[Results of the SHOP LNHB98 (LMB89) trial in pediatric patients with B-cell non-Hodgkin's lymphoma].

Background And Objective: After the good results obtained by the Société Française d'Oncologie Pédiatrique (SFOP) regarding the pediatric B-type non-Hodgkin's (Burkitt and large B-cell) lymphoma and L3 leukemia, the Sociedad Española de Hematología y Oncología Pediátricas (SHOP) decided to use the same treatment protocol.

Patients And Method: Pediatric patients diagnosed with B-type non-Hodgkin's lymphoma without a previous history of malignant diseases were eligible for this study. They were classified in 3 groups of risk: group A (resected stage I and abdominal stage II), group B (not eligible for groups A or C), and group C (with central nervous system involvement and L3 leukemia).

[Molecular diagnosis of retinoblastoma: molecular epidemiology and genetic counseling].

Background And Objective: Retinoblastoma, a prototype of hereditary cancer, is the most common intraocular tumor in children and a potential cause of blindness from therapeutic eye ablation, second tumors in germ line mutation carriers, and even death when untreated. The molecular scanning of RB1 in search of germ line mutations in 213 retinoblastoma patients from Spain, Cuba, Colombia and Serbia, has led to the detection of 106 mutations whose knowledge is important for genetic counselling and characterization of phenotypic-genotypic relations.

Patients And Method: Mutational study (PCR-sequentiation and microsatellites analysis) in patients with retinoblastoma, from Spain, Cuba, Colombia and Serbia.

Identification of 26 new constitutional RB1 gene mutations in Spanish, Colombian, and Cuban retinoblastoma patients.

Constitutional mutations in the RB1 gene predispose to retinoblastoma development. Hence genetic screening of retinoblastoma patients and relatives is important for genetic counseling purposes. In addition, RB1 gene mutation studies may help decipher the molecular mechanisms leading to tumors with different degrees of penetrance or expressivity.

[Results of treatment with 2-chlorodeoxyadenosine in refractory or relapsed Langerhans cell histiocytosis. Study of 9 patients].

Background: To analyse the results of the treatment with 2-chlorodeoxyadenosine (2CdA) in 9 patients with refractory or relapsed Langerhans cell histiocytosis (LCH) tracted in 8 Spanish hospitals between 1993 and 1999.

Patients And Method: In the 9 patients the following data were recorded: age, sex, organ involvement by LCH, first treatment and response, dose, number of cycles and schedule of administration 2CdA, response to 2CdA treatment, toxicity, disease-free survival (DFS) and overall survival (OS).

Results: Median age was 25 years (range, 6-63).

[Rothmund-Thomson syndrome and osteosarcoma].

Background: The Rothmund-Thomson syndrome is a hereditary dermatosis frequently accompanied by less well-known non dermatologic features including osteogenic sarcoma.

Case Report: A girl developed the classical dermatologic features of the Rothmund-Thomson syndrome since the first months of life. When she was 6 years old, she suffered from painful limitation of motion of her left leg.

Long term survival in metastatic nephroblastoma. A case report.

We describe a case of metastatic nephroblastoma presenting as renal rupture. Histology was "favorable". Lung metastases were discovered early during follow-up.