Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) treated with 2-chlorodeoxyadenosine.

Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease (RDD) is a rare but well-defined histiocytic proliferative disorder of unknown etiology that usually presents with cervical lymphadenopathy, fever, leukocytosis, and hypergammaglobulinemia in an otherwise healthy child. Although many patients undergo spontaneous remission, a subset of patients with systemic disease has a more serious course. For those patients with a poor outcome, steroids and chemotherapeutic agents such as etoposide or 6-mercaptopurine plus low dose methotrexate have been used. We present a child with a massive cervical lymphadenopathy treated with 2-chlorodeoxyadenosine (2-CdA, cladribine) after other approaches failed.

[Analysis of paediatric neuro-oncological information on the Internet in German language]. / Analyse pädiatrisch neuro-onkologischer Informationen in deutschsprachigen Internetseiten.

BACKGROUND: The fast growing internet offers easy access to medical information. So far there are limited data concerning the quality of this information. This study examined quality and readability of paediatric neuro-oncological information on the internet in german language. METHOD: Using the search terms "medulloblastoma", "ependymoma", "craniopharyngeoma", "brainstem glioma" and "low grade astrocytoma" in six different search engines, the first 30 universal/uniform resource locators (URLs) of each search engine were assessed. Appropriate Web sites were evaluated in regards to quality using DISCERN-Instrument and checklist rating system. Readability was rated by Flesch Reading Ease score. RESULTS: Out of 889,56 web sites remained evaluable. Most of the sites rated as poor to very poor (49 %), 30 % rated as fair and 21 % as good to very good. Readability was scored as very difficult with complex vocabulary content limiting the usefulness of good web sites. CONCLUSIONS: Search-ing for childhood brain tumours via internet is time consuming and most often ineffective. There is a lack of high-quality and comprehensible information on childhood brain tumours on german web sites. Cooperation of scientific medical societies and the Federal Ministry of Health is essential to provide comprehensible and high-quality information on internet as an effective and supportive resource for patients and their relatives.

Anafilaxis debida a tenipósido (VM-26) en Pediatria / Anaphylaxis caused by teniposide in Pediatrics

Propósito: La anafilaxis relacionada con la administración de agentes citostáticos ha sido descrita en varios tipos de antineoplósicos, incluyendo las epipodofilotoxinas, Etopósido y Tenipósido. El objetivo de nuestro trabajo es comunicar el desarrollo de reacciones anafilácticas en pacientes pediátricos tratados con Tenipósido (VM-26).Material y métodos: Las reacciones anafilácticas se desarrollaron en dos niños diagnosticados de neuroblastoma y leucemia linfoide aguda respectivamente, tratados con VM-26 como parte de la terapia antineoplásica. Resultados: Ambos pacientes sufrieron reacciones anafilácticas graves durante la primera o sucesivas administraciones del citado antineoplásico, provocando la muerte en uno de los pacientes por shock anafiláctico fulminante. Conclusiones: Dada la gravedad de las mismas, así como de la incidencia de anafilaxis asociada a la administación de Tenipósido publicada por otros autores, concluimos que sería conveniente considerar la premedicación en todos los niños que van a recibir Tenipósido durante el tratamiento antineoplásico (AU)

Neuroblastoma IV-S followed by extra-adrenal pheochromocytoma 15 years later.

Common origin of sympathoblasts and pheochromoblasts from the neural crest cells is generally accepted. Neuroblastoma and pheochromocytoma result from an abnormal proliferation of these committed cells. They are included in the group of neuroendocrine neoplasms, formerly named Apudomas. Previous reports of mixed tumours of neuroblastoma and pheochromocytoma, and ganglioneuroma and pheochromocytoma, support this hypothesis. A case of extra-adrenal pheochromocytoma in an adolescent who 15 years before was successfully treated for a stage IV-S neuroblastoma without evidence of primitive tumour is reported. Two hypothesis are formulated: (1) the second neoplasm may have arisen from the unlocated primitive tumour, probably silent evolution could be due to its maturation into ganglioneuroma-pheochromocytoma; (2) taken into account a common origin of both neoplasms, the patient may have a genetic predisposition to develop neuroendocrine tumours. Whatever the mechanism, second neoplasms, as the case reported, late relapses, and late toxic effects may justify long follow-up of neuroblastoma survivors.

G-CSF after autologous bone marrow transplantation for malignant diseases in children. Spanish Working Party for Bone Marrow Transplantation in Children.

The use of recombinant human granulocyte-stimulating factor (G-CSF) has been shown to effectively accelerate granulocytic recovery after autologous bone marrow transplantation (BMT) in adults. The experience, however, is limited in children. We evaluated the hematopoietic reconstitution in 41 consecutive children undergoing autologous BMT for hematologic malignancies (21 acute lymphoblastic leukemia, five non-Hodgkin's lymphoma) and solid tumours (seven neuroblastoma, two brain tumor, three Ewing's sarcoma, two Wilms' tumor, one rhabdomyosarcoma). Their ages ranged from 2 to 16 years (mean 7.2 years). rhG-CSF was given at a dose of 10 micrograms/kg/day i.v. in a 2h infusion from day +1 until +28 or until the absolute neutrophil count (ANC) was > 1 x 10(9)/L. These patients were compared with a similar historical control group of 38 children who did not receive rhG-CSF after autologous BMT. The number of cells infused was similar in both groups. At the dose and schedule used in the present study, rhG-CSF was well tolerated and no side-effects were observed. The number of cell infused was similar in both groups. At the dose and schedule used in the present study, rhG-CSF was well tolerated and no side-effects were observed. Our data show that rhG-CSF accelerates engraftment and reduces the number of febrile days and antibiotic use. Furthermore, patients who were treated had less infections.

Acute nonlymphoblastic leukemia in children treated for acute lymphoblastic leukemia with an intensive regimen including teniposide.

Some cases of conversion from acute lymphoblastic leukemia (ALL) to acute nonlymphoblastic leukemia (ANLL) at relapse have been reported recently. We report three cases initially diagnosed as having ALL and showing morphological, cytochemical, and immunophenotypic features of ANLL at relapse (lineage switch). Conversion was observed among 14 patients who developed bone marrow relapse while undergoing intensive treatment with our ALL protocol, which includes teniposide, and that had been administered to 62 patients. The three cases converted at first relapse, with a mean time of 20 months (13-29 months). Clinical and immunologic characteristics of T-cell leukemia were present in one patient. Changes documented in cytogenetic studies are discussed. The underlying mechanisms for the lineage switch remain unclear as does its relation with mixed lineage leukemias, but we believe that drugs employed in our therapy protocol could have had an influence on this conversion.

Hepatosplenic candidiasis in children with acute leukemia.

Three children with acute lymphoblastic leukemia developed disseminated fungal disease predominantly involving the liver and spleen. The three patients were undergoing induction chemotherapy and had neutropenia when they presented prolonged fever not responsive to antibiotics. Once neutropenia was recovered, hepatosplenomegaly leukocytosis, elevated serum alkaline phosphatase, and hypoechoic areas in the spleen and liver ultrasound were observed. All fungal blood cultures were negative, with the diagnosis being confirmed by histologic study. One of the patients died without achieving control of the candidiasis. The other two patients received prolonged antifungal treatment concurrently with chemotherapy and both are alive, one of them cured and in complete remission. The increasing frequency of this infection in recent years and the importance of a prompt and prolonged administration of antifungal therapy to obtain the cure are discussed.

Fine-needle aspiration biopsy in children: experience in 70 cases.

Results of 70 fine-needle aspiration biopsies (FNAB) were evaluated retrospectively in 61 pediatric patients. Over a period of 9 months all mass lesions suspected being malignant were aspirated. Twelve of the 70 aspirations were performed in children having known tumours, in order to exclude recurrence or metastasis. The others were carried out to obtain a diagnosis. Satisfactory specimens were obtained from 58 (83%). There were 21 benign diagnoses, 36 malignant diagnoses, and 1 with suspected malignancy. Correlation of histologic and cytologic diagnoses was possible in 45 cases. The diagnostic sensitivity and specificity were 95% and 80%, respectively. We have found FNAB more accurate in the diagnosis of malignancies than in benign lesions. The results suggest that this is a useful technique for obtaining a first diagnosis of malignancy, as well as for excluding recurrence or metastatic disease.

[Acute lymphoblastic leukemia of high risk: results of a therapeutic protocol]. / Leucemia linfobláastica aguda de alto riesgo: resultados de un protocolo terapéutico.

Sixteen consecutive children diagnosed of "high risk" acute lymphoblastic leukemia were treated with a protocol including VM-26, ARC and intermediate-dose Mtx. Complete remission was obtained in 94 por 100 of patients. Actuarial haematologic remission rate at 36 months is 87% and continuous complete remission rate 58%. Twelve doses of Mtx (IV + IT) appear to be insufficient as SNC prophylaxis in these high risk children. Nevertheless hematologic relapse rate is very low. Mean follow-up time is 31 months and survival rate 87%.

[131I-MIBG-meta-iodobencylguanidine (131I-MIBG) in the study of neuroblastoma]. / Gammagrafía con 131I-MIBG-metaiodobencilguanidina (131I-MIBG) en el estudio del neuroblastoma.

Ten patients with neuroblastoma were scintigraphed with I131-meta-iodobenzylguanidine (MIBG). Lugol solution was previously administered orally to all patients in order to avoid uptake of radioactive iodide by the thyroid gland. The compound was injected intravenously, 0.5 mCi/1.73 m2 of body surface. Scintigraphy was performed at 24, 48 and 72 hours and 6-7 days after administration of the radioactive preparation. Positive radiotracer uptake was demonstrated in all primitive tumors and metastases, showing a positivity of 100%. MIBG has proven to be the most useful technique in defining the stage of the disease. Mechanism of the tumoral uptake in relationship to catecholamine metabolism is discussed. Differences observed in MIBG radioactivity seen in the urinary bladder, heart and liver in children with or without. Neuroblastoma are discussed. MIBG has also been useful in the follow-up of patients with neuroblastoma.