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Gastrointestinal stromal tumors (GISTs) are rare in the childhood period. The authors reported a case who was admitted to the neonatal intensive care unit (NICU) on a suspicion of intestinal obstruction. She was operated and a mass in a size of 6 x 4.5 x 4 cm was resected from the ileum. Histologic and immunohistochemical studies showed a GIST. CD34, small muscle actin (SMA), and desmin were positive. The baby was discharged on the 13th day after operation.

Although brucellosis is endemic in Middle Eastern and Mediterranean countries, febrile neutropenia caused by Brucella species has rarely been reported in childhood. In this report the authors described an unusual case of febrile neutropenia due to Brucella melitensis.

Primary renal primitive neuroectodermal tumor (PNET) is an extraordinarily rare neoplasm in childhood. It generally occurs in young adults and only a few pediatric cases have been reported. PNET in the kidney acts aggressively and the record shows poor therapeutic response. The authors present the case of a 16-year-old girl who was diagnosed with renal PNET and treated with high-dosage chemotherapy and peripheral blood stem cell transplantation (PBSCT).

Plexiform neurofibroma is a relatively common but potentially devastating manifestation of neurofibromatosis type 1 (NF 1). A substantial number of plexiform neurofibroma causes morbidity. Various treatment modalities are considered to decrease pain. In this paper a case with plexiform neurofibroma causing severe pain and in whom alpha-interferon was used is presented.

The present study was carried out to clarify the role of matrix metalloproteinase-2 and -9 (MMP-2 and MMP-9), tissue inhibitor of metalloproteinase-1 (TIMP-1), vascular endothelial growth factor (VEGF), and microvessel density (MVD) in the clinicopathologic behavior of childhood B-cell non-Hodgkin lymphoma (NHL). Paraffin-embedded biopsy specimens from 25 children with NHL were studied by immunohistochemically and the correlate the expression of these markers to clinicopathologic characteristics. Positive MMP-9 staining was associated with an increased prevalence of B-symptoms (p = .046). High microvessel density (MVD) showed a tendency toward an adverse outcome and it was correlated with clinical stage (p = .023). The event-free survival of high MVD patients was less than for those with low MVD, but the difference was not statistically significant (64.1% vs. 85.71% respectively, p = 0.15). The overall survival of high MVD patients was less than for those low with MVD and the difference was statistically significant (55.53% vs. 100% respectively, p = .039). Neither gelatinases nor VEGF correlated with age, sex, disease stage, the occurrence of bulky disease, or extranodal disease. The results showed that angiogenesis and angiogenic factors might have a role in development and clinical behavior of childhood NHL. Larger series of patients are needed to determine the prognostic value of angiogenesis in childhood non-Hodgkin lymphoma.

The aim of this study was to evaluate and compare the clinical characteristics of the B-cell non-Hodgkin lymphoma (NHL) patients and therapeutic efficacy of modified NHL BFM-90 and NHL BFM-95 protocols in the authors' center. From January 1993 to December 2003, 61 newly diagnosed children with B-NHL were enrolled to the study. The patients were stratified by risk factors and treated either with a modified B-NHL BFM-90 or BFM-95 protocols. The use of 1 or 3 g/m2 of methotrexate instead of 5 g/m2/24 h was the only important modification in BFM-90 protocol. Sixty-one children (12 girls, 49 boys) with a median age of 6.5 years (range: 2.5-16) were treated in the center. There were 14 patients in stage II, 28 in stage III, and 19 in stage IV. The most common initial primary tumor sites were abdomen, head, and neck. Forty-five patients were treated with modified B-cell BFM-90 and 16 patients were treated with B-cell BFM-95 regimens. The 5-year overall survival (OS) for all patients was 85.8%, and event-free survival (EFS) was 82.8%. The 5-year OS rates in modified BFM-90 and in BFM-95 protocols were 85.2 and 87.5%; the 5-year EFS rates in these 2 protocols were 84.6 and 70%, respectively (p >.05). Factors associated with lower EFS by univariate analysis were bulky disease, risk groups, and LDH level > or = 500 IU/L. By multivariate analysis only LDH level was significant. In conclusion, the treatment results in this study were similar to those of BFM group.

Nodular regenerative hyperplasia (NRH) and focal nodular hyperplasia (FNH) of the liver rarely occur in children after completion of tumor therapy. These lesions mimic hepatic metastasis and they must be distinguished from metastatic lesions. The authors present 2 children, one with NRH and one with FNH, after undergoing antineoplastic therapy for non-hepatic childhood solid tumors and discuss their patients in the context of the literature.

The aim of this study was to evaluate children with lymphadenopathy and clinical approach to the suspicion of malignancy. The authors evaluated 457 patients with peripheral lymphadenopathy, less than 19 years of age, and referred to the Pediatric Oncology Department of Gazi University Medical School during the periods March 1996-April 2004. A total of 346 patients had benign disorders and 111 had malignant pathologies. Excisional biopsies were performed to 134 patients. A specific etiology could be found 39% in the benign group. Of the 457 patients, 218 were presented as acute, the rest as chronic lymphadenopathy. In the acute lymphadenopathy group, 98.2% of the patients had benign etiologies. The malignant disorders were mostly represented as chronic lymphadenopathy. Concerning the extension, 193 patients had localized lymphadenopathy and 264 had generalized lymphadenopaties. Cervical region was the most frequent site in both localized and generalized lymphadenopathy groups. Malignancies occurred as generalized lymphadenopathy. Supraclavicular area were involved only in the malignant group. Axillary involvement was predominant in BCG vaccine associated lymphadenitis and mycobacterium tuberculosis. All the lymph nodes less than 1 cm were due to benign causes. The malignant lesions were usually more than 3 cm in diameters. The following findings should alert the pediatrician for the probability of a malignant disorder: lymphadenopathy of more than 3 cm in size, of more than 4 weeks in duration, with supraclavicular involvement, and with abnormal laboratory and radiological findings.

Carnitine is ingested through animal-derived foods as well as synthesized in vivo. It plays an important role in the energy metabolism of many tissues. Iron acts as a co-factor for the synthesis of carnitine. However, the importance of iron deficiency as a cause of secondary carnitine deficiency is not well established. The aim of this study was to investigate the serum levels of carnitine in children with iron-deficiency anemia compared to those of healthy children and to determine if serum carnitine levels in with or without pica differ. The mean serum carnitine concentration in the iron-deficiency group was significantly lower than that in healthy children (12.44+/- 5.09 and 32.48 +/- 7.92 micromol/L, respectively, p < .001). In the iron-deficient group, serum carnitine levels, ferritin levels, and other hematological parameters were lowest in patients with pica (p < .001). Pearson correlation test indicated a positive correlation between serum carnitine and ferritin levels in iron-deficient patients. Based on the evidence about the effect of low iron on carnitine stores in animal studies, the authors propose that low serum carnitine levels in these children may be secondary to iron-deficiency anemia. However, further large-scale studies are needed to establish the frequency of carnitine deficiency in children with iron-deficiency anemia.

A prospective, open-label, randomized, comparative study in pediatric cancer patients was conducted to evaluate the efficacy and safety of cefepime and meropenem in the empiric therapy of febrile neutropenic patients. Febrile episodes were classified as microbiologically documented infection, clinical documented infection, or fever of unknown origin. Clinical response to therapy was classified as success or failure. In this period 37 children with solid tumors including lymphoma, 25 males, 12 females, had neutropenia on 65 occasions. Microbiologically documented infections occurred in 21 episodes (32.31%). Frequency of positive bacteria isolated was higher than gram-negative bacteria. There was no infection-related death. There were no statistical differences between the cefepime and meropenem groups for duration of fever or neutropenia, response rate, and necessity for modification. Cefepime appears to be as effective and safe as meropenem for empiric treatment of febrile episodes in neutropenic pediatric cancer patients.

Anterior mediastinal and adenoid masses in children after cessation of chemotherapy for malignant disease often cause a diagnostic problem. Differential diagnosis of thymic enlargement and adenoid hyperplasia from recurrence frequently poses a challenge both for the radiologist and the physician. In this study the authors evaluated 491 patients with different malignant tumors for thymic and adenoid hyperplasia. Thymic hyperplasia was seen in 18 patients (5 Hodgkin disease (HD), 5 non-Hodgkin lymphoma (NHL), 4 Wilms tumor, 2 germ cell tumor, 1 Ewing sarcoma, and 1 neuroblastoma), only adenotonsillar hyperplasia was seen in 6 patients, all with NHL, and both thymic and adenotonsillar hyperplasia were seen in 3 patients (1 HD, 2 NHL). In 5 patients, adenoid hyperplasia was proven by biopsy; 1 patient underwent to adenoidectomy. Their histopathologic investigation showed polyclonal follicular hyperplasia. The authors recommend that patients with thymic and/or adenotonsillar enlargement after successful treatment of their primary malignancy should be evaluated cautiously before an invasive procedure is planned.