72.8% at 3 years, respectively. Disseminated diseases, no response to first line treatment, anaplastic lymphoma kinase (ALK) negativity were found as significant predictors on survival of ALCL. The proper diagnosis and early referral is essential in these children for a better survival rate. The children with ALK negative status should be monitored carefully because of the poor prognostic factors, and treated differently. The survival rates in this study are need of further improvement since the survival rates with current protocols are achievable at a level more than 80%. This is mainly related with late referral of those children with advanced disease.

Salivary gland tumors in children: a retrospective clinical review.

Seven patients with salivary gland tumors who underwent between 1972 and 2012 were retrospectively evaluated. The age of the patients ranged from 6.3 to 13 years old; five were females and two were males. Five patients had stage IVa, one patient had stage I, and one patient had stage II disease. The surgical margin was found to be positive in five cases. There were three adenoid cystic carcinoma, two adenocarcinoma, one anaplastic carcinoma, and one mucoepidermoid carcinoma. There were five parotid, one lacrimal gland, and one palatal involvement. Three patients who had low stage tumors were treated with surgery alone. Four of the cases received adjuvant radiation and chemotherapy. One patient with parotid tumor died with progressive disease. One case with lacrimal gland neoplasm was alive 48 months after discontinuation of treatment. The other palate case was lost to follow-up 1 month after the beginning of the treatment. Surgery is the primary treatment, with radiotherapy and chemotherapy used as adjuvant treatments. The treatment options need to be selected and planned for each individual patient.

Clinical features and treatment results of children with diffuse large B-cell lymphoma.

The demographic, clinical characteristics, and treatment groups of 33 children with diffuse large B-cell lymphoma (DLBCL) were recorded and analyzed among 1486 non-Hodgkin lymphoma (NHL) cases since 1972. The median age was 9.7 years (range 1.4-16.9) and male/female ratio was 24/9 = 2.6. Kaplan-Meier methods and logrank tests were used in treatment analysis. The frequency of DLBCL among 1486 NHL cases was 2.2%, however, the percentage was 9.3% in cases diagnosed after 2000. The event-free survival (EFS) and overall survival (OS) rates for 33 children were 61% and 65.1% at 5 years, respectively. The EFS and OS rates of low stage (stages I and II) disease decreased to lower level in advanced stage (stages III and IV) disease. Associated conditions and ages older than 14 years were found as poor prognostic factors in multivariate analysis. The survival rates in children with DLBCL need further improvement. This is mainly related with late referral of those children with advanced disease. The proper diagnosis and early referral is essential in these children for a better survival rate. The children with associated conditions and older children must be handled with care since these are found as poor prognostic factors.

Primary liver tumors in children: Hacettepe experience.

We aimed to review our experience with the clinical characteristics and outcome in childhood liver tumors. We investigated the clinical, laboratory and pathological characteristics, treatments and outcome in hepatoblastomas (HBL) and hepatocellular carcinomas (HCC). We identified 91 HBL and 42 HCC cases. Distant metastases were detected in 16% of HBLs and 22% of HCCs. PRETEXT stages were I/II in 34% and III/IV in 66% of HBLs and I/II in 16% and III/IV in 84% of HCCs. Most cases received cisplatin + doxorubicin chemotherapy. At a median of 58 months, 90 cases had died, 28 were alive, and 15 were lost to follow-up. Five-year survival rates were 32.4% for all HBLs and 15.6% for HCCs. Five-year survival rates were 47% in HBLs and 22.8% in HCCs diagnosed after 1990. In HBLs, distant metastases and absence of chemotherapy response indicated poor prognosis. Prognosis for childhood liver tumors has improved over the last two decades with preoperative chemotherapy with cisplatin + doxorubicin. Surgical resectability is important for cure. For HCC, more effective chemotherapy approaches are essential.

Infectious complications and conservative treatment of totally implantable venous access devices in children with cancer.

Besides their complications, totally implantable venous access devices (TIVADs) increase the quality of life in children with cancer. The aim of this study was analysis of infectious complications and results of conservative management in TIVADs. Three hundred and one catheters were implanted in 283 patients between February 1991 and January 2005. Infectious complications were analyzed retrospectively. Cumulative duration of implantation was 153,757 days. In 140 devices (46.5%), no complication was detected. Total rate of infection was 1.96/1000 catheter days. Types of infections were as follows: catheterrelated bloodstream infections: 190; catheter-related systemic infections: 74; pocket infections: 19, exit site infections: 14; and tunnel infections: 5. Staphylococcus epidermidis and non-albicans candida were the most common isolations. During follow-up, a total of 119 catheters had been removed. Most of them were due to infection (n=42). In conclusion, TIVADs are important in children with cancer who need prolonged intravenous access, so they should be used carefully and managed conservatively in case of complications.

Evaluation of patients with intracranial tumors and central diabetes insipidus.

The aim of the study is to evaluate the etiologic and clinical characteristics, treatment regimens, and outcome of the patients with intracranial tumors presenting with central diabetes insipidus (DI). Sixty-nine patients with intracranial tumors presenting with central DI between 1972 and 2012 were retrospectively evaluated. Fifty-three out of 69 patients were included in the analysis. Male/female ratio was 1.52, median age was 7.6 years. Of 53 patients, 37 patients (69.8%) were diagnosed with Langerhans cell histiocytosis, 14 patients (26.4%) with germinoma, 1 (1.9%) with astrocytoma, and 1 (1.9%) with optic glioma. 10-year overall survival (OS) rate and disease-free survival rate for all patients were 91.7% and 52%. 10-year OS rate according to diagnostic criteria was 91% for Langerhans cell histiocytosis (LCH) cases, 79% for intracranial germinoma, which was statistically significant (P = .0001). Central DI may be very important clinical presentation of serious underlying disease in children. Intracranial tumors are the most frequent cause of DI. Most frequent diagnosis were LCH and germ cell tumors in our series.

Cardiac rhabdomyomas in childhood: six cases from a single institution.

Primary cardiac tumors are rare during childhood. The most frequently encountered tumors are rhabdomyomas. We reviewed the clinical characteristics, treatment results, and outcomes of six pediatric patients with primary cardiac rhabdomyomas. The mean age was 16.8 days. Only one patient was symptomatic. The tumors mostly originated from the left ventricle. The diagnosis was established by magnetic resonance imaging (MRI) plus echocardiography with or without histopathology. Total tumor resection was performed in two patients. After a median follow-up of 39 months, one patient had a stable tumor, two patients had marked tumor regression and one had complete tumor regression. Considering the fact that rhabdomyomas often show spontaneous regression, close follow-up may be sufficient in hemodynamically stable cases. Although rhabdomyomas do not cause any symptoms at the time of diagnosis, they may lead to sudden death; thus, further studies may be required for the decision of surgery and/or followup. The localization and infiltrative characteristics of the tumor are critical factors for decision-making in children with symptomatic rhabdomyoma even if surgery is indicated in such cases.

Malignant sacrococcygeal germ cell tumors in children: a 30-year experience from a single institution.

BACKGROUND: Our aim was to analyze treatment results and survival characteristics of our patients with malignant sacrococcygeal germ cell tumors. PROCEDURE: Patient files of children with malignant sacrococcygeal germ cell tumors, treated at our institution between 1979 and 2009, were searched. Patient characteristics, histopathological subtypes, extension of disease, alpha-fetoprotein (AFP) level at the time of diagnosis and relapse, extent of surgical resection, chemotherapy protocols, details of radiotherapy and survival characteristics were recorded. RESULTS: A total of 58 patients (M/F = 20/38) with malignant sacrococcygeal germ cell tumor was included in analysis. With a mean follow-up of 156 months (range, 26 days to 288.8 months) overall and event-free survival rates of the 58 patients were 50.9% and 43.8%, respectively. AFP status of the patients (37% in patients with <10,000 ng/ml, 68.9% in patients with ≥ 10,000 ng/ml), type of resection (total vs others), coccygeal resection, chemotherapy protocol (PEB vs others) and number of chemotherapy courses had an impact on event-free survival in univariate analysis. In multivariate analysis, AFP status had the greatest effect on prognosis. CONCLUSIONS: Our treatment results are worse than those reported in the literature. Elevated AFP level at the time of diagnosis had a beneficial effect on prognosis, but year of diagnosis, tumor stage, presence of metastasis, tumor size and histopathological subtype had no impact on survival in patients with malignant sacrococcygeal germ cell tumors.

Educational achievement, employment, smoking, marital, and insurance statuses in long-term survivors of childhood malignant solid tumors.

BACKGROUND/OBJECTIVE: Survivors of childhood cancer experience many social adaptation problems. We aimed to identify social, educational, and occupational issues of this growing population. PATIENTS AND METHODS: Survivors treated for childhood malignant solid tumors who were older than 18 years and in remission for at least 3 years were surveyed. The educational achievement, employment, type of habitation, marital status, parenthood, social insurance, and smoking status of the patients were inquired and recorded. RESULTS: Two hundred one patients (126 male patients/75 female patients) were included in the study between 2007 and 2009. The median ages at the time of diagnosis and at the time of study were 10 years (range, 0 to 19 y) and 23 years (range, 18 to 39 y), respectively. The median follow-up duration was 13.5 years (range, 3 to 31 y). Nearly half of the participants were lymphoma survivors. One hundred eleven (55.5%) survivors were high school graduates and 47 (23%) were university graduates. Unemployment rate was 36.8%. Public social insurance rate was 90.5%. Fifty-three (26.4%) survivors had independent habitation. Thirty percent of survivors were married and 7.5% had at least 1 child. Marriage rates were significantly higher in survivors who were older than 23 years, had a follow-up duration of >13 years, had a job, and lived independently (for each parameter P=0.001). University degree was significantly lower in survivors who were treated for central nervous system tumors. CONCLUSIONS: Our results have drawn a more marked picture with lower educational achievement and marital rates when compared with the results of large survivorship studies conducted in developed countries. However, they can be interpreted as intriguing when limited resources are taken into account.

Thyroid cancer in pediatric age group: an institutional experience and review of the literature.

Very few have been reported on children with differentiated thyroid cancer (DTC), although 15% of them are diagnosed below 20 years of age. Children with DTC present with more advanced disease; however, they have a more favorable outcome. In this paper, we aimed to present the data in our institution on pediatric DTC patients, making an emphasis on the risk factors of metastasis and recurrence, as well as to the outcome of treatment. Clinical data of 50 pediatric patients referred to our institution for radioiodine treatment (RAI) between 1976 and 2010 were obtained. Papillary carcinoma was the most common histopathologic diagnosis (36 patients) followed by papillary carcinoma with follicular variant (10 patients). Multifocality was reported in 66% of the pathology reports. At the time of diagnosis 35 patients had regional lymph node metastasis, 18 had local invasion, and 11 had distant metastasis. No distant metastasis was present in patients with unifocal disease (P=0.018). The mean duration of follow-up was 77.6±62.7 months. Patients with local disease had longer disease-free survival than patients with distant metastasis (P=0.033). Despite the small number of patients, the follow-up was relatively long and the presented results confirmed overall good prognosis in children with DTC.

Optic glioma in children: a retrospective analysis of 101 cases.

AIM: To evaluate the clinical characteristics and long-term outcome of pediatric patients with optic glioma. PATIENTS AND METHODS: A total of 101 patients with optic glioma newly diagnosed between 1975 and 2008 were evaluated retrospectively. COPP (cyclophosphamide, vincristine, procarbazine, prednisolone) and cisplatin plus etoposide were the most commonly used chemotherapy regimens. Radiotherapy was administered in patients with progressive or unresponsive disease. RESULTS: The median age at the time of diagnosis was 6 years, and the male/female ratio was 1.15. The most common referral complaint was strabismus. The most common site of optic glioma was the hypothalamic-chiasmatic region (31.7%). Fifty-three patients (52.5%) had neurofibromatosis type 1 (NF-1). Treatment consisted of surgery, radiotherapy, and chemotherapy. Forty-nine patients (48.5%) underwent surgery, which was predominantly subtotal resection, radiotherapy was administered to 39.4%, and 30 patients received chemotherapy. The 5-year progression-free survival (PFS) and overall survival (OS) rates were 65.8% and 88.4%, respectively, and the 10-year PFS and OS were 54.2% and 83.4%, respectively, with an 8-year median follow-up. OS was significantly lower in patients with hypothalamo-chiasmatic involvement and significantly higher in patients with NF-1. The 5- and 10-year PFS rates were significantly higher in patients 10 years or older at diagnosis (P=0.0001) and in patients with intraorbital involvement (P=0.032). Eighteen patients (17.8%) died of disease. CONCLUSIONS: Patients with NF-l and those older than 10 years have a better prognosis, whereas patients younger than 3 years and those with hypothalamic-chiasmatic optic glioma have a worse outcome. Further studies are needed to find appropriate treatment strategies.

Tissue Doppler and myocardial deformation imaging to detect myocardial dysfunction in pediatric cancer patients treated with high doses of anthracyclines.

AIM: To detect subtle changes in myocardial function in pediatric cancer patients treated with high dose anthracyclines. METHODS: In all, 19 pediatric cancer patients with no cardiotoxicity findings treated with a cumulative anthracycline dose of ≥350 mg/m(2) and healthy participants of the same age were included in the study. Transthoracic echocardiography measurements, pulsed-wave tissue Doppler from the basal portions of all walls and strain and strain rate imaging of all walls from basal portion were obtained. RESULTS: A total of 19 patients (13 boys and six girls) and 17 participants (10 boys and seven girls) in the control group were enrolled. Three patients had subnormal ejection fraction (EF) and fractional shortening (FS) values. There was no significant difference in EF and FS between the two groups. The left ventricle isovolumic relaxation time was prolonged (P = 0.02) and the mitral color propagation velocity was decreased in patients (P = 0.001). Peak systolic strain and strain rates of the right ventricle, septal, lateral and inferior wall basal region of the left ventricle were significantly lower in patients than controls (all P < 0.05). Tissue Doppler measurements of early and late diastolic myocardial velocities of the right ventricular free wall, lateral wall of left ventricle and septum, and peak systolic myocardial velocity of the left ventricle anterior wall were significantly lower in patients (all P < 0.05). CONCLUSION: Myocardial Doppler and deformation imaging techniques provide a thorough evaluation of cardiac functions with the advantage of detecting subtle early changes before a global cardiac functional impairment occurs.