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Cerebral and meningeal involvement in patients with primary extracranial neuroblastoma (NB) is unusual although it is generally present in disseminated disease. The intensification of chemotherapy that has prolonged survival in these children has changed the pattern of relapse presentation, as occurs with isolated central nervous system (CNS) disease. We report 4 patients with secondary CNS metastases. Three infants of 16, 14, and 10 months of age, diagnosed with primary abdominal NB stage 4, presented neuromeningeal metastases during maintenance chemotherapy with seizures and cranial hypertension as the first manifestation. Another 8-year-old patient diagnosed with NB stage 3 presented local relapse with later neuromeningeal metastases. All died in the following 3 months. The possibility of CNS relapse in patients with NB should be considered when neurological symptoms and signs appear. These new relapse forms overshadow the prognosis of these children.

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From October 87 to April 92, 172 children were admitted in the N-I-87 protocol of the Spanish Society of Pediatric Oncology for the diagnosis and treatment of neuroblastoma. Forty-eight were considered Evans stage III, 33 of them being older than 1 year. All children were treated with induction chemotherapy (IC) and surgery. IC consisted of three courses of high-dose cisplatin-VM-26 alternating with three further courses of cyclophosphamide-doxorubicin (CAD). Infants less than 1 year received the same drugs at lower doses. After surgery, maintenance chemotherapy was administered to all children during 14 months. It consisted of four pairs of drugs rotated every 4 weeks. Radiotherapy was administered exclusively to patients older than 1 year with residual tumor after IC and surgery. Response was evaluated after IC and surgery. In children older than 1 year, response was obtained in 28/33 (88%). Fifteen of them (47%) achieved complete remission (CR), seven (22%) good partial response (GPR), six (19%) partial response (PR); and in three patients (9%) there was progressive disease (PD). Actuarial survival at 48 months was 0.60 +/- 0.10 and EFS was 0.61 +/- 0.12. Audiologic impairment was considered the worst toxicity. In children less than 1 year the response rate to IC and surgery was 93% (14/15); nine infants obtained complete response and four had GPR. Only one patient experienced PD in the first 6 months of therapy and died. The other 14 are alive and well at a mean follow-up time of 48 months. Chemotherapy toxicity was mild and reversible.

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Megaloblastic anaemia is very rare in the first weeks of life and it is related to impaired metabolism of folic acid or vitamin B12. One of this disorders is the congenital transcobalamin II deficiency. The case of a three month old infant, with vomiting, diarrhoea and severe anaemia is presented. Both parents and the child had very low or undetectable levels of serum TC II, respectively. Using i.m. hydroxycobalamin at high doses, the clinical and laboratory responses have been satisfactory.

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From 1972 to 1986, a total of 46 children with the histological diagnosis of Non-Hodgkin's lymphoma were treated in our hospital. Twelve had emergency surgery. Nine of them presented with an acute abdominal condition and three bowel perforation during treatment for childhood Non-Hodgkin's lymphoma. Survival rate was 58%.

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The authors reviewed 92 patients diagnosed as ALL. Age range was 1 to 12 years (x = 5 years), 50 males and 42 females. The patients were classified as high risk, intermediate and low risk according to the following prognostic factors: sex, age at diagnosis, visceromegaly, adenopathy, initial WBC, HB, immunoglobulins, and platelets, FAB morphological classification, and bone marrow response to initial therapy, immunological markers, CNS infiltration and mediastinal mass. The previous factors were analyzed individually and also the results obtained with each risk group according to the treatment applied.

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From 1972 to 1985, a total of 35 children with histological diagnosis of non-Hodgkin's lymphoma were treated. A previously undiagnosed abdominal non-Hodgkin's lymphoma produced intussusception in three of these children between 5 and 10 years of age. Our experience indicates that any child around 6 years of age with abdominal pain, bloody stools and a palpable mass plus radiographic evidence of intussusception should be considered to have an intestinal wall lymphoma until proven otherwise. Inadequate surgical treatment may result from failure to recognize relationship between intussusception and lymphoma in older child.

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Eighteen cases of histiocytosis X are presented and prognostic factors, such as localized or disseminated disease, organ dysfunction and age, are analyzed in relation to results of therapy. A new classification of childhood histiocytosis X, based on prognostic factors, seems to be justified.

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Two cases of congenital fibrosarcoma are presented. Both tumors showed histological features of malignancy, but were successful treated with simple resections. Recurrent tumor does not seem to predispose to metastatic disease. Local excision is the initial treatment of choice for lesions occurring in newborns and infants.

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A case of Niemann-Pick's disease is described. This finding is the result of infant's interstitial pneumonitis study. Anatomopathological and enzymatic results are showed.

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The results of 14 cases of Wilms tumor, treated according to the SIOP, nephroblastoma trial and study protocols, are presented. Later, an up to date review of the state of knowledge on the problem is done, mainly in relation to histology, treatment and prognosis.

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Eighty four cases of meningococcal infections are reviewed. Fifty seven cases presented themselfs as meningococcal meningitis, twelve cases as sepsis with moderate hypotension and 15 cases were sepsis with septic shock. A brief course of the disease, shock, echymosis, absence of meningeal signs, leucopenia and intravascular coagulation were findings more frequent in the group of patients with hiperacute sepsis, whereas other signs as fever, headaches, vomiting and petechiae were present with equal frequency in the three groups. N. meningitis was isolated in 73% of the cases. Shock (18.85%) and intravascular coagulation (12%) were the complications more frequently found, followed by convulsions (4.81%), arthritis (4.81%), skin necrosis (4.81%), subdural efusion (3.57%), cerebral palsy (3.40%), thrombophlebitis (1.20%), recurrence (1.20%), inapropiate antidiuretic hormone secretion (1.20%) and subaracnoideal hemorrage (1.20%). The overall mortality was 10.70% and 60% of the patients which initially presented with shock and intravascular coagulation died. Autopsy findings included wide spred hemorragic lesions and intravascular thrombi in skin, mucous membranes and viscera. Adrenal hemorrhage was present in five of the six cases studied.

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Ten two to ten weeks old children are presented with urinary tract infection and jaundice. In six cases "E. coli" was the causative organism. Laboratory data seemed to indicate that hemolisis was an important factor in the pathogenesis of jaundice. Treatment with appropriate antibiotics was favorable in all cases. Urinary tract infection with bacteriemia and jaundice is probably the most frequent cause of jaundice among children of two to ten weeks of age.

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