Fatma Betul Cakir

A Modified Protocol with Vincristine, Topotecan, and Cyclophosphamide for Recurrent/Progressive Ewing Sarcoma Family Tumors

Purpose: Topotecan has recently been used in the treatment of pediatric cancer. We evaluated our experience with the modified combination of vincristine, topotecan, and cyclophosphamide (VTC) given in 3 days, in children with recurrent Ewing sarcoma. Method: Children received vincristine (1.5 mg/m2/1st day), cyclophosphamide (600 mg/m2/day × 2 days) + mesna, and topotecan (1 mg/m2/day × 3 days) every 21 days. Result: A total of 118 courses of VTC were given to 13 patients. One patient received VTC both at first and at second relapse. Thus, 14 relapse episodes in 13 patients were evaluated. After three courses of VTC chemotherapy (CT), two achieved complete response (CR), five achieved partial response, thus an objective response was attained in 7/14 (50%) episodes. Two patients had stable disease and two patients progressed. In three episodes, CR was achieved by surgery before CT. One of them had a second relapse and attained CR with VTC. Median time from diagnosis to relapse was 23 months (5–45 months). Site of relapse was local in four patients, and metastatic in 10 episodes of nine patients. Seven patients are alive, three with no evidence of disease and four alive with disease; six have died of disease. Local treatment was used in 11 episodes. The toxicity of the VTC combination was limited mainly to the hematopoietic system. Conclusion: In conclusion, the modified VTC protocol in 3 days every 3 weeks seems to be effective and tolerable in children and adolescents with recurrent/progressive Ewing sarcoma.

Chronic Eosinophilic Pneumonia With Mucous Plugs in a Child

Chronic eosinophilic pneumonia is a rare cause of chronic lung disease in children. A 7‐year‐old girl who attended our clinics with cough and sputum lasting for 5 years, has been evaluated for bilateral alveolar infiltration and ground‐glass opacities. Peripheral eosinophilia was detected in total cell blood count. Flexible bronchoscopy showed mucous plugs. Bronchoalveolar lavage fluid and cell block of mucous plugs determined hypereosinophilia. Chronic eosinophilic pneumonia was confirmed after the elimination of other eosinophilic lung diseases and the case was accepted to be idiopathic. She showed a dramatic response to oral corticosteroids. This is the first reported case of chronic eosinophilic pneumonia presenting with mucous plugs in children described to date in the literature. Pediatr Pulmonol. 2010; 45:1040–1042.

Invasive respiratory aspergillosis is a treatable disease with early diagnosis and aggressive therapy.

This study aimed to document outcome of invasive respiratory aspergillosis (IRA) in pediatric malignancy patients. Patients with febrile neutropenia episodes followed between January 2003 and May 2007 were enrolled. Antifungal therapy was added to those who were still febrile on the 5th day of febrile neutropenia treatment. Patients were screened with computerized tomographies. IRA was identified in 22 of 98 patients. There were 13 males and the mean age was 97 months. Proven infection was established in 3, probable in 7, and possible in 12 patients. Liposomal amphotericin B was administered to all patients and was successful in 10 patients. Modifications with caspofungin or voriconazole were done in liposomal amphotericin B failures. The median duration of antifungal therapy was 5.5 months. The median follow-up time was 29 months. There was no evidence of IRA in 12 patients after completion of cancer chemotherapy. Six patients died due to underlying disease, whereas IRA was either in remission or stable disease. Four patients were lost due to IRA. The remission rate for IRA was 82%. Survival at 37 months was 55% (95% confidence ınterval 25–47 months). The amount of time that absolute neutrophil count after initiation of treatment for IRA remained at zero was found to be an independent prognostic factor on survival (P = .01). These results suggest that early diagnosis and aggressive treatment may increase the successful outcome of IRA.

Endobronchial findings of hydatid cyst disease: A report of five pediatric cases†

Hydatid disease is still an important public health problem throughout the world. Diagnosis of the disease is generally based on clinical and radiological findings. Evaluation of pulmonary disorders by flexible bronchoscopy (FOB) is a rapidly developing facility, but diagnostic and therapeutic FOB for pulmonary hydatid cysts is still controversial. This study examines the findings of endobronchial hydatid cyst disease in five pediatric patients from Turkey, and clinical experience about this subject is reviewed. All our patients presented with unusual symptoms of the disease, and for all of them, diagnosis had been delayed using current diagnostic methods. As a result of our experience, it can be reported that the endobronchial appearance of the hydatid cyst membrane is whitish‐yellow, and it is difficult to differentiate it radiologically from some other common causes of endobronchial lesions in childhood, such as endobronchial tuberculosis, foreign body aspirations, mucous plaques, and granulation scars. The findings of these cases show that, hydatid cyst should also be kept in mind in differential diagnosis of endobronchial lesions. In the diagnosis of pulmonary hydatid cyst in children without typical clinical and radiological findings of the disease, FOB examination is a valuable diagnostic procedure. Pediatr Pulmonol. 2012; 47:706–709.

Interferon-α for unresectable progressive and symptomatic plexiform neurofibromas.

Neurofibromas are the most common manifestations of neurofibromatosis type-1. They occasionally cause pain or progressive loss of function due to nerve compression. Optimal treatment approach is still challenging and the current treatment results are not satisfactory. Four cases of plexiform neurofibromas with various clinical presentations and an addendum to a previously published report on a patient who had relief from pain and/or regression of tumor volume after treatment with interferon-&agr; 2a are presented.

Cancer Care in a Country Undergoing Transition: Turkey, Current Challenges and Trends for the Future

Cancer and cancer care are increasingly recognized as a major worldwide challenge given their global, financial, social, and health implications. The prevalence of cancer, primarily a disease of aging, is increasing with the increasing life-span and the adaptation of Western lifestyle in both high and low income countries. Of the 14 million people diagnosed with cancer worldwide in 2012, more than 60 % live in low and middle income countries (LIC/MIC). 70 % of the cancer deaths worldwide occur in LIC/MIC as well. Global cancer incidence is predicted to reach 25 million by 2030, 70 % of which is expected to be reported in developing countries (J Oncol Pract 7(5):324–329, 2011). Turkey, according to the World Bank’s classification for income is considered as an upper middle income country. Turkey is located on two continents Europe and Asia, located at the crossroads of East and West; it also has parts in the Mediterranean and the Middle East. At the beginning of the millennium, the performance of Turkey’s health system in terms of public health, insurance of the patients, and the patient satisfaction was at the bottom of the Organisation for Economic Co-operation and Development (OECD) countries and in the European Region of World Health Organization (WHO) (Health Syst Transit 13(6):1–186, 2011). Turkey has undertaken the Health Transformation Program (HTP) from 2002 on, including major reforms to transfer and improve the health system and its outcomes and the situation has improved in the following decade.

Nontuberculous pulmonary cavitary diseases of childhood.

We describe the demographic, clinic and radiologic features of nontuberculous cavitary pulmonary diseases in 42 patients with a mean age of 91.1 ± 6.8 months. Infectious etiology was the most common cause (64%), including necrotizing pneumonia (n = 15), ruptured hydatid cyst (n = 5), lung abscess (n = 5) and fungal infection (n = 2). Other causes were bronchiectasis, congenital anomalies, foreign body aspiration, sarcoidosis and tumor.

A Difficult Case of Hodgkin Lymphoma with Differential Diagnosis of Tuberculosis and Sarcoidosis

We report here the case of a 14-year-old boy with history of fever, weight loss, and mediastinal lymphadenopathy. The clinical symptoms and laboratory findings mimicking tuberculosis and sarcoidosis complicated the diagnostic process. He was diagnosed with Hodgkin’s lymphoma after several X-rays, computed tomography, positron emission tomography-computed tomography, laboratory tests and three lymph node biopsy. Clinicians should be alerted on new lesions and symptoms in high risk patients and should repeat diagnostic tests and lymph node biopsies as indicated.

Vincristine, irinotecan, and temozolomide treatment for refractory/relapsed pediatric solid tumors: A single center experience:

Background Although the survival of pediatric cancer has increased dramatically in the last decades, the survival of refractory, relapsed, and metastatic cases is still dismal. The combination of irinotecan and temozolomide has shown activity against refractory/relapsed pediatric solid tumors. Method Thirty-four children with refractory/relapsed solid tumors who had previously been heavily pretreated and who were given vincristine, irinotecan, and temozolomide as third- or further line chemotherapy during 2004–2015 were evaluated. Results Patients were diagnosed with Ewing sarcoma (n = 15), rhabdomyosarcoma (n = 8), neuroblastoma (n = 8), osteosarcoma (n = 2), and Wilms’ tumor (n = 1). Thirty patients presented with disease progression on therapy and the other four presented with relapsing. A total of 141 cycles were administered. Radiotherapy was used in 17 patients and surgery in 4 as local therapy. Among all patients, 6 had complete response, 3 had partial response, 14 had stable disease, and 11 had progressive disease. The objective response was 26.4% (complete response + partial response) and median survival duration was six months. The first and second year overall survival rates were 22.3% and 16.8%. The objective response in Ewing sarcoma patients was 40%. Diarrhea was the most common toxicity and 14 (10%) courses were associated with grade 3–4 diarrhea. Conclusions In heavily pretreated patients with refractory/relapsed solid tumors, the vincristine, irinotecan, and temozolomide regimen seemed promising in Ewing sarcoma patients and was well tolerated.

Effects of Malnutrition on Neutrophil/Mononuclear Cell Apoptotic Functions in Children with Acute Lymphoblastic Leukemia

ABSTRACT Background: Recent studies claim that apoptosis may explain immune dysfunction observed in malnutrition. Objective: The objective of this study was to determine the effect of malnutrition on apoptotic functions of phagocytic cells in acute lymphoblastic leukemia (ALL). Materials and Methods: Twenty-eight ALL patients (13 with malnutrition) and thirty controls were enrolled. Neutrophil and mononuclear cell apoptosis of ALL patients and the control group were studied on admission before chemotherapy and repeated at a minimum of three months after induction of chemotherapy or when the nutritional status of leukemic children improved. Results: The apoptotic functions of both ALL groups on admission were significantly lower than those of the control group. The apoptotic functions were lower in ALL patients with malnutrition than those in ALL patients without malnutrition, but this was not statistically significant. The repeated apoptotic functions of both ALL groups were increased to similar values with the control group. This increase was found to be statistically significant. Conclusions: The apoptotic functions in ALL patients were not found to be affected by malnutrition. However, after dietary intervention, increased apoptotic functions in both ALL patient groups deserve mentioning. Dietary intervention should always be recommended as malnutrition or cachexia leads to multiple complications. Enhanced apoptosis might originate also from remission state of cancer.