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We report a case of infiltrating angiolipoma who presented with a large mass on the shoulder and the signs of Kasabach-Merritt syndrome. She improved dramatically within one month and the [...]

Maxillofacial osteosarcoma constitutes a minor percentage of all the head and neck tumors. We describe a 10 year-old girl presenting with swelling and pain in left maxillary region and diagnosed as low grade osteosarcoma. The patient was operated and given a chemotherapy protocol consisted of Cisplatin and Doxorubicin. After six courses of chemotherapy the patient was in complete remission and she is well with no evidence of disease for five years. Since high local recurrence rates have been reported in craniofacial osteoarcoma and we know the deleterious side effects of radiation therapy in children, we believe that best management strategy for osteosarcomas in maxillofacial region in children is radical surgical excision and postoperative chemotherapy (Fig. 3, Ref. 11).

Cystatin, a proteinase inhibitor, is involved in the intracellular catabolism of proteins. We investigated the change in concentration of serum Cystatin C (CysC) in children with lymphomas and its diagnostic utility. Twenty-eight newly diagnosed patients with lymphoma were included in this study. The male/female ratio was 20/8, with a median age of 8.5 years (range 3-17 years). Thirteen patients had Hodgkin's lymphoma (HL) and 15 had non-Hodgkin's lymphoma (NHL). Cystatin C concentration was determined at the time of diagnosis and during remission. In the entire group, CysC concentrations at diagnosis and during remission were 0.87+/-0.29 mg/L and 0.86+/-0.21 mg/L, respectively (p=0.93). In the NHL group, CysC concentrations at diagnosis and remission were 0.89+/-0.32 mg/L and 0.85+/-0.23 mg/L, respectively (p=0.73). The CysC concentrations in the HL group at diagnosis and remission were 0.88+/-0.36 mg/L and 0.88+/-0.18 mg/L, respectively (p=0.73). No significant difference was observed between CysC concentrations in the HL (0.88+/-0.36 mg/L) and NHL (0.89+/-0.32 mg/L) groups. Cystatin C concentrations in all the patients with localized versus those with advanced disease were 0.91+/-0.41 mg/L and 0.88+/-0.3 mg/L, respectively (p=0.83). Cystatin C concentrations of the patients with localized and advanced HL were 0.95+/-0.45 mg/L and 0.77+/-0.14 mg/L, respectively, (p=0.41). Cystatin C level was higher in patients with localized disease, in those without B symptoms, and, at diagnosis, in those with an unfavorable response (Tab. 2, Ref. 20). Full Text in free PDF www.bmj.sk.

Background: We aimed to review clinical characteristics, treatment results and outcome of pediatric patients with primary pleuropulmonary neoplasms., Methods: Medical records of 14 cases diagnosed between 1972-2009 were reviewed retrospectively., Results: The male/female ratio was 5/9 and the mean age at diagnosis was 9.1 years (2-16). All but one were symptomatic, presenting with fever, coughing, dyspnea, or weight loss. One patient presented with hemoptysis, and another with digital clubbing. One mesothelioma was diagnosed incidentally. Some 8/14 patients were initially diagnosed as having pneumonia (median delay in diagnosis of 2.5 months). Diagnoses included pleuropulmonary blastoma (PPB, n = 5), inflammatory pseudotumor (n = 3), mesothelioma (n = 2), mucoepidermoid carcinoma (MEC, n = 2), and carcinoid tumor (n = 2). Patients with PPB underwent surgery and received chemotherapy with or without radiotherapy. Two carcinoid tumor cases underwent surgery, one further received chemotherapy. Patients with mesothelioma were treated with chemotherapy. Inflammatory pseudotumors were all resected. Two cases with MEC received chemotherapy, one after surgery. 2/5 PPB patients survived without recurrence, 3 died; all carcinoid tumors and inflammatory pseudotumors were alive; 1/2 MEC patients was alive after 252 months, the other one was lost without disease; 1/2 mesothelioma patients was alive without disease, the other was died. For all cases, median follow-up was 30.5 months (0.6-252)., Conclusions: Primary pleuropulmonary tumors are rare but clinical presentation can be varied and delay in diagnosis is common. Children with persistent coughs, recurrent pneumonia or hemoptysis should be considered as indicators for early diagnosis, very important because the prognosis of these tumors varies with histology and stage.

Background: A number of clinical studies conducted in adults have demonstrated the prognostic significance of angiogenic factors in malignancies, however, only a limited number of studies have been conducted in children. The aim of this study was to determine serum vascular endothelial growth factor (VEGF), endostatin, and leptin levels in children with lymphoma and to investigate whether these factors provide prognostic information., Procedure: Serum samples from 36 children with lymphoma (non-Hodgkin lymphoma (NHL) N = 21, Hodgkin lymphoma (HL) N = 15) were collected at diagnosis and during remission. Serum samples were also collected from 18 healthy children as the control group. Serum VEGF and endostatin levels were quantified by using enzyme-linked immunosorbent assay (ELISA) and serum leptin by immunoradiometric assay., Results: The serum VEGF levels were found elevated in patients compared to controls (P = 0.033), while endostatin and leptin levels were lower in patients than in controls (endostatin, 43.9 ± 5.8 ng/ml vs. 123.6 ± 13.5 ng/ml, P < 0.001; leptin, 5 ± 1.5 ng/ml vs. 6.7 ± 1.2 ng/ml, P = 0.013). VEGF levels declined (pre, 151.6 ± 55.9 pg/ml vs. post, 16.2 ± 7.9 pg/ml, P = 0.041), while endostatin and leptin levels increased in patients who achieved remission (33 of 36 patients) when compared to pre-treatment levels (endostatin pre, 43.1 ± 5.9 ng/ml vs. post, 65.9 ± 6.8 ng/ml, P = 0.047; leptin, pre, 5.3 ± 1.6 ng/ml vs. post, 9.8 ± 2.7 ng/ml, P = 0.012). Serum VEGF, endostatin, and leptin levels were not predictive of survival., Conclusion: Serial measurement of serum VEGF, endostatin, and leptin levels could potentially be used to predict response to treatment or progressive disease in children with lymphoma., (Copyright © 2010 Wiley-Liss, Inc.)

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.

Wilms tumor (WT) is the most common primary renal tumor in childhood. The occurrence of WT in patients with growth retardation, mental retardation and central nervous system abnormalities in association with premature chromatid separation (PCS) and mosaic variegated aneuploidy has been previously described in only 10 patients. Here we report the very rare occurrence of WT with two other malignancies, acute myeloid leukemia and medulloblastoma in association with chromosomal instability. This is a novel presentation of Fanconi anemia with this cytogenetic abnormality., ((c) 2009 Wiley-Liss, Inc.)

In clinical practice, when neutropenic-fever patients present with no microbiologically and clinically defined infection, the risk of underestimating an occult infection is of major concern, the clinicians have to make a decision on when to modify antibiotic therapy. Hence, a reliable, specific, and sensitive marker, which is regulated independently from the leukocyte count and the underlying disease, is needed for the early diagnosis of infections in cases of neutropenic fever. We have evaluated the diagnostic and follow-up value of procalcitonin (PCT) compared with C-reactive protein (CRP) and erythrocyte sedimentation rate in documenting the infection in neutropenic-fever patients undergoing intensive chemotherapy, as evidenced by the durational change in these parameters in the presence of defined infection. Forty-nine patients, who had 60 febrile episodes, and who were hospitalized in the Hacettepe University Ihsan Doğramaci Children's Hospital between January 1, 2004 and January 1, 2005 were included in this prospective study. All patients had been diagnosed with neutropenic fever after intensive chemotherapy. In our study, PCT and CRP levels were significantly higher in neutropenic-fever patients (group I and group II separately) than in control patients (P<0.001) throughout the study period; but erythrocyte sedimentation rate levels did not show any significant difference (P>0.05). In sequential analyses of patients without documented infections, the median of PCT concentrations shows a tendency to fall after the 8th hour of onset of fever, whereas in patients with documented infections PCT concentrations fell after the 48th hour. In conclusion, our study suggests that PCT, when measured periodically, is a more useful diagnostic inflammation parameter in pediatric neutropenic-fever patients than CRP, both in estimating the severity of the infection and, the duration and origin of the fever. Hence, PCT might be helpful when deciding on initial therapy modification.

Purpose: To evaluate the outcome of patients 17 (90.5% versus 64.7%; p=0.02). Patients aged <16 years had longer 5 year DMFS (90.5% versus 67.7%; p=0.05) with a borderline significance. DMFS is significantly shorter in patients initially presented with N3 disease compared to N0-2 disease (58.8% versus 84.4%; p=0.04)., Conclusions: We suggest that RT combined with multiagent chemotherapy is effective in achieving satisfactory DFS and comparable OS in young patients with NPC. Results of both previous series and ours are evoking the necessity of early administration of efficient chemotherapeutic agents in addition to radiotherapy, but indistinct is the precise regimen and the intensity along the radiotherapy. All efforts towards decreasing late side effects of treatment should be encouraged in this long life expected group.

The symptoms, histology, extent and course of disease in 16 adolescents with colorectal carcinoma who were admitted to Hacettepe University Children's Hospital between 1972 and 1990 are presented. Most patients presented with vague abdominal complaints. Twelve of the 16 patients had mucin-producing adenocarcinoma. Extensive disease at diagnosis and unresponsiveness to medical management were determined. Only one patient survived free of disease four years after diagnosis. Nine of the patients died between one day and one year following the initial surgery. The remaining six patients were very ill when they were discharged from the hospital, after which time no information was received concerning them.

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.