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Purpose: This study evaluated the results of polidocanol sclerotherapy in the treatment of venous malformations (VM) including patient satisfaction, perceived improvement, and predictors of satisfaction., Material and Method: Patients with VM that underwent polidocanol foam sclerotherapy between June 2013 and July 2021 in a single center were retrospectively evaluated. Patient demographics, VM, and treatment characteristics were analyzed. Patient-reported outcomes and satisfaction were analyzed with a questionnaire., Results: This study included 232 (136, 58.6%, female) patients. The mean age was 24.49 ± 12.45 years (range 3-72). The clinical response rate was 82.3%. The rate of satisfaction was 82.3%, and 116 (50%) patients were significantly satisfied. There were no major complications. Clinical response and VM margin were related to satisfaction ( p < 0.01, p = 0.012, respectively). Clinical response to pretreatment swelling was related to significant satisfaction ( p = 0.02)., Conclusion: Polidocanol sclerotherapy was safe and effective in VM treatment with high satisfaction and low complication rates.

Background: Turkey hosts the world's largest refugee population of whom 3.5 million are Syrians and this population has been continuously growing since the year 2011. This situation causes various problems, mainly while receiving health-care services. In planning the migrant health-care services, for the policy makers of host countries, health literacy level of migrants is an important measure. Determination of health literacy level of Syrian refugees in Turkey would be supportive for planning some interventions to increase health-care service utilization, as well as health education and health communication programs. An "original health literacy scale" for 18-60 years of age Turkish literate adults (Hacettepe University Health Literacy Scale-HLS) was developed to be used as a reference scale in 2018. Since it would be useful to compare the health literacy levels of Turkish adults with Syrian adult refugees living in Turkey with an originally developed scale, in this study, it was aimed to adapt the HLS-Short Form for Syrian refugees., Methods: This methodological study was carried out between the years 2019-2020 in three provinces of Turkey where the majority of Syrians reside. The data was collected by pre-trained, Arabic speaking 12 interviewers and three supervisors via a questionnaire on household basis. At first, the original Scale and questionnaire were translated into Arabic and back translated into the original language. The questionnaire and the Scale were pre-tested among 30 Syrian refugees in Ankara province. A total of 1254 refugees were participated into the main part of the study; 47 health-worker participants were excluded from the validity-reliability analysis. Confirmatory factor analysis (CFA) was performed. Cronbach's alpha and Spearman-Brown coefficients were calculated., Results: Of the participants, 52.9% was male; 26.1% had secondary education level or less; almost half of them had moderate economic level; 27.5% could not speak Turkish. The Cronbach's Alpha was 0.75, Spearman-Brown Coefficient was 0.76; RMSEA = 0.073, CFI = 0.93, TLI = 0.92 and GFI = 0.95 for the Scale. The Cronbach's Alpha was 0.76, Spearman-Brown Coefficient was 0.77; RMSEA = 0.085, CFI = 0.93, TLI = 0.91 and GFI = 0.95 for self-efficacy part., Conclusion: In conclusion, the adapted HLS would be a reliable instrument to evaluate the health-literacy level of Syrian refugees living in Turkey and could allow for a comparison of the host country's health literacy level to that of the refugees using the same scale., (© 2021. The Author(s).)

Aim: Pheochromocytoma (PCC) and paraganglioma (PGL) are rare tumors in childhood. They are catecholamine secreting tumors and present with signs or symptoms related to their excess. Most common signs and symptoms are hypertension, headache and diaphoresis. The management of children usually depend on experience of adulthood. This study is conducted to present the clinical characteristics, surgical management and outcome of childhood PCC and PGL in a tertiary care center., Material and Methods: We reviewed clinical records of all patients operated for PCC and PGL between 2000 and 2020 retrospectively., Results: There were 18 children operated for PCC and PGL in the study period. The female to male ratio was 1:1. The median age at diagnosis was 13 (IQR, 9-15) years. The most common presenting symptoms were headache and diaphoresis. Hypertension was the most common sign. Three patients had von Hippel-Lindau (VHL). Tumors of two patients with VHL were detected during routine follow-up. Three patients had multifocal disease. Medical preparation for surgery was carried out in all patients. Antihypertensive treatments were administered preoperatively. Since the patients are at risk for postoperative hypotension due to chronic vasoconstriction and blood volume contraction, high salt diet was recommended. Intravenous normal saline at a rate of 3000 ml/m 2 body surface area per day was started for intravascular volume expansion preoperatively. The mean duration for preoperative medication to achieve normal blood pressure was 22 days (range, 16-30). Twenty-five tumors were excised in eighteen patients. One patient who had bone metastases on diagnosis and is on I 131 MIBG therapy. The median follow-up time was 5.6 years (range, 1 months - 21 years). Five patients reached adulthood during the study period. Four of these had recurrent metastases (n = 2) and new tumors (pancreatic neuroendocrine tumor, n = 1 and pancreatic neuroendocrine tumor and renal cell carcinoma, n = 1) after the age of 18., Conclusion: Multidisciplinary approach is necessary to achieve safe surgical treatment and surveillance of PCC and PGL. Detection of associated familial cancer susceptibility syndromes and long-term follow-up is essential to detect late recurrences and new tumors., Competing Interests: Conflicts of interest We have no conflicts of interest to declare., (Copyright © 2021 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Background: Adrenocortical tumours (ACT) are rare tumours of childhood usually presenting with endocrine dysfunction. This retrospective study is designed to review our institutional experience in surgical management., Methods: Records of children treated for ACT between 1999 and 2019 were reviewed retrospectively., Results: The median age of 24 children was 78 months. Fourteen patients had adrenocortical carcinoma, nine had adrenocortical adenoma and one had neuroendocrine differentiation of ACT. Endocrine dysfunction was noted in 79% of the patients. Five patients had preoperative chemotherapy but none had a decrease in tumour size. Transabdominal approach was used in all but two patients who had thoracoabdominal incision for excision of giant tumours and ipsilateral lung metastases. Two patients had visceral excision to achieve R0 resection. Five patients, four of whom had spillage and one with partial resection died of widespread disease. Two patients with stage 4 adrenocortical carcinoma are still on chemotherapy. All patients with stage I-III disease who had total excision without spillage (n = 17) are disease-free for 2-170 months., Conclusions: Our results show the importance of excision in ACT without spillage for survival. However, multicentre prospective studies should enhance the knowledge of children about ACT and develop alternative therapies for stage III and IV cases., (© 2021 Royal Australasian College of Surgeons.)

The COVID-19 pandemic has disrupted cancer care. An audit at a major Paediatric Oncology Department in Turkey was performed to determine its impact on paediatric cancer care. A comparison was made among the number of daily paediatric cancer patients, diagnostic and treatment procedures. The data for the 'COVID-19 period' (10 March to 31 October 2020) were compared with the corresponding 'prior year control period' (10 March to 31 October 2019). Moreover, presentation delay (duration between first symptoms to healthcare visit) was calculated for new cases. The findings indicate that the mean 34.7 outpatients per day during 'COVID-19 period' was significantly lower than the 'prior year control period' (52.2). There were 17.7 inpatients per day during the 'COVID-19 period' which was significantly lower than 23.8 inpatients per day during the 'prior year control period'. Significant reduction in the daily mean number of patients undergoing chemotherapy, radiotherapy, surgery and imaging studies during the 'COVID-19 period' was also evident. A negative trend in the diagnosis of new paediatric cancers was evident with 128 new cancer cases during the 'COVID-19 period', whereas the corresponding number was 212 for the 'prior year control period'. The presentation delay (median 31 days) remain unchanged during the 'COVID-19 period'. The findings suggest significant damage to paediatric cancer care during the COVID-19 pandemic. Appropriate obligatory actions by oncology societies and policymakers can minimise longer term negative impacts., Competing Interests: The authors declare that there is no conflict of interest regarding the publication of this article., (© the authors; licensee ecancermedicalscience.)

Aim of Study: Cardiac complications may be observed after hematopoietic stem cell transplantation (HSCT). Despite significant improvement in supportive care, HSCT may be associated with significant morbidity and mortality. In this study, the aim was to evaluate the frequency of clinically serious cardiac complications after HSCT in our patients., Materials and Methods: This is a retrospective study. Cardiac complications were analyzed in 75 patients undergone to HSCT with physical examination, electrocardiography, echocardiography, and cardiac monitorization., Results: The median age was 12 years (range 11-16) and M/F ratio was 2/3 = 0.66. There are five patients with six complications among the retrospective cohort of 75. These were pericardial effusion in three patients, and sinus bradycardia in two patients and ventricular tachycardia in one patient. The incidence of cardiac complications among 75 patients with HSCT was 6.7%. The mortality rates of patients with and without cardiac complication were 40% and 34%, and both of them with cardiac complication had pericardial effusion. However, the cardiac complication was not found statistically significant factor on survival ( P = 0.82)., Conclusion: Poor risk factors of patients, myocarditis, pericarditis, and heart failure owing to cumulative doses of anthracycline, cyclophosphamide, cytomegalovirus infection or other infections, mediastinal irradiation, and cryopreserved stem cell product with dimethyl sulfoxide may be effective on the development of cardiac complications individually. Early intervention can prevent death related to this complication., Competing Interests: None

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.

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.

The objective of this study was to evaluate urinary vanillylmandellic acid (VMA) as a marker for prognosis and progression of neuroblastoma. A retrospective file search of 444 patients during 33 years was performed and correlation of VMA with clinical prognostic parameters and outcome was evaluated. Event-free survivals (EFS) were 33.5% and 21% (P = .04) and overall survival (OS) were 36.6% and 25.8% (P = .1) for all patients with normal/negative and increased/positive VMA. EFS and OS were higher in VMA(-) pelvic (P = .03) and thoracic and neck (P = .04) tumors, compared to their VMA(+) counterparts. Survival was not different in patients with abdominal primaries according to VMA status. Positive urinary VMA prevalence was low in localized disease and high in disseminated disease (P < .001). In disseminated disease, 10-year EFS was higher in VMA(+) patients than VMA(-) patients (16%, 9.5%, P = .054). Two-year OS was higher in VMA-positive patients with stage 4 disease (20.6% and 7%, P = .04). The patients with VMA(+) progressive disease died later than those with VMA(-) tumors (P = .047). These results show that increased urinary VMA predicts poor outcome for patients with favorable tumor sites. Urinary VMA can be useful risk determinant in combination with other biological determinants to predict prognosis of patients with localized neck, thoracic or pelvic tumors. The relationship of positive or high urinary VMA and survival were inversely correlated in disseminated disease and in patients with progressive disease. In high-risk patients, negative spot test or normal level of urinary VMA at diagnosis might be used for identification of a group of patients with more favorable prognosis.

Aim: This study aims to evaluate the prevalence and clinical characteristics of neurological complications in patients with newly diagnosed neuroblastic tumors (NT)., Patients and Method: Hospital file search was performed in patients with NT, and neurological complications, clinical, and treatment features were analyzed., Results: Out of 523 patients with NT, 19 patients had Horner's syndrome, 9 patients had opsoclonus-myoclonus-ataxia syndrome (OMA), 11 patients had central nervous system (CNS) involvement, and 49 patients had spinal cord compression (SCC) at diagnosis. During follow-up, seven more patients had CNS metastases. Survival rates were poor in patients with CNS involvement; all died at a median of 7.9 months after diagnosis. The neurological symptoms and signs of patients with SCC were mild, moderate, and severe in 13, 9, and 22 patients, respectively. Sphincter deficiency and sensory loss were diagnosed in 52.3% and 43.2% of patients. The neurological deficits of 11/17 patients who had symptoms less than 4 weeks were completely resolved or improved. The incidence of sequelae after laminectomy, radiotherapy, and chemotherapy were 46.2%, 66.6%, and 13.6%, respectively., Conclusion: The prevalence rates of Horner's syndrome, OMA, CNS metastasis, and SCC were found to be 3.6%, 1.8%, 2.2%, and 9.4%, respectively. Metastasis to the CNS indicated a very poor prognosis. No differences in neurological improvement in patients with SCC were found between those treated with radiotherapy, laminectomy, or chemotherapy alone, but laminectomy and radiotherapy caused significant late sequelae. Early diagnosis and proper management is critical to avoid long-term sequelae in patients with SCC.

Objective and Method: The survival of the patients with neuroblastoma has improved in last few decades. But still it depends on various clinical and biological factors. To assess the clinical features and trends in survival, the data for 500 newly diagnosed patients between January 1972 and December 2004 from a single center were retrospectively analyzed., Results: Histopathologic subtypes were neuroblastoma (NBL) in 462 patients (92.4%) and ganglioneuroblastoma in 38 patients (7.6%). The median age was 2.9 years and Male/Female ratio was 1.3/1. Primary tumor sites were abdomen, thorax, pelvis, neck, and others with the frequency of 72.2%, 14.9%, 3.8%, 3.2%, and 5.9%, respectively. There were 30, 49, 133, 257, 31 patients with stage 1, 2, 3, 4, 4S disease and their 10-year survival rates were 100%, 75.8%, 34.1%, 6.5%, and 59.4%, respectively. The outcome has significantly improved according to 10-year periods. The 5-year overall survival rates were 14%, 26.1%, 39.2%, and 52.4% for the years of 1970s, 1980s, 1990s, and after 2000. Surgical procedure involving total or near total tumor removal improved the survival (P=0.002). Both 5-year overall survival and event free survival rates were higher when partial resection was performed, especially in stage 3 disease (P=0.002 and P=0.02). In multivariate analysis, age above 18 months at diagnosis (P=0.01), stage 4 disease (P<0.001), abdominal primary tumor site (P<0.001), NBL subtype in histopathology (P=0.001), responsiveness to chemotherapy (P<0.001) positive or high Vanillyl mandelic acid levels (P=0.02) and male sex (P=0.008) were the determinants of poor prognosis., Conclusions: The survival rates in children with local disease are comparable with the results of developed countries; however, the results in children with advanced disease are still not satisfactory. To improve the outcome, especially in children with advanced disease, more effective chemotherapy regimens and molecular therapies should be investigated. Sharing the knowledge and capacity building to improve the treatment results in NBL are also critical for developing countries.

We investigated retrospectively 992 children with central nervous system tumors who were treated at our center between 1970 and 2004. All of the patients were treated by surgery, chemotherapy, and/or radiotherapy. Six patients developed second malignant neoplasms, and their clinical and histopathologic characteristics are reviewed in this article. The second malignant neoplasms were diagnosed as non-Hodgkin lymphoma, myelodysplastic syndrome, basal cell carcinoma, malignant melanoma, Kaposi sarcoma, and high-grade neuroectodermal tumor. The initial diagnoses were ependymoblastoma in one, medulloblastoma in three, and low-grade astrocytoma in two patients. The median latency time was 3.03 years (range 0.39-22.93 years). The outcome varied according to the histopathologic type of the second tumor. The patients who developed non-Hodgkin lymphoma and myelodysplastic syndrome died of progressive disease. The patients with second skin neoplasms are alive as of the time of this writing. The patient with Kaposi sarcoma developed one of the rare reported second malignant neoplasms following a primary brain tumor in childhood. A wide spectrum of second malignant neoplasms was detected after treatment of primary brain tumors with surgery, radiotherapy, and chemotherapy. Long-term follow-up is therefore necessary for the child who has survived a primary central nervous system tumor.

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.

The authors investigated the diagnostic value of cerebrospinal fluid (CSF) soluble CD27 (sCD27) for leptomeningeal involvement of non-Hodgkin lymphomas (NHL). Cytospin slides were prepared from CSF samples of 64 children treated for NHL. sCD27 levels were determined by sandwich ELISA method using two CD27 monoclonal antibodies. 8/194 (4.1%) samples were considered tumor-positive by cytology. Mean sCD27 values were 5.8 and 13.8 U/mL in tumor-negative and tumor-positive samples, respectively (p =.18). 26/194 samples were false positive and 2/194 false negative (cutoff: 7 U/mL) (sensitivity, 75 %; specificity, 86%; positive predictive value, 18.8%; negative predictive value, 98.8%; accuracy, 85.6%). With these results, the value of adding sCD27 determination to the cytological CSF examination remains questionable.

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.

A one-week-old boy had undergone resection of a sacrococcygeal benign cystic teratoma. At the age of 12 months, he had a serum alpha-fetoprotein level of 139,000 IU/ml and a recurrent pelvic mass which was removed, and the microscopic examination revealed endodermal sinus tumor. Postoperatively, massively enlarged inguinal lymph nodes and abdominal distention developed. Computerized tomography displayed enlarged inguinal lymph nodes, metastatic lesions in the liver, and a pelvic recurrent mass. He received BEP (bleomycin, etoposide, cisplatin) chemotherapy regimen, and a complete remission was achieved with a normal serum alpha-fetoprotein. Close follow-up and serum alpha-fetoprotein monitoring are mandatory after the resection of a sacrococcygeal teratoma.

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.

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., (Copyright 2002 Wiley-Liss, Inc.)

Congenital/infantile fibrosarcoma occurs frequently in the first year of life and differs from fibrosarcoma in adulthood. The clinical course of congenital/infantile fibrosarcoma is more favorable and metastatic spread is rare. While adult fibrosarcomas are common in the thigh, congenital/infantile fibrosarcomas affect chiefly the distal portions of the extremities. Standard treatment is primarily wide surgical excision. Chemotherapy may be given as neoadjuvant or adjuvant treatment in order to avoid the morbidity associated with wide excision. In this case report we present our experience of a newborn affected by congenital/infantile fibrosarcoma of the left thigh.

Pleuropulmonary blastoma is an uncommon primary malignant tumor of the lung. Surgery is the most important part of the treatment. Despite the use of chemotherapy with or without radiotherapy, the prognosis is poor. Here we report on the case of a 2.5 year-old boy presenting with pneumothorax who turned out to have a right hemithoracic mass. The patient underwent surgical resection of the mass. Pathologic diagnosis was type II pleuropulmonary blastoma. Postoperative thoracic CT showed nodular residual densities so the patient was given adjuvant combination chemotherapy with cisplatin and etoposide. He has been disease free for 12 months.

Skeletal metastases is relatively rare in hepatocellular carcinoma and accounts for 4-16% of extrahepatic metastases. The authors report a 13-year-old girl with fibrolamellar hepatocellular carcinoma, who rejected further systemic chemotherapy following hepatic lobectomy and experienced sternal and vertebral painful metastases nearly 5 years after the operation. The sternal metastatic lesion was removed surgically, whereas external irradiation was delivered to the lumbar vertebral lesion. The patient received no systemic treatment following metastases and died with widespread disease. Despite metastatic disease, the patient survived 6.5 years following the initial diagnosis.

The effectiveness of the different pharmacological agents and different doses of systemic corticosteroids was analyzed. A total of 1109 patients (median age 8 months; F/M: 2.3) with hemangioma, followed up in our unit for 23 years, were evaluated retrospectively. Forty-five of them received systemic corticosteroids. Two different pharmacological agents, prednisolone (in 26 patients) and methyl prednisolone (in 19 patients), had been used in three different regimens. Groups were compared according to the final results and rebound regrowth. Response was considered good or excellent in 16 patients (36%). Then were no differences in response to therapy among the three regimens. No difference was found in response to therapy between prednisolone and methylprednisolone and the two different doses of the methylprednisolone. Rebound regrowth was significantly higher in methyl prednisolone than in the prednisolone group (p = .045). In multivariate analysis the dimension of the lesion (p = .0065) and age at initiation of treatment (p = .0041) were the most important factors affecting the response. In conclusion, the systemic corticosteroids are effective in 36% of patients, independent of dosage and pharmacological agents and duration of the therapy. The dimension of the lesion and age at initiation of treatment are the most important factors affecting the response to treatment.

An 11-year-old boy admitted with a right cervical mass was found to have Hodgkin disease. On admission, he also had right Homer syndrome and severe cerebellar ataxia. Cranial MRI revealed marked cerebellar atrophy. He was treated with chemotherapy consisting of doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD), in addition to radiotherapy. Three months after initiation of therapy, he had a partial remission of tumor. Neurologic symptoms improved dramatically after chemotherapy started. Hodgkin disease should be included in the differential diagnosis of children with cerebellar findings and Horner syndrome.

This study was conducted to evaluate the signs and symptoms on admission, diagnosis, localization, therapy, and survival of patients with primary intracranial germ cell tumors (PICGCT). Eight patients with surgically confirmed PICGCTs were treated and followed up at Hacettepe University's Department of Pediatric Oncology between 1974 and 1995. While one patient was admitted with a second recurrence of her disease, the others were admitted or referred primarily to our institution. In this period, 357 germ cell tumor and 684 primary intracranial malignant tumors were diagnosed and treated at our institution. Thus, PICGCTs comprised 1.1 percent of the primary intracranial malignant tumors and 2.2 percent of the germ cell tumors. There were four females and four males and the median age was eight years (13 months to 12 years). On admission, the most common symptoms were diabetes insipidus (3/8) and vomiting (3/8). One patient also and Down's syndrome. Locations of the tumors were suprasellar in three, in the third ventricle in two, and in the cerebral parenchyma, and pineal and hypothalamic regions in the remainder. There were germinomas, three malignant teratomas, and two mixed germ cell tumors. Only two patients could be treated with appropriate and adequate chemotherapy and radiotherapy. Three patients died: one in the postsurgical period, one after the third surgical approach and one 11 months after the diagnosis of progressive disease; three were lost to follow-up. The remaining two patients (with second recurrence and disseminated disease) are alive and without disease. Our experience with these patients demonstrated that appropriate and adequate chemotherapy is as effective a treatment as radiotherapy, even with recurrence of the disease.

Vascular lesions in childhood are classified as vascular malformations and hemangiomas. Vascular malformations are congenital abnormalities thought to arise from defects during embryological development of vascular tissue. Hemangiomas are benign tumors of vascular endothelium and can spontaneously become involuted in almost all cases. One thousand one hundred and twenty-seven patients with vascular lesions were followed by the Department of Pediatric Oncology, Hacettepe University, for 19 years. Diagnosis was based mainly on history, clinical condition and follow-up data in 98.2 percent of cases. The distribution of the vascular lesions was as follows: 969 patients had hemangiomas, 120 had lymphatic malformations, 18 had combined vascular malformations, 11 had venous malformations, six had port-wine stain, and three had angiokeratoma. Except for ten cases with Klippel-Trenaunay Weber syndrome, vascular malformations were not accompanied by any syndrome.

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.

The case of a 9-year-old boy diagnosed and treated for cutaneous anthrax is presented. We discuss the clinical features and course of this disease, encountered today only rarely in less developed regions where humans are in contact with infected animals.

In this article a family with five members suffering from primary hypertrophic osteoarthropathy (PHO) is reported. Our cases are characterized by swollen and painful joints and clubbing of distal phalanges of hands and feet. The diagnosis made upon on radiological and clinical findings.

The effects of low-dose and short-time acyclovir therapy in 14 children with malignant disease of ages 4-18 years who had developed varicella zoster virus infections while receiving aggressive chemo-/+radiotherapy are reported. Ten of them had chickenpox and 4 herpes zoster. Acyclovir 5 mg/kg was infused IV every 12 h in 9 patients and every 8 h in 5 patients for a median of 4 days' duration. We resumed the primary therapy when the patients' lesions had dried out and became crusted and new lesions had not reappeared. The period of initiation of the acyclovir therapy to the resumption of oncological treatment was 8.4 +/- 2.7 days for chickenpox and 12.0 +/- 3.4 days for herpes zoster patients. After restarting the oncological therapy, no adverse effects of acyclovir or complication of infection were observed. The efficiency of early, short-term, and relatively low dose acyclovir therapy is discussed and compared to the results in the relevant literature.

An unusual case of non-Hodgkin's lymphoma with primary renal involvement in a four-year-old boy is presented. The diagnosis was established by renal biopsy. The findings of an exploratory laparotomy and an extensive staging procedure showed no other primary site. The patient has been followed up for 14 months and no evidence of recurrence has been observed.