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The incidence of leukemia in Valencia in children zero-seven years old, during a period of ten years (1970-1979), is studied. One hundred and fifty five cases of leukemia were diagnosed on five hospitals during this time. This makes an incidence of 3.5 cases per 100,000 children and years. A light predominance of males was observed and the peak age of presentation was between two and five years. Acute lymphoblastic leukemia was the more frequent variety, followed a big distance by acute myeloid. The relation with other factors, as chromosome abnormalities, parents age, socioeconomic level, are also studied.

A series of 42 children with Wilms' tumor collected in 11 years is reported. The female-to-male ratio was 1,5:1 and 85 per cent of the patients were under 5 years at diagnosis. The majority of the tumors were Stage I (38 per cent) followed by Stages III and IV. Pathological findings following Belkwith's criteria are commented upon. The 5 year survival was 72 per cent, and tumor-free survival was 62 per cent. There was a clear relationship between survival and Stage, age and the presence or absence of intra-abdominal lymph-node spread. Efficacity of preoperative chemotherapy and its' influence on the stage of tumor at operation is discussed.

A case report of ganglioneuroma secreting catecholamines is presented. The 131-I-MIBG gammagraphy was positive. We conclude that an uptake of 131-I-MIBG is not synonimous of a malignant tumour.

We review 37 patients diagnosed of Wilms' tumor at the Clinica Infantil "La Fe", in the last ten years, looking at the misdiagnosed cases. In two children, histological study showed a multilocular renal cyst. We also report a case previously diagnosed as being a multicystic kidney; the histological study revealed, however, a cystic partially differentiated nephroblastoma. No case of extrarenal tumors was present in the misdiagnosed patients. We comment the difficulties to make a correct diagnosis when confronted with the above mentioned entities and before histological data are available.

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%.

A study is made about the normal and abnormal gammagraphic patterns with 131-I-metaiodobenzylguanidine for the diagnosis of neuroblastoma in children. The authors find 100 per 100 positive results.

Three cases of malignant hystiocytosis in infants are reported. Two of them are medullary hystiocytic reticulosis and the other one familial erytrophagocytic lymphohystiocytosis. Clinical and pathological differences and similarities between both entities are discussed. Our experience sustains the hypothesis that RHM and LEF are two forms of a same disease.

We present the cytogenetic and hematologic study of a 6-year-old child with a variant form of acute promyelocytic leukemia (M3) who died of a brain hemorrhage. The brief survival and the hyperleukocytosis (greater than 200,000 mm3) suggests a very bad prognosis in children with this variant form of M3 leukemia.

Cancer patients show an immune deficit whose beginnings are influenced to one extent or another, by two factors: the cancer itself, and the antimitotic-immunosuppressive treatment to which the patient is subjected. The immune deficit will have repercussions in the anti-infectious defense that these patients show: --Greater number of infections. --Greater severity of infections. --Tendency towards generalization and showing of septicemic states. The problem is most serious in hematological tumors (leukemia, lymphosarcoma) where the primary cause of death is infection. During the period of activity of the disease, and also in relation to the antimitotic treatment, the PMN will decrease in absolute count and will function poorly. The consequence will be a high frequency of bacterial infections, sepsis, pneumonia, skin infections, etc., predominantly caused by gram-negative germs and staphylococcus (any germ considered not to be "very virulent" can be found); and it will result in a high mortality rate. When these children are in remission or have solid tumors, the problem is not so acute, and bears more relation to antimitotic treatment and other extraneous factors (hospitalization, catheters, antibiotics, gastrointestinal ulcers...). Patients most frequently show localized bacterial, viral and protozoon infections (varicella, zooster, herpes simplex, cytomegalia, pneumocystis) because of the predominance of cellular immunity deficit.

Forty patients with primary mediastinal masses, treated at the Children's Hospital "La Fe" between 1971-1983, were review. Malignant neoplasms were the most common pathology in this series (23 cases), followed by benign lesions (10 cases), congenital malformations (five cases) and inflammatory diseases (two cases). Complementary explorations for the preoperative evaluation of theses masses is discussed. Surgical removal was carried out on all tumors. Mortality caused by performing biopsy on lymphomas was high. Two of the five congenital malformations were removed. The other three did not receive any therapy. All patients having benign tumors are alive except one who had a cardiac teratoma diagnosed at necropsy. Eleven of the patients with malignant neoplasms are alive, with a median follow-up time two years and nine months.

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.

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.