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We report a restrospective study about 11 patients (9 girls) referred from 1980 through 1994. We undertook this study to determine the efficiency of several methods for diagnosis and treatment. We performed ultrasound, computerized tomography (CT) in each one and magnetic resonance (MR) in 4 cases. Six patients were prescribed steroids, 4 cases were not treated and one patient was operated. A solid lesion with variable ecotexture is noted at ultrasound. CT typically shows a low-attenuation solid lesion with peripheral enhancement. At MR imaging performed with T2-weighted pulse secuences the lesion usually has high signal intensity. We obtained completed remission in 5 cases, one patient was out of control and in the other 5 the sonography showed marked regression in tumor size. We believe CT is an specific method for diagnosis hemangioendothelioma. Sonographic control or steroids must be the initial therapy.

Since Gross established the basic rules for nephrectomy in Wilms' tumour (WT) in 1953, the management of nephroblastoma has been more straightforward. However, some cases with intravascular involvement, currently detected by ultrasound (US), may represent a daunting challenge for the surgeon. Inferior vena cava with tumour thrombus, induced by WT, can be asymptomatic and, if undetected, can contribute to poorer prognosis for two main reasons: possible neoplastic cells inside the thrombus and higher morbidity risk of surgery. From 1979 to 1993, 81 WT were studied by routine US. Intracaval thrombosis was diagnosed in four (5%), in one of which the thrombus extended to the right atrium. In our experience, the surgical strategy in each of the four cases (100% survival) depended on the length of the thrombus and whether or not it infiltrated the vena cava wall. If the thrombus can be easily removed: complete resection. However, in cases of atrial thrombus, and more particularly if the thrombus involves the intima, we suggest the thrombus not be touched since the problem may be solved by preoperative and postoperative chemotherapy. Thus the favourable prognosis would be maintained and superfluous risky surgery avoided.

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.

This retrospective study compares the results obtained in 56 children with medulloblastoma treated with two different protocols at our center between 1975 and 1990. Since 1985 we have been enrolled in the medulloblastoma SIOP II protocol in which we have entered 27 patients. These patients from the SIOP group (SG) and the 29 children treated before 1985, the historical group (HG). When the two groups were compared for age and sex distribution, no differences were found; however, prognostic factors were worse in the HG than in SG: 21 versus 15 T3-T4 and 13 versus 8 high-risk, respectively. The relapse rate was 63% for the HG and 39% for the SG. Five-year disease-free survival was 31% for the HG and 56% for the SG (p = 0.037). Five-year survival was 52 and 70%, respectively (p = 0.055). When SG and HG were compared by stratifying for tumor size, surgical resection and the risk variable, better disease-free survival was obtained in SG than in HG. In a multivariate analysis, the HG and the high-risk proved to be variable independent predictors of poor survival. We observe an increase in survival in our patients with medulloblastoma.

Since Gross established the basic rules for nephrectomy in Wilms' tumour (WT) in 1953, the management of nephroblastoma has been more straightforward; however, some cases with intravascular involvement, currently detected by ultrasound (US), may represent a daunting challenge for the surgeon. Inferior vena cava with tumour thrombus induced by WT can be asymptomatic, and if undetected, contribute to poorer prognosis for two main reasons: possible neoplastic cells inside the thrombus and higher morbidity risk of surgery. From 1979 to 1993, 81 WT were studied by routine US. Intracaval thrombosis was diagnosed in four (5%), in one of which the thrombus extended to the right atrium. In our experience, the surgical strategy in each of the four cases (100% survival), depended on the length of the thrombus and whether or not it infiltrated the vena cava wall. If the thrombus can be easily removed: complete resection. However in cases of atrial thrombus and more particularly if the thrombus involves the intima, we suggest the thrombus not be touched since the problem may be solved by preoperative and postoperative chemotherapy. Thus, the favourable prognosis would be maintained and superfluous risky surgery avoided.

Background: Childhood mortality from tumors has decreased in industrialized countries. The aim of this paper is to study the trend of mortality from tumors in children (under 15 years old) during the period 1975-1992., Methods: Using data from the Department of Health and Social Security's Register of Mortality, age-group standardized (to world population) mortality rates for each sex were analyzed. Analysis was carried out on total mortality caused by childhood tumors and on main causes of death (leukemia, brain cancer, non-Hodgkin lymphoma, bone cancer and non-specified tumors). A Poisson's regression model was used to analyze mortality trends., Results: Childhood tumors caused 8.7% of all deaths in boys and 8.5% of all deaths in girls between 1990-1992. Thus, standardized mortality rates have decreased significantly, in 1975-1977 standardized mortality rates for boys and girls were 71.20 and 68.05 respectively, in 1990-1992 these rates had fallen to 55.45 and 41.30. The annual percentage decrease in cancer mortality rates for boys was 2% and for girls was 3.1%. Main causes of deaths were leukemia and brain cancer., Conclusions: Between 1975-1992 a significant downward trend in childhood tumors mortality has been observed, more prominent in main causes of death and in girls. The principal explanation for this trend is an increased survival, consequence of changes in childhood tumors therapy.

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.

Congenital mesoblastic nephroma (CMN) is a rare renal neoplasm which Bolande differentiated from Wilms tumour given its benign nature. We describe 7 cases of CMN which have been treated over the last ten years, in order to highlight to what extent a tumour which is generally considered to be benign can have an aggressive behaviour. 5 patients were diagnosed during their neonatal period (3 of them prenatally) and 2 after the third month of life. Clinical presentation, simple abdominal radiology, echography and abdominal CT scan, which showed characteristic images, led us to a final diagnosis. Tumoral resection was carried out in the 6 typical CMN. The atypical or aggressive CMN was urgently operated under critical circumstances due to spontaneous tumoral rupture. The pathological study showed a CMN of cellular variety with a sarcomatous component. Despite adjuvant chemotherapy, there was a tumoral recurrence which required a surgical second-look and subsequent chemotherapeutical treatment. Bolande's tumour is generally benign, but there is an aggressive cellular variant that fits into the intermediate zone of a pathological spectrum. This aggressive variant have different clinical, radiological and anatomo-pathological features and therefore it involves radical surgical treatment and additional chemotherapy with a rigorous follow-up in the short and the long term.

Recurrence of Wilms' tumour after 5 years of disease-free survival is rare. We present the case of a 26-year-old man who had been diagnosed of Wilms' tumour at the age of 6 years treated by surgery, chemotherapy, and radiotherapy and who remained disease free for 8 years, after which lung metastases were detected. Second complete remission was attained with surgery and chemotherapy and 20 years after initial diagnosis he again presented with lung metastases, similarly achieving complete remission with surgery and second-line chemotherapy. The clinical and biological aspects of late metastasis in this neoplasm are discussed.

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.

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.

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)

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.

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.

We present a case of osteolytic lesions for Langerhans' cell histiocytosis with complete response to cytotoxics.

Two new cases of cystic partially differentiated nephroblastoma, which were treated by nephrectomy, are reported. Their gross appearance is undistinguishable from a multilocular cyst, with the only difference being the presence of buds of blastema within the cystic septa. While cystic partially differentiated nephroblastoma as well as multilocular cyst have never been reported to be associated with local or metastatic recurrence and therefore their treatment consists of simple nephrectomy, the presence of blastema suggests a differentiates stage of Wilms' tumor, thus demanding a close surveillance of the patient.

Authors report their experience with 33 pediatric patients with ovarian tumors seen in the last 17 years. Total mortality was 12%. The condition was beginning in 76% of cases, most of them cystic tumors, with a 100% survival rate. Only 4 patients, with malignant tumors of different histologic nature, died. Clinical features, diagnostic procedures, therapeutic approaches and outcome are analyzed. The fact that the older the patient higher the probability of harboring a malignant tumor is stressed.

From january 1982 to january 1986 we treated 23 pediatric patients who had non Hodgkin's lymphoma, with a new therapeutic protocol. This protocol is based on the LSA2 L2, modified during the induction phase with high-dose methotrexate and increasing of intrathecal therapy. Nineteen patients (83%) were in stages III and IV and the 47.4% were in stage IV. The distribution according to histology was: 10 Burkitt's undifferentiated lymphomas; 2 non-Burkitt's undifferentiated lymphomas; 5 lymphoblastic lymphomas convoluted-cells; 5 lymphoblastic lymphoma non-convoluted cells and 1 indeterminate lymphoma. The follow-up oscillated between 6 months and 4 years. The total survival rate is 82.6% with a 100% survival in stages I, II and III and 63.5% in stage IV. The survival according histology is 91.7% for undifferentiated lymphomas and 70% for lymphoblastic lymphomas; the actuarial survival rates in the two groups at 30 months are 87% and 55% respectively (p greater than 0.10). We concluded that adding high-dose methotrexate to the LSA2 L2 protocol improve the survival rate in undifferentiated lymphomas and in a less extent in lymphoblastic lymphomas, without significant toxicity. This new protocol is mainly indicated in undifferentiated and indeterminate non-Hodgkin's lymphomas.

Sixty-two patients with metastatic neuroblastoma admitted to the Hospital Infantil "Valle de Hebrón" de Barcelona during the period of October 1966-March 1985 are reviewed. The survival rate was 16% with an overall cure rate of 9.6%. The age, sex, site of primary tumor, site of metastases, and urinary catecholamine excretion were not independent prognostic factors. Patients with initial stage I, II or III and late evolution to stage IV had a worse prognostic (survival rate 13%, p less than or equal to 0.05). We compare five treatment protocols. We obtain a better response by a multichemotherapy made of cyclophosphamide, vincristine, adriamycin, DTIC combined in a sequential order with cis-platin and VM-26. The median survival for this group was 19.9 months and the survival rate 57.1% (p less than 0.001).

A review of the findings in seven cases of stage IV-S neuroblastoma, that have been observed between 1966 and 1984. Patients under one year stage IV-S neuroblastoma have a favorable prognosis; survival rate was 71%. Primary tumor in some may be relatively small. Chemotherapy and radiation therapy may not be necessary in the management of certain children.

The authors present his experience in the treatment of 8 patients with primary tumors of cervical sympathetic ganglions. The presence of a hard, painless, adherent and slow evolution latero-cervical mass is the first sign in 7 cases. The other one, a neuroblastoma, had a Horner's syndrome. The authors expose the methods of diagnostic and staging, some characteristics of surgical technique, chemotherapy treatment and follow-up methods (TAC, Gammagraphy with i-MIBG and Tumoral Markers). This imply a survival in the 100% of cases.

Four patients diagnosed of having a tumor of Bolande and treated at our institution in the past ten years are presented. In two of them, early diagnosis was aided by prenatal echographic techniques; the other two diagnoses were suspected ar three and ten days of age because of an abdominal mass. The four of them underwent a complete study which included: a plain abdominal film, an ultrasound scan and an intravenous urogram; the last three patients were also surveyed by a CT scan of the abdomen. Surgery was undertaken early on all the patients, the time of operation being in every instance within the first month of life. A laparotomy was performed and, once the contralateral kidney had been carefully examined, a nephroureterectomy was implemented, having been able to preserve in all the cases the suprarenal gland in view of the benign nature of the tumor. A histologic study confirmed the diagnosis and revealed the complete delimitation of the tumor by the renal capsule. The authors underscore the importance of this tumor's suspicion in the face of any solid renal mass detected by prenatal echography or in the neonatal period, since early diagnosis and treatment are imperative in order to prevent the ensuing complications in the rare and unfortunate cases that show a malignant tumoral trend.