Neurological complications of neuroblastic tumors: experience of a single center.

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.

The prognostic significance of vanillylmandellic acid in neuroblastoma.

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.

Treatment of Wilms tumor: a report from the Turkish Pediatric Oncology Group (TPOG).

AIM: To standardize diagnosis and treatment of childhood Wilms tumor (WT) in Turkey. METHODS AND PATIENTS: Between 1998 and 2006, WT patients were registered from 19 centers. Patients <16 years with unilateral WT whose treatment started in first postoperative 3 weeks were included. Treatments were stage I favorable (FH) and unfavorable histology (UH) patients, VCR + Act-D; stage IIA FH, VCR + Act-D; stage IIB FH, VCR + Act-D + radiotherapy (RT); stage III-IV FH, VCR + Act-D + adriamycin (ADR) + RT; stages II-IV UH tumors, VCR + Act-D + ADR + etoposide + RT. RESULTS: 165/254 registered cases were eligible (bilateral, 5.9%) [median age 3.0 years; M/F: 0.99; 50/165 cases < or =2 years]. 9.7% cases had UH tumors. Disease stages were stage I 23.6%; IIA 36.4%; IIB 5.5%; III 22.4%; IV 12.1%. Cases >2 years had significantly more advanced disease. 1/11 cases with recurrent disease died; 2/165 had progressive disease, 2/165 had secondary cancers, and all 4 died. In all cases 4-year OS and EFS were 92.8 and 86.5%, respectively. Both OS and EFS were significantly worse in stage IV. CONCLUSIONS: Despite problems in patient management and follow-up, treatment results were encouraging in this first national experience with a multicentric study in pediatric oncology. Revisions and modifications are planned to further improve results and minimize short- and long-term side effects.

Undulant course of AFP: a sign of tumor activation or not?

OBJECTIVE: To assess the predictive value of undulant alpha fetoprotein (AFP) course in recurrence of germ cell tumors (GCT). PATIENTS/METHOD: A retrospective file search of 491 patients with GCT was performed and 18 patients who had an undulant course of AFP levels (increased and spontaneously decreased) without any demonstrable tumor on radiological images were selected for the study. RESULTS: The median age of the 10 boys and 8 girls was 2 years (1.1-16 years). All the patients were disease-free for a median of 16.2 months (8.4-132 months). At follow-up, radiologically proven tumor recurrences were diagnosed in 8 patients in median 12.2 +/- 3.2 months from the initial diagnosis (8.4-18.2 months) and 7.4 +/- 2.2 months from the last normal level of AFP (5.0-11.2 months). The mean peak AFP values within the first 90 days were 24 +/- 38.1 ng/mL and 11.1 +/- 10.9 ng/mL in patients who did and did not have recurrent disease, respectively (p = .0051). The patients whose AFP levels were higher than 10 ng/mL were likely to have recurrent disease (p = .02). CONCLUSION: Without a demonstrable tumor, the small amplitude undulations may only require careful follow-up. However, it should be kept in mind that despite the decrease in AFP levels, there still might be a risk of recurrence, especially in patients who have greater amplitude undulations.

Ewing sarcoma in an infant and review of the literature.

Bedük Esen ÇS, Gültekin M, Aydin GB, Akyüz C, Karli Oguz K, Orhan D, Cengiz M, Gürkaynak M, Yildiz F. Ewing sarcoma in an infant and review of the literature. Turk J Pediatr 2019; 61: 760-764. Ewing sarcoma (ES) is a rare tumor in infants and prognosis is controversial. There are no standard recommendations for treatment in such very young patients. Generally, radiotherapy (RT) is not a part of treatment in infants due to the risk of severe late side effects. In this case report, we report a 7-month-old boy with diagnosis of left mastoid bone ES with lung metastases at diagnosis, showing a rapidly fatal outcome despite aggressive systemic chemotherapy and RT without surgery.

Extranodal type T/NK-cell lymphoma with an atypical clinical presentation.

Peripheral-type natural killer (NK)- or T-cell lymphomas are rare disorders characterized with clonal proliferation of mature lymphocytes. They have been linked to chronic and active Epstein-Barr virus infection (CAEBV), which itself is not defined as a malignant hematological disorder. The authors present a patient with T/NK-cell lymphoma involving skin, kidneys, spleen, pancreas, and meninges. She was remarkable for having the mosaic feature of more than one type of extranodal T/NK-cell lymphoma. She also had mixed findings of CAEBV that might have been attributed both to hypersensitivity to mosquito bites and to hemophagocytic lymphohistiocytosis.

Comparison of accelerated and rapid schedules for monovalent hepatitis B and combined hepatitis A/B vaccines in children with cancer.

The aim of this study was to determine the efficacy of immunization against hepatitis A and B infections with "rapid" or "accelerated" schedules in children with cancer receiving chemotherapy. Fifty-one children were recruited to receive either vaccination schedule, in the "rapid vaccination schedule"; hepatitis B (group I) or combined hepatitis A/B vaccines (group III) were administered at months 0, 1, 2, and 12; in the "accelerated vaccination schedule," hepatitis B (group II) or combined hepatitis A/B (group IV) vaccines were administered on days 0, 7, 21, and 365 intramuscularly. The seroconversion rates at months 1 and 3 were 35.7 and 57.1% in group I and 25 and 18.8% in group II, respectively. Group I developed higher seroconversion rates at month 3. In group III the seroconversion rates for hepatitis B at months 1 and 3 were 54.5 and 60% and in group IV 50 and 70%, respectively. For hepatitis A, the seroconversion rates at months 1 and 3 were 81.8 and 90% in group III and 80 and 88.9% in group IV, respectively. The accelerated vaccination schedule seems to have no advantage in children receiving cancer chemotherapy except for high antibody levels at month 1. In conclusion, the accelerated vaccination schedules are not good choices for cancer patients. The combined hepatitis A/B vaccine is more effective than monovalent vaccine in cancer patients, which probably can be explained by an adjuvant effect of the antigens. The seroconversion of hepatitis A by the combined hepatitis A/B vaccination is very good in cancer patients.

Immunogenicity of hepatitis a vaccine in children with cancer.

This study evaluated the immuned response of the hepatitis A vaccine in children with cancer who were receiving chemotherapy. Twenty-eight patients with lymphomas or solid tumors and who had negative serology for hepatitis A were enrolled. The median age was 4.7 years (range 2-16). The patients received 1440 IU hepatitis A vaccine at 0 and 6 months. Seroconversion rates at the first and seventh months were 60% (n = 17/28 patients) and 89% (n = 24/27 patients). No adverse effects were observed. The hepatitis A vaccine was found to be effective and safe in children with cancer.

Paratesticular metastasis from Wilms tumor associated with a hydrocele.

Metastatic sites other than the lungs, lymph nodes, and liver are unusual for Wilms tumor (WT). Intra-scrotal metastasis is very rare. We report a 3-year-old boy with stage IIA WT, who experienced paratesticular metastasis 2 months after surgery for an abdominal recurrence. He had right scrotal hydrocele at initial diagnosis. The patient underwent right radical orchiectomy, and pathological examination revealed paratesticular WT metastasis. Intra-abdominal and peritoneal disseminated metastases followed. We considered that tumor cells spread through the patent processus vaginalis and grew at paratesticular space in hydrocele. One month after the end of 12 months of salvage chemotherapy and abdominal radiotherapy, the patient has no evidence of disease.