Ali Varan

Evaluation of Coronary Artery Disease by Computed Tomography Angiography in Patients Treated for Childhood Hodgkin's Lymphoma

PURPOSE To detect pathologies in coronary arteries by computed tomography angiography (CTA) in survivors of childhood Hodgkins lymphoma who have been treated with radiotherapy and/or cardiotoxic agents. PATIENTS AND METHODS Patients with HL who have been in remission for at least 2 years after completion of therapy were included. CBC, lipid profile, urine analysis, brain natriuretic peptide, troponin-T, creatinine kinase-myocardial band, ECG, telecardiography, echocardiography, and CTA of the patients were performed. Cardiac vascular abnormalities were noted. Results A total of 119 patients were included in the study. In 19 patients (16%), we found coronary artery abnormalities. There was a significant difference between the patients who received mediastinal radiotherapy and those who did not (P = .02). By multivariate analysis, in patients receiving mediastinal radiotherapy the risk of developing a coronary artery abnormality was found to increase 6.8 times compared with patients who did not receive mediastinal radiotherapy (P = .009). Stent implantation was performed in a 28-year-old patient because of critical stenosis in right coronary. In two patients some irregularities were detected both in CTA and conventional angiography, and they remained in close follow-up. A 22-year-old patient whose CTA showed critical stenosis in his left anterior descending artery refused the conventional angiography. CONCLUSION To our knowledge, this is the first large study using CTA for detection of coronary abnormalities in patients treated for HL in pediatric age group. Coronary CTA is a minimally invasive tool for early diagnosis of coronary artery disease in patients who were treated with mediastinal radiotherapy and/or cardiotoxic chemotherapy.

Treatment results of 84 patients with nasopharyngeal carcinoma in childhood

To evaluate the clinical characteristics, treatment regimens, survival, and late complications in patients with nasopharyngeal carcinoma.

Second neoplasms in pediatric patients treated for cancer: a center's 30-year experience.

To investigate the incidence and outcome of secondary neoplasms in pediatric patients treated for childhood cancer. Between December 1971 and January 2000, a total of 5859 patients younger than age 17 were diagnosed and treated for childhood cancers in our center. Of this group, 1511 (36%) patients were followed for more than 36 months. These long-term survivors were included in this analysis. Twenty-six patients developed a secondary malignancy with an overall risk of 1.7% in this cohort. The male:female ratio was 17:10, with a median age of 7.66 at diagnosis (range, 2 to 16 y). Four patients (14.8%) with Hodgkin lymphoma; 3 each (11.1%) with retinoblastoma and rhabdomyosarcoma; 2 each (7.4%) with Wilms tumor, Ewing sarcoma, medulloblastoma, ganglioneuroblastoma, and non-Hodgkin lymphoma; and 1 each (3.7%) with ependymoma, nasopharyngeal carcinoma, osteosarcoma, astrocytoma had a secondary malignant disease during the long-term follow-up period. Secondary malignant diseases were osteosarcoma in 6 patients, acute lymphoblastic leukemia in 2, acute myelogenous leukemia in 2, and rare malignant disease in others. Four patients with osteosarcoma developed disease within the radiation field. Osteosarcoma was the most frequently occurring secondary neoplasm. Less toxic treatment modalities should be used to decrease the risk of secondary malignant diseases.

Childhood cancer and hypercalcemia: Report of a case treated with pamidronate

Hypercalcemia in a 4-year-old boy with non-Hodgkin lymphoma treated with pamidronate is presented. The child had relapsed disease with bone metastasis. Hypercalcemia is rare in children, and bisphosphonates are relatively new agents for the treatment of hypercalcemia. Information concerning their use in the treatment of hypercalcemia in childhood is limited. We found that pamidronate is effective and has no significant side effects in a child.

Wilms' Tumor in Children : An Overview

Wilms’ tumor is the most frequently occurring renal tumor in children and is one of the most treatment-responsive tumors. A tumor-suppressor gene and other genetic abnormalities have been implicated in its etiology. In addition, patients with several congenital anomalies, such as Beckwith-Wiedemann syndrome, WAGR syndrome, and Denys-Drash syndrome, have an increased risk of Wilms’ tumor. Previously, a three-drug chemotherapy regimen with surgery and radiotherapy was used with patients in all stages. Now, patients with early-stage Wilms’ tumor are treated with a two-drug regimen without radiotherapy, whereas those in advanced stages still receive the three-drug regimen and radiotherapy. Two large collaborative groups – the National Wilms’ Tumor Study Group (NWTS) in the United States and the International Society of Pediatric Oncology (SIOP) in Europe – are involved in Wilms’ tumor management, which differs in some aspects. Multimodality treatment has been used successfully, and in Europe preoperative strategies are used as well. As the survival rate has now reached 90%, the primary objectives of the physician are to perform nephron-sparing surgery in selected cases and to reduce the dosage and duration of chemotherapy and radiotherapy in appropriate cases. Other renal tumors occur rarely, but have also been treated successfully in the last decade.

Treatment of a tongue lymphangioma with sirolimus after failure of surgical resection and propranolol

Lymphangiomas of the tongue are rare, and their treatment is problematic. A 10 year‐old patient with tongue lymphangioma who was previously treated with surgery and propranolol with no response was treated with sirolimus in our department. We used sirolimus with a dose of 1.6 mg/m2/day. After 3 months of treatment, the mass had decreased by more than 60%. We continued the treatment for 1 year with a maximum response of 70% decrease in mass. Disease remained stable 6 months after stopping therapy, the latest time of follow‐up. Sirolimus appears to be effective in lymphangioma but requires further study. Pediatr Blood Cancer 2014;61:931–932.

Langerhans cell histiocytosis: retrospective analysis of 217 cases in a single center.

Langerhans cell histiocytosis (LCH) is a disorder with unclear etiology and pathogenesis, which is characterized by abnormal clonal proliferation and accumulation of langerhans cells at various tissue and organs. A total of 217 patients with LCH were evaluated retrospectively for clinicopathological features, laboratory findings, treatment modalities, long-term outcome, and factors affecting the outcome. Median age at the time of diagnosis was 3.5 years and male/female ratio was 1.8. The most common complaint at presentation was a bone lesion-related symptom. Fifty percent of the patients younger than 2 years had organ dysfunction (OD). Treatment consisted of surgery, chemotherapy, and radiotherapy alone or in combination. Vinblastine with or without prednisolone was the most common used chemotherapy regimen. Overall (OS) and event-free survival (EFS) rates were 84% and 51.5%, respectively, at an 8-year median follow-up time. Overall survival was significantly lower in patients younger than 2 years of age and patients with OD. The age at diagnosis, pulmonary, liver, or hematological involvement, and elevated acute-phase reactants were found to have a statistically significant effect on the OS or EFS rates.

Evaluation of thrombotic children with malignancy

The purpose of this study was to evaluate inherited and acquired prothrombotic risk factors among children with malignancies who have thrombosis and emphasize the importance of inherited prothrombotic risk factors. Thirty-seven consecutive children with thrombosis and malignancy were included in this study. The patients were evaluated separately for time of development of thrombosis, insertion of a central venous line (CVL), history of l-asparaginase usage, and recent infections. Prothrombotic risk factors such as factor V G1691A and prothrombin G20210A mutation, protein C, protein S, antithrombin III deficiencies, factor VIII and lipoprotein(a) elevation, and antiphospholipid antibodies were analyzed for all patients. Of 387 children with thrombosis, 37 (9.5%) had a malignancy. Thrombosis was detected in 9 patients at the time of diagnosis, during maintenance therapy in 25 patients, and after the discontinuation of treatment in 3 patients. One or two additional prothrombotic risk factors other than l-asparaginase therapy and insertion of central venous lines were present in 20 of these patients (54%). It was found that eight patients had the factor V G1691A mutation in the heterozygote state. One of them had the factor V G1691A mutation associated with a history of infection and one patient had the factor V G1691A mutation associated with factor VIII elevation. One had the the prothrombin G20210A mutation in the heterozygote state, four had lipoprotein(a) elevation, two had factor VIII elevation, one had a decreased protein S level, one had a decreased protein C level, one had antiphospholipid positivity, and two had histories of infection. Malignancy is an important risk factor for the development of childhood thrombosis. However, the risk of thrombosis increases when accompanied by additional prothrombotic risk factors. For this reason, especially children with malignancy and at high risk for the development of thrombosis, such as those who have received l-asparaginase or a replaced CVL during their therapy, might be screened for additional prothrombotic risk factors and appropriate measures might be taken to prevent the development of thrombosis.

Prognostic importance of magnetic resonance imaging in bone marrow involvement of Hodgkin disease.

BACKGROUND Determination of bone marrow involvement is important in staging Hodgkin disease (HD), so we compared the effectiveness of magnetic resonance imaging (MRI) with bone marrow biopsy in diagnosing bone marrow involvement in HD patients. PROCEDURE Twenty-six patients with the diagnosis of HD were included in this study. The ages of the patients were between 4 and 24 years, with a median of 12. Eleven of them had stage III or IV disease and 15 had been previously diagnosed as having HD and were in relapse. They were evaluated by bone marrow biopsy and MRI of lumbar vertebrae. The biopsies were taken from the anterosuperior iliac spine with an age-appropriate Jamshidi biopsy needle. Within 14 days following biopsy, MRI of lumbar vertebrae was carried out. RESULTS MRI revealed decreased signal intensity in T1-weighted images in 7 of 26 patients. On the other hand, bone marrow biopsies showed HD involvement in three out of seven patients. The remaining 19 patients who had normal bone MRI were negative for HD in their bone marrow biopsies. The patients with positive MRIs and negative biopsy for HD had bone pain. One of them had a femoral periosteal reaction on bone survey; the other two had height loss in their lumbar vertebral bodies. There was a statistically significant difference in the disease-free survival rates between MRI-positive and -negative patients in the following 24 months period (P < 0.0001). CONCLUSIONS This study suggests that MRI is a useful method for diagnosing bone marrow involvement in HD, in that our MRI-positive patients had a higher relapse rate in the 24 months follow-up period than the MRI-negative patients.

Hypertrophic osteoarthropathy in a child with nasopharyngeal carcinoma

Abstract We report a 13-year-old boy with nasopharyngeal carcinoma, skull metastases and hypertrophic osteoarthropathy. Although the metastases and the primary tumour responded well to chemotherapy, hypertrophic osteoarthropathy persisted during follow-up.