[Hepatoblastoma today. Our experience]. / El hepatoblastoma hoy. Casi a nuestro alcance.

UNLABELLED: Prognosis of hepatoblastoma in children has improved in the last few years particularly because of preoperative chemotherapy and total surgical removal. The aim of this study was to present the evolution of HB therapy at our centre. Since 1966, 44 patients have been treated. The first 20 cases (group I) were treated with chemotherapy alone. The next 19 patients (1975-1989) (group II) underwent preoperative chemotherapy and delayed surgery. After 1990, 5 patients (group III) were treated with protocolized preoperative chemotherapy and accurate surgery (SIOPEL-1). RESULTS: survival rates for the three groups were: 0%, 15% and 100%, respectively. CONCLUSIONS: HB differs from hepatocellular carcinoma pathologically and in onset of symptoms and prognosis. SIOPEL-1 results showed that preoperative chemotherapy aids tumour involution and therefore facilitates resectability and that accurate surgical technique permits safe removal of huge masses. Acceptance of these pillars for treatment of HB is currently essential for paediatric surgeons.

[The first year of life of children with myelodysplasia: a multicenter study of 393 cases]. / El primer año de la vida del niño con mielodisplasia. Estudio multicéntrico sobre 393 casos.

A multicentric study about the first year of life of myelodisplastic children, was done. Family, pregnancy, delivery, newborn somatometry, and anatomical-pathological and terapeuthical aspects were review in 393 patients born before july 1992. Annual prevalence vary from 6 (1973) to 28 (1984). The families clinical history was not significant. There were 9 (2,3%) gemelar pregnancies, but both children were affected only in one case. Prenatal ultrasound was not diagnostic in 61%-84% of patients are meningomyeloceles, the anatomical level of myelodisplasia was lumbar and or sacral in 98%. There was hydrocephalus in 59%, hip dislocation in 24%, feet deformities in 50%, upper urinary tract dilatation in 12%, vesicoureteral reflux in 15%. In total 825 surgical procedures were performed (M = 2); the neurosurgery was 94% of them.

[Spina bifida: management after 25 years]. / Espina bífida. Planteamiento después de 25 años.

The authors present their experience of 468 patients with spina bifida treated in a period of 25 years. They discuss the philosophy of the management of the severe neural tube defects based in the follow up of these patients. This attitude is based in the prevention that could be: 1) following Smithells theory; 2) trying a prenatal diagnostic before 20 weeks of pregnancy, and 3) doing a selection of spina bifida that could be operated.

[Thyroid cancer in childhood: review of 7 cases]. / Carcinoma de tiroides en la infancia: revisión de 7 casos.

Thyroid carcinoma in childhood is a rare, but nevertheless malignant disease, with a high incidence of nodal metastasis. Seven children, 6 boys and 1 girl, aged 4 to 9 years with thyroid carcinoma were treated at the Hospital Infantil Valle de Hebrón of Barcelona between 1965 and 1990. The diagnosis was established by cervical nodal biopsy in all cases except one. Papillary carcinoma was found in 4 cases, follicular type in 2 cases and one case of mixed carcinoma was diagnosed. Surgical treatment consisted in lobectomy on the affected side, followed by total thyroidectomy on the opposite side and bilateral resection of involved nodes one or more times. In two patients, treatment with sodium-iodine I131 showed active tissue in the thorax and wound. Both were treated effectively. All patients are alive without recurrences during a follow-up period of 1 to 23 years. Main complications were: temporary recurrence of the nerve injury in two patients and a controlled hypoparathyroidism in another two cases. In all cases, treatment with L-thyroxine was required. Thyroid carcinoma in children carries a considerably better prognosis than in adult patients. We must suspect thyroid carcinoma and indicate biopsy in all cases of cervical nodes resistant to antibiotic treatment. Early diagnosis, total thyroidectomy, radioiodine treatment and hormone administration permit radical cure even in the case of metastatic disease.

[Anomalous course of a lipomeningocele]. / Anómala evolución de un lipomeningocele.

A 9 months old male with a lipomeningocele developed progressive flaccid paraparesis with loss of deep tendon reflexes and sensation to pink prick on the lower extremities. The clinical picture was secondary to a tethered spinal cord syndrome associated to the lipomeningocele. Surgical untethering of the spinal cord was followed by clinical recovery over a 3 months period.

[Neoplasms during the 1st year of life: a study of 116 cases treated during the last 10 years]. / Neoplasias en el primer año de la vida: estudio de 116 casos tratados en los ultimos diez años.

116 infants under one year of age (14 per 100) with malignancies including 35 neuroblastomas, 23 retinoblastomas, 14 Wilm's tumours, 10 hepatoblastomas, 10 brain tumours, 9 germ cell tumours, 8 histiocytic and 7 soft tissue sarcomas, were treated in the last ten years. Hepatoblastoma is the highest relative incidence tumor in the first year of life and with brain tumours has the worst prognosis (50 and 40 per 100, respectively). The disease-free survival rate is most than 80 per 100 in neuro and nephroblastoma with medical and surgical treatment. Familiar incidence in Wilm's tumor is 42 per 100 and chemotherapy side effects are 23 per 100. Familiar incidence in neuroblastoma is 20 per 100 and chemotherapy side effects 21 per 100. In conclusion, in children with malignancies under one year of age have good prognosis (75 per 100 survival at five years), with lower surgical complications rate. Early diagnosis and response to chemotherapy, the side effects of which are considerable and should be strictly controlled, are key factors in the better prognosis and increased life expectancy in this group of patients.

[A little known entity: aggressive fibromatosis]. / Una entidad poco estudiada: la fibromatosis agresiva.

Aggressive fibromatosis is an unfrequent and little known entity, which in spite of being a histologically benign tumoration with scarce mitosis and without metastasis at distance, frequently presents with a high degree of local malignancy that can cause serious functional and aesthetical disturbance for the patient and even lead to death if infiltration of vital organs is presented, above all in cases of abdominal or maxillo-facial mass localization. The authors present their experience with 17 cases of aggressive fibromatosis observed in our centre: four of abdominal localization, six in extremities, five in the maxillo-facial mass, one in the torax and one in the lumbo-sacral region. Histological diagnosis, either by puncture or biopsy, is complemented by studies of extension of the tumour based on ecography and TAC. All cases were treated according to the classical criteria of ample resection of the lesion, always when practicable, except in one infant case and in the torax, in which only a biopsy was effected. Of the 15 cases resected, nine cases had local relapses, six of which remained free of disease with a second operation, another two required a third operation and the remaining case needed five interventions. In six children chemotherapy was applied with vincristina, cyclophosphamide and adriamicina. A follow up was carried out in 14 patients, one of which died and the remaining 13 are free of disease. In spite of the fact that progestagene receptors were not evidenced in two of our cases, one presented complete remission of the tumor after treatment with medroxyprogesterone. In this case the coincidence of Gardner's syndrome arises in the family history.(ABSTRACT TRUNCATED AT 250 WORDS)

[Surgical treatment in tumors of the anterior mediastinum]. / Tratamiento quirurgico en los tumores de mediastino anterior.

Anterior mediastinal tumours (A.M.T.) suppose a diagnostic problem owing to nonspecific clinical signs and symptoms. In the last twenty years, we have seen ten patients with A.M.T., 50 per 100 of which were fortuitous radiologic findings and the remaining 50 per 100 were diagnosed from nonspecific symptoms. Diagnostic imaging techniques were used to classify the anterior location of the tumours. Posterolateral thoracotomy was performed in the first six patients and sternotomy in the remaining four. In conclusion, sternotomy provides a wider surgical field and is to be recommended in A.M.T. surgery. Low morbidity and mortality support an early decision for thoracic surgery.

[Embryonal fatty tumors. Lipoblastoma-lipoblastomatosis]. / Tumores de grasa embrionaria. Lipoblastoma-lipoblastomatosis.

The authors present four cases. The first case is a diaphragmatic-mediastinal, the second one is a cervical-mediastinal one, the third case has a buttock, the last one is a case of hip-thigh. Their ages are one, eight and three years old and the fourth case is seven days old. They appear in axilla, neck, thoracic wall and fat prevertebral tissue, in below three year old children. Despite their benignity, the tumors grow very fast. The complete extirpation is fundamental to avoid relapses, which are presented approximately in a 15 for 100 of the cases. Mutilations are not necessary, since it is a benign tumor. Finally, the authors amply review the literature. They differentiate the concepts of lipoma, hibernoma and lipoblastoma.

[The prevention and prenatal diagnosis of neural tube defects]. / Prevención y diagnóstico prenatal de los defectos del tubo neural.

The AA. provide with the schemes for the study of Neural Tube Defects (D.T.N.) yet the continuation and the prevention of NTD'S clinical cases. Also, the AA. show a protocol of action. It is indispensable for all the Spina Bifida Sections to work together by means of an Orthogenesis and Familiar Planning Unit.