Correspondence: treatment of the neuroblastoma-associated opsoclonus-myoclonus-ataxia (OMA) syndrome with high-dose methylprednisolone.
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Anti-Inflammatory Agents↗
Biomedical subjects
Canan Akyüz
Publications and source records attributed to Canan Akyüz.
Acute blindness as a presenting sign in childhood non-Hodgkin lymphoma.
Non-Hodgkin lymphoma is the third most common malignancy in childhood. Occasionally, a patient with lymphoma may present with neuroophthalmologic symptoms and signs. It is usually associated with 3rd, 4th, and 6th cranial nerve palsies. Here, we report three children presenting with loss of vision. Two of them were cured with modified LMB-89 (lymphoma malignant B) chemotherapy regimen and radiotherapy. In one patient, loss of vision returned to normal in the left eye but persisted in the right eye. The second patient suffered from permanent visual loss, although the primary disease responded well to treatment. Unfortunately, the third patient died at an early phase of treatment. Acute blindness as a presenting symptom is rare in lymphoma. Early diagnosis and treatment is critical to increase the survival rate and to improve the quality of the vision.
Acute Disease↗
High-dose methylprednisolone treatment of hepatic veno-occlusive disease in a child with Wilms tumor.
Veno-occlusive disease (VOD) is a rare complication of chemotherapy in children with cancer. In the literature successful treatment of severe VOD has not been well determined. The authors report a 5-year-old boy with Willms tumor whose condition was complicated with VOD while receiving chemotherapy. He was treated successfully with high-dose methylprednisolone. After therapy, the clinical signs of VOD, such as abdominal ascites, oliguria, and jaundice, disappeared without any adverse effects of therapy.
Antineoplastic Combined Chemotherapy Protocols↗
Is epoetin alfa a treatment option for chemotherapy-related anemia in children?
BACKGROUND: The efficacy and safety of epoetin alfa in ameliorating cancer- or chemotherapy-related anemia and reducing red blood cell (RBC) transfusion requirements have been demonstrated in numerous trials in adult patients. However, limited information is available about recombinant human erythropoietin (rHuEPO, epoetin alfa) as a treatment option in pediatric cancer patients. PROCEDURE: To gain more information about the efficacy and safety of epoetin alfa in the treatment of chemotherapy-induced anemia in children with solid tumors receiving either platinum- or nonplatinum-containing chemotherapy, an 8-week randomized trial was conducted. Epoetin alfa 150 IU/kg was given 3 times a week for 8 weeks to 17 patients; 17 control patients received standard of care. RESULTS: Transfusions, administered if the hemoglobin (Hb) level dropped to below 6 g/dL, were necessary for only one patient in the epoetin alfa group, as compared with eight patients in the control group (change in Hb from 8.5-10.21 g/dL in the epoetin alfa group vs. 8.48-8.41 g/dL in the control group). CONCLUSIONS: The data from this study suggest that this dosing regimen of epoetin alfa is effective and safe in pediatric cancer patients with chemotherapy-related anemia. Further studies with epoetin alfa in more children with different chemotherapy regimens are needed.
Adolescent↗
Myeloprotective effect of short term prechemotherapy recombinant granulocyte-macrophage colony stimulating factor.
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Antineoplastic Combined Chemotherapy Protocols↗
Value of surveillance imaging in the management of medulloblastoma.
BACKGROUND: To investigate the value of surveillance scanning for the detection of recurrences in medulloblastoma. PROCEDURE: The charts of 95 patients with medulloblastoma were retrospectively reviewed. Information regarding the patient characteristics, treatment modalities, dates, types and results of CT and MRI studies, the frequency with which recurrences were identified on surveillance images, changes in patient management, outcome of the patients following recurrences, and survival data were analyzed. RESULTS: Thirty-one patients had a recurrence of tumor in the central nervous system; none experienced extraneural relapses. Of all recurrences, 21 were symptomatic and 10 were discovered by surveillance scans asymptomatically. None of the patients with a recurrence survived. For all 95 patients, 5-year overall and event-free survival rates were 47.1 and 49.8%, respectively. In patients with symptomatic and asymptomatic recurrences, the mean time to recurrence since initial diagnosis, the mean duration of survival post-recurrence, and the mean duration of overall follow-up were 19.2 and 26.1 months, 3.6 and 8.0 months, and 22.8 and 34.1 months, respectively. For 95 patients, 468 surveillance and 38 symptomatic images were reviewed as 313 CTs and 193 MRIs. Rate of diagnosis of recurrence per surveillance image was 2.1% (10/468). CONCLUSIONS: In our study, surveillance scanning brought no survival advantage since it detected a minority of recurrences. Longer survival achieved by early detection of recurrences might be a reflection of lead-time and length biases. Surveillance procedures will gain more importance as new effective therapeutic options are developed for recurrent medulloblastoma.
Adolescent↗
Hodgkin's disease and ataxia telangiectasia with pulmonary cavities.
Ataxia telangiectasia (AT) homozygotes have an increased risk for development of Hodgkin's disease (HD). Parenchymal lung involvement is not uncommon in HD; however, cavitary pulmonary lesions are quite unusual. We report on 3 cases of AT with HD who had mediastinal disease and parenchymal pulmonary involvement with cavitation. Of 6 AT patients in our HD series, 3 developed pulmonary cavities. The patients displayed pulmonary infiltration, cavitation in the lung parenchyma, and mediastinal enlarged lymph nodes on both plain chest X-rays and thoracic computed tomographies. No infectious etiologies were established for the pulmonary findings. Histopathological examination of open lung and mediastinal biopsies revealed HD, and all patients received multiagent chemotherapies. The outcome was fatal in all 3 patients. Respiratory infections are the principle cause for morbidity and mortality in AT patients. Reports on cavitating pulmonary lesions in HD are quite rare. Furthermore, data regarding the patterns of pulmonary involvement in AT patients with or without HD are lacking. The increased incidence of malignancies in AT patients may relate to immunodeficiency and to the chromosomal alterations identified.
Ataxia Telangiectasia↗
Involvement of the thyroid and the salivary glands in childhood non-Hodgkin's lymphomas at initial diagnosis.
OBJECTIVE: The clinical spectrum of childhood non-Hodgkin's lymphoma (NHL) is different from that in adults, with frequent extranodal involvement. We searched the incidence and radiological appearance of the secondary involvement of the thyroid and salivary glands by ultrasonography (US) to assess its importance for staging of the disease. MATERIALS AND METHODS: We examined the thyroid, parotid and submandibulary glands of 43 children with NHL, age ranging between 9 months and 17 years, by US. RESULTS: According to US findings, involvement of the parotid gland was very common as detected in 18 of the cases with NHL (41%). It was the most common organ involved in lymphoma. Lymphomatous infiltration of the thyroid gland, with bilateral multiple focal lesions, was detected in two patients and proved by fine needle aspiration biopsy in all cases (5%). Salivary gland and thyroid involvement changed staging in three (6%) of the patients, however, the treatment plan was unchanged. CONCLUSIONS: This study shows that anatomic sites, known to be very rarely involved at the initial presentation of the disease is actually more commonly involved in childhood NHL. However, it does not cause any significant change in the treatment planning or in the survival.
Adolescent↗
Renal involvement in multifocal osteosarcoma.
A rare case of multifocal osteosarcoma metastatic to kidney, lung and lymph nodes is presented. Radiological findings included multiple foci of sclerotic lesions involving the axial and peripheral skeleton, a heterogeneous renal mass with areas of calcification, multiple pulmonary metastases with pneumothorax, and partially calcified enlarged mediastinal and abdominal lymph nodes.
Bone Neoplasms↗
The comparison of antibody response to different hepatitis b vaccines with and without pre-S2 antigen in children with cancer.
Children with cancer are at an increased risk of hepatitis B infection and chronic liver disease. Since hepatitis B vaccines containing pre-S2 antigen has been recently reported as being more efficient in providing immunization in healthy individuals, the authors compared antibody response to pre-S2-containing vaccine with no-pre-S2-containing hepatitis B vaccine, when given in double doses to 100 children receiving chemotherapy. Patients, aged 1 to 16 years with negative HBV serology, were vaccinated with 2 different types of HBV vaccines between 1997 and 1999. Group 1 received Gen Hevac B containing pre-S2 (n = 41) in a dose of 20 microg for patients younger than 10 years old and 40 microg for older patients. Group 2 was vaccinated at the same dose with hepatitis B vaccines not containing pre-S2 antigen. All vaccinations were repeated at 0, 1, and 6 months. Serum samples were drawn for determination of anti-HBs titers at 1, 3, 6, and 8 months. After the third dose of vaccine, the seroconversion rate was 72% in group 1 and 62% in group 2. The anti-HBs levels were higher in the group receiving pre-S2-containing hepatitis B vaccine. However, the difference between groups was not statistically significant (p > .05). The administration of pre-S2-containing hepatitis B vaccines may give a better seroconversion and higher antibody response to vaccination in children with cancer. But a further large-scale study is needed to confirm this finding.
Adolescent↗
Clinical characteristics and treatment results of LMB/LMT regimen in children with non-Hodgkin's lymphoma.
Lymphomas are the second most common cancers after leukemias seen in children in Turkey, although they rank third in many western countries. Ninety-seven patients with newly diagnosed, untreated non-Hodgkin's lymphoma, between April 1994 and December 1997, were included in this study. Modified lymphoma malign B (LMB) and lymphoma malign T (LMT) regimens were used for treatment of B- and T-cell disease, respectively. Ten (10.3%), 68 (70.1%), and 19 (19.6%) patients had stage II, III, and IV disease, respectively. Forty-eight, 19, 15, 9, 5, and 1 patients had tumors at abdominal, mediastinal, disseminated, head and neck, extranodal, and peripheral nodal locations, respectively. Seventy-two patients were treated with LMB89 regimen and 25 were treated with LMT89 regimen. Thirty-four patients had tumor lysis at diagnosis, and 9 patients required dialysis. Objective response rates were 75% for patients treated by LMB regimen and 92% for those treated by LMT regimen. Two-year overall survival rates were 90, 66.1, and 50.8% for patients with stage II, III, and IV disease, respectively. Two-year overall survival rates were 64.2% for LMB-treated patients and 70.8% for LMT-treated patients. Poor response at the end of cytoreductive treatment and age younger than 4 years were poor prognostic factors. Pediatric lymphomas could be treated safely and effectively by LMB and LMT regimens.
Adolescent↗
Serum carnitine levels of pediatric cancer patients.
Secondary carnitine deficiency may occur in some diseases including malnutrition. The present study examined the serum carnitine levels of children with cancer and their relationship with nutritional status. Fifty-one (mean age: 104 +/- 23 months) patients with cancer were evaluated at diagnosis and 3 months after the initiation of the treatment. There were significant differences between the mean carnitine levels of patients before and during the treatment (p: .004). Although initial carnitine levels of patients were similar to those in control groups, there was a significant dif ference between the mean carnitine levels of patients and those of the control group at the third month (p: .02). The prevalence of malnutrition at the third month of the treatment (43%) was higher from the prevalence at diagnosis (33%) but this was not significant. No significant relationship was found between carnitine levels and nutritional status of patients either at diagnosis or during treatment. These results showed that inadequate intake of carnitine or its precursors could not be responsible for decrease in carnitine levels. Metabolic changes that result from therapy and/or from neoplastic process may be responsible for the decrease in carnitine levels. Detailed studies, including measurements of fractions of carnitine and urinary carnitine excretion according to individual drug, are necessary to determine real cause of this decrease.
Adolescent↗
Malignant solid tumors associated with congenital immunodeficiency disorders.
This objective of this study was to evaluate patients with immunodeficiency syndromes who had developed malignant solid tumors and to examine survival rates and prognosis with respect to type of immunodeficiency disease. Twenty-two patients who were diagnosed with malignant solid tumors and immunodeficiency syndromes between January 1972 and February 2003 were analyzed retrospectively. There were 12 (55%) patients with non-Hodgkin lymphoma, 8 (37%) with Hodgkin disease, 1 (5%) with mucinous adenocarcinoma of the colon, and 1 (5%) with brain stem glioma. Fifteen (68%) patients had ataxia-telangiectasia, 3 (14%) had common variable immunodeficiency disease, 2 (9%) had Bloom syndrome, 1 (5%) had combined immunodeficiency, and 1 (5%) had selective immunoglobulin A deficiency. Out of the 15 patients with ataxia-telangiectasia 9 patients had non-Hodgkin lymphoma, 5 had Hodgkin disease, and 1 had brain stem glioma. Two patients with common variable immunodeficiency disease had non-Hodgkin lymphoma and 1 had Hodgkin disease. One of the patients with Bloom syndrome had Hodgkin disease and 1 had colon carcinoma. The overall survival for the whole group was 24%. Overall survival rates in non-Hodgkin lymphoma, Hodgkin disease, colon carcinoma, and brain stem glioma were 17, 44, 0, and 0% (p =.25), respectively. Overall survival in ataxia-telangiectasia patients was 20%. In this series, most of the patients had ataxia-telangiectasia (68%). The survival rates of the malignant diseases were very poor in immunodeficiency. Overall survival in non-Hodgkin lymphoma patients was relatively worse than Hodgkin disease patients.
Adolescent↗
Treatment results and prognostic factors in Ewing sarcoma.
Files of 133 children with Ewing sarcoma (median age 10 years) were reviewed. Frequent primary sites were extremities, trunk, pelvis, and cranium. Half of 43 patients with metastases had disease in the lungs. Ten-year overall and event-free survival rates were 31% and 19%, respectively. Five-year overall survival rates were 42% in localized and 15% in metastatic disease (p < .0001); 66% in cases with primary tumors < 8 cm and 29% in larger tumors (p = .013). VAC (vincristine, actinomycin D, and cyclophosphamide) regimens with anthracyclines resulted in better survival. Presence of distant metastases, large primary tumors, and pelvic localization were related to poor prognosis. Novel therapeutic approaches are needed to produce better results, especially in high-risk patients.
Adolescent↗
Intracranial involvement in Hodgkin's disease.
The authors report 3 cases of Hodgkin's disease with intracranial involvement. The patients were 4, 12, and 15 years old (male/female=1/2). Initially, they were treated with ABVD or COPP chemotherapies and low-dose involved field radiotherapy. Intracranial recurrences occurred 27, 40, and 42 months after initial diagnosis, respectively. Two patients experienced convulsions and the other complained of diplopia. The metastatic lesions were located supratentorially with CT or MRI. Despite initial response achieved following systemic chemotherapy and external irradiation to cranial lesions, all patients died with disseminated disease. In patients with intracranial involvement of Hodgkin's disease, prolonged disease-free survival may be achieved by combined modality treatment.
Adolescent↗
Isolated breast involvement at recurrence in a child with T-cell lymphomas.
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Adolescent↗
Solid tumors in the neonatal period.
From 1972 to 2000, 123 patients with solid tumors whose complaints had started in the first 28 days of life were retrospectively evaluated. Fifty-five patients were diagnosed in the first 28 days and 68 patients were diagnosed after 28 days. In the former group, 85.5% of patients had symptoms in the first day of life. In the latter group, 77.9% had the onset of symptoms in the first day. Tumor subgroups in the neonatal period included teratoma (34), neuroblastoma (11), rhabdomyosarcoma (3), Wilms tumor (1), and retinoblastoma (3), and the others (3). Three patients had other, less common tumors. In the second group the numbers were the following: for teratoma (32), neuroblastoma (15), germ cell tumors other than teratomas (8), rhabdomyosarcomas (4), the other soft tissue sarcomas (3), Wilms tumor (1), retinoblastoma (1), and other, rare tumors (4). There were 22 malignant tumors in the first group, and 44 in the second group. Fourteen patients in the first group died in the early postoperative period or with progressive disease. Nineteen of 44 patients died in the second group. Overall survival rates were 24.9% and 51.6% in first and second groups, respectively (p = 0.015). Event-free survival rates were 14.7% and 47.7% in these groups, respectively (p = 0.0063). This is the first report comparing clinical features and prognosis of tumors diagnosed in the first 28 days of the life with those diagnosed after 28 days. The prognosis was worse in infants diagnosed in the first 28 days of life.
Age of Onset↗
Nutritional status of children with cancer and its effects on survival.
In the present study we aimed to determine the nutritional status of our patients and to assess its relationship with survival. The nutritional status of 47 patients with cancer was evaluated at diagnosis, three months after initiation of the treatment and at the end of therapy. Weight for height, height for age, and weight for age of children were expressed as percent of standard. Values for each nutritional index were converted into standard deviation (Z) scores. Three-year overall survival (OS) and event-free survival (EFS) rates of patients were determined according to their nutritional status. The overall prevalence of malnutrition at diagnosis was 29.8%. Three months later the malnutrition ratio reached 38.3% and then decreased again to 18.5% at the end of the therapy. Although the prevalence of malnutrition at the third month of treatment was significantly higher from the prevalence at diagnosis (p: 0.001) and at the end of the therapy (p: 0.009), the mean Z scores of the nutritional indexes before and during the treatment were not significantly different. The survival rates of malnourished patients were not different from those of well nourished patients. In conclusion, malnutrition is one of the main problems in children with cancer; hovewer, nutritional status has no effect on survival.
Adolescent↗