Case report: pulmonary blastoma in children--response to chemotherapy.

Pulmonary blastoma (PB) is a rare primary malignancy of the lung, with about 54 cases reported in children. The tumor consists of mesenchymal and epithelial components resembling the fetal lung. It has been treated primarily with surgery and the effect of combination chemotherapy has not been systematically investigated. A 15-year-old girl with PB with metastases to bone and regional lymph node, and high levels of alphafetoprotein, is reported. A preoperative combination chemotherapy consisting of cisplatinum, etoposide alternating with iphosphamide with mesna, vincristine and epirubicine resulted in an objective response that permitted subsequent safe surgical excision of the primary tumor. This intensive combination chemotherapy should be tested in the management of advanced PB in children, as initial therapy as well as an adjuvant to surgery.

Pediatric Hodgkin's disease in Costa Rica: twelve years' experience of primary treatment by chemotherapy alone, without staging laparotomy.

This is a prospective and nonrandomized study in which 86 children with previously untreated Hodgkin's disease (HD) were clinically staged (CS) and treated with chemotherapy (CT) alone. Fifty-two (CS IA-38, IIA-7, IIB-3, IIIA-4) received six courses of cyclophosphamide, vinblastine, procarbazine, and prednisone (CVPP). Ten (CS IA with peripheral nodes) received only three courses of CVPP with a reinforcement of C on day 8. Twenty-four (CS IIIB-18, IVA-2, IVB-4) received six courses of CVPP alternating with six courses of epirubicin, bleomycin, and vincristine (EBO). Surgical staging was not performed in any patient. Two patients (CS IIIB) had partial remission and died from progressive disease. Seventy out of 86 children have not relapsed and are in complete remission with a median follow-up of 65 months (range 13-156 months); 14 children relapsed seven to 37 months from diagnosis (median 16 months); one of them (IV B) died of disease. Thirteen are in second and third remission (median 55 months). Actuarial five year survival rates and relapse-free survival rates are 100% and 90% for CS I to IIIA and 81% and 60% for CS IIIB and IV, respectively. As a result of this study, we can conclude that in developing countries most of the children with HD staged by noninvasive diagnostic techniques can be cured with CT alone as primary treatment and thus will not suffer from the late effects of radiotherapy (RT) and the morbidity of laparotomy and splenectomy. RT alone or with other CT combinations should be considered for children who develop relapse of HD.

Stage III abdominal non-Hodgkin's lymphoma in Costa Rican children: comparison of two consecutive trials of treatment.

Seventy-three patients with Stage III abdominal non-Hodgkin's lymphoma were prospectively treated following two sequential protocols (P): L278 P (group A, 33 patients) (1978-1983) and L384 P (group B, 40 patients), (1984-1991). No patient received radiotherapy. The L278 P included 7 drugs: cyclophosphamide, vincristine (VCR), adriamycin (ADR), prednisone, methotrexate (MTX), dexamethasone, and 6-mercaptopurine, given for remission induction, maintenance, and CNS prophylaxis. In the L384 P we introduced a consolidation phase consisting of intravenous MTX and citrovorum factor rescue, and IV cytosine arabinoside. VCR was also added to the monthly doses and the maintenance phase was reduced from 18 to 15 months. From January 1988 we changed ADR for epirubicin in the same doses. Prophylactic treatment of the CNS, in the L384 P, was intensified by increasing the number of doses of MTX IT in the remission, induction, and consolidation phases, and with the use of ara-C IT. Laparotomy in 50 patients allowed partial resection in 16, and second-look laparotomy was performed in 27 patients. Viable tumor was found in four patients. Three patients (G-A) died from metabolic complications and another 4 (2 G-A and 2 G-B) failed to attain CR and died. A total of 28 (85%) of 33 children of G-A and 38 (95%) of 40 children in G-B achieved CR. Five children died in remission (2 G-A, 3 G-B). Three patients (G-A) relapsed in the CNS and one (G-B) relapsed in the abdomen and died. Disease-free survival at 120 months was 70% in G-A and 84% in G-B.

[Functioning tumors of the adrenal cortex in children. Clinical and therapeutic considerations on 11 cases]. / Tumores funcionantes de la corteza suprarrenal en niños. Consideraciones clínicas y terapéuticas en once casos.

In the last 20 years, eleven children with adrenocortical functional tumors were treated in the National Children Hospital of Costa Rica. There were nine females and two males and their ages ranged from nine months to 14 years. Eleven patients had features of virilism, five had stigmas of Cushing's syndrome and three hyperaldosteronism. The clinical diagnosis was established given the symptoms, hormonal tests and radiological and imagenological studies. The histologic diagnosis was carcinoma in six by clinical picture in one, and were adenoma in four. Three patients had regional and distant metastases. Four patients with carcinoma were treated by surgery and five received chemotherapy, two of them in presurgical stage, and four received radiotherapy. Two patients with carcinoma are alive and had no evidence of tumor recurrence ten and six years after diagnosis. Five are dead, two of them after partial response to chemotherapy. The four patients with adenoma were cured by complete surgical tumor resection, furthermore one of them received chemotherapy because there was not sure of his histologic benign condition. It is necessary more studies in use of chemotherapy in treatment of this tumors but in our experience CFM, VCR, Epi and Actin is a regimen that appears to be an active combination for the treatment in presurgical stage of adrenal cortical carcinoma.

[Neuroblastoma in Costa Rica: the experience with 76 cases treated at the Hospital Nacional de Niños]. / El neuroblastoma en Costa Rica: experiencia con 76 casos tratados en el Hospital Nacional de Niños.

Between 1970 an 1988, 76 patients with neuroblastoma (39 males and 37 females) were treated at the National Children's Hospital. The children's ages ranged from 1 month to 10 years. The location of the neuroblastoma was retroperitoneal in 71% of the cases, mediastinal in 20%, in either the head or neck 4% and unknown in 5%. Twelve percent were classified as E I-II, 35% in E III, 50% in E IV and 3% in E IVS. Seventy-seven percent of the cases died and the remaining 33% are in remission, with a follow-up of 12 to 220 months. Fifty of the cases were histologically reclassified according to Shimada. Survival rates were correlated with age, histology, E classification and treatment, in order to establish prognostic factors in relation to the disease: children under 24 months have a survival rate of 43%, statistically greater than in those children older who have an 8% survival rate. Shimada's classification is useful in predicting the child's survival when under 18 months of age and with tumors with a low ICM (81% survival) and a high ICM (6%). Patients classified as E I-II have a survival rate of 74%, those in E III-38% and in E IV-4%. In relation to the chemotherapy, a ten year survival is thought to be 14% for the 22 cases in E III and IV who were given medication and 21% for the other 36 who were given three drugs.(ABSTRACT TRUNCATED AT 250 WORDS)

[Thyroid nodules in children. Experience at the National Children Hospital of Costa Rica]. / Nódulos tiroideos en el niño. Experiencia en el Hospital Nacional de Niños de Costa Rica.

Clinical charts and biopsies of 32 children with thyroid nodules were reviewed at the National Children's Hospital from 1970 to 1988. The classification between benign and malignant types was only possible by surgery and biopsy. Twenty three (72%) were found to be benign forms and nine (28%) were carcinomas (8 papillary and one follicular). Two of the carcinomas had been irradiated previously because of neuroblastoma, as well as one of the benign type who received radiation to the neck and mediastinum because of a Hodgkin's disease. No patients showed alteration in thyroid functional test (T4 and TSH). Twenty two per cent of the carcinomas and 16% of the benign forms presented higher retention in the gammagram test. Seventy eight per cent of the carcinomas and 70% of the benign types showed a normal gammagram test. Surgery in the benign cases included 10 hemithyroidectomy, 7 sub-total thyroidectomy, 3 total thyroidectomy and 3 node resection. Carcinoma cases included 6 patients with total thyroidectomy with ganglionar modified dissection in three patients; 2 hemithyroidectomy and one with sub-total thyroidectomy. Complications included 3 hypoparathyroidisms, one of them permanent, 3 transitory recurrent paresis and only one child died because of pulmonary metastasis. All carcinoma patients were treated with levothyroxine and three of them also received 1131 in order to control ganglionar metastasis. Total survival rate for carcinoma patients was 83% at 90 months. It is concluded that only with surgery it is possible to classify correctly the histological type of children with thyroid nodes. Clinical evaluation and laboratory tests are useless. However, it has not been defined how big the surgery must be.(ABSTRACT TRUNCATED AT 250 WORDS)

Treatment and outcome of undifferentiated carcinoma of the nasopharynx in childhood: a 13-year experience.

Between 1970 and 1983, 22 pediatric patients diagnosed as having undifferentiated nasopharyngeal carcinoma (UNC) were treated at the National Children's Hospital of Costa Rica; primary tumor with local extension (T3-T4) was present in 45% of the patients, and metastasis of the cervical nodes (N1-N3) in 82%. Patients were divided into groups A and B according to the type of treatment received. A description of both groups is as follows. Ten patients were treated with 5,000 to 6,000 rads tumor dose to the primary and a 5,125-rad dose was administered to the lower neck between 1970 and 1977 (group A). Six patients received postradiation therapy with cyclophosphamide and vincristine and two patients also received 5-fluorouracil. The outcome in group A was as follows: four (40%) failed to respond, six (60%) achieved full remission initially with two subsequent relapses, and the remaining four are still alive and free of relapse after 90 to 160 months (average 130) (two were treated with Co60 alone and two with chemotherapy. Twelve patients were treated with chemotherapy pre- and postradiation with CPM and adriamycin for a total of 12 months (group B) between 1978 and 1984. All (100%) achieved complete initial remission; two patients relapsed and died. Ten patients have remained in relapse-free survival for 18-67 months (median 32). Though this is not a controlled study, our experience indicates that pre- and postradiation chemotherapy is effective in prolonging the disease-free survival in UNC and may allow a decrease of the radiation tumor dose.