An interview with Purificación García de Miguel, chief of Haematology-Oncology and the Paediatric Bone Marrow Transplantation Unit of the Hospital La Paz, Madrid, and President of the Spanish Society of Paediatric Oncology (SEOP). Interview

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[Respiratory function in pediatric survivors of malignant neoplasms]. / Función respiratoria en niños supervivientes de neoplasia maligna.

AIM: To evaluate post-treatment pulmonary function in patients with malignant extrapulmonary neoplasia and its relationship with age, type of neoplasty and treatment received. METHODS: Cohort study of 95 pediatric patients after chemotherapy with or without surgery or extrapulmonary thoracic radiotherapy. The patients were in remission without treatment and able to undergo pulmonary function testing. Personal history and exposure to risk factors or toxic habits were evaluated. Clinical examination, chest radiographs, pulmonary gammography, basal and stress pulmometry, forced spirometry, whole body phletismography and carbonmonoxide transfer test were performed. RESULTS: Mean age at diagnosis was 5 +/- 3.3 years. Treatment duration was 2.4 +/- 1.3 years and time without treatment 4.3 +/- 3. 3 years. Thirty-six patients were reviewed two and a half years after the first control. Thirty-nine patients had acute lymphoblastic leukemia and 57 had solid tumors. Sixty percent showed functional restriction due to chest deformity after undergoing chest surgery. The association between functional restriction and radiotherapy (6 patients) did not produce poorer functional results. Forty-three percent showed initial change in TLCO. Nineteen percent showed basal hemoglobin saturation under 93% which in 16% fell after physical exercise. These alterations improved with time. However, restrictive change initially present in 11.5% persisted at the second evaluation. Thoracic surgery was the main cause of thoracic deformity and therefore of restrictive change. The children under 8 years old and those who received longer treatments tended to show the worst TLCO values. The patients with neuroblastoma showed greater restrictive change while the change in TLCO was more frequent in patients with Burkitt s lymphoma and in those treated with cyclophosphamide. CONCLUSIONS: Neither personal or family history of respiratory disease nor the presence of symptoms such as cough served to identify risk of functional change. Restrictive change in pulmonary function was greater in patients who had undergone thoracic surgery. Functional values were worse in patients with neuroblastoma. Pulmonary function should be followed up in pediatric survivors of malignant neoplasia in order to prevent restrictive alterations.

Función respiratoria en niños supervivientes de neoplasia maligna / Pulmonary function in pediatric survivors of malignant neoplasty

OBJETIVO: Valorar la función pulmonar postratamiento, en pacientes que padecen neoplasias malignas extrapulmonares y su relación con la edad, tipo de tumor y tratamiento recibido. MÉTODOS: Estudio de una cohorte de 95 pacientes pediátricos tras recibir tratamiento quimioterápico con o sin cirugía o radioterapia torácica extrapulmonar, en fase de remisión sin tratamiento, y capaces de colaborar en la realización de las pruebas de función respiratoria.Se valoraron antecedentes personales y de exposición a factores o hábitos nocivos. Se realizaron controles clínicos, radiografía de tórax, gammagrafía pulmonar, pulxiosimetría basal y de esfuerzo, espirometría forza-da, plestimografía corporal total y transferencia de CO (TLCo). RESULTADOS: Los pacientes presentaron una edad media al diagnóstico de 5 ñ 3,3 años, duración del tratamiento de 2,4 ñ 1,3 años y un tiempo sin tratamiento de 4,3 ñ 3,3 años; 36 de ellos fueron revisados dos años y medio después del primer control; 39 correspondieron a leucemia linfoblástica aguda y 57 a tumores sólidos. El 60 por ciento de los que fueron sometidos a cirugía torácica presentaron deformidad responsable de restricción funcional. La asociación de ésta con radioterapia (6 pacientes) no condicionó peores resultados funcionales. El 43 por ciento presentaron inicialmente alteración de la TLCO, el 19 por ciento SatO2 basal inferior al 93 por ciento y el 16 por ciento un descenso de ésta con el ejercicio. Estas alteraciones mejoraron con el tiempo. Por el contrario, la alteración restrictiva que estuvo presente inicialmente en el 11,5 por ciento persistió en el segundo control. Los pacientes exclusivamente irradiados no presentaron alteración restrictiva con más frecuencia. Los menores de 8 años y los que sufrieron tratamientos más prolongados tendieron a presentar peores valores de TLCO. Los neuroblastomas presentaron mayor alteración restrictiva, mientras que la alteración de la TLCO fue más frecuente en los linfomas tipo Burkitt y en otros pacientes tratados con ciclofosfamida. CONCLUSIÓN: Ni los antecedentes personales, los antecedentes familiares de enfermedad respiratoria ni la presencia de síntomas como la tos sirvieron para identificar el riesgo de alteración funcional. Los pacientes sometidos a cirugía torácica presentaron patología restrictiva; su asociación con tratamiento radioterápico no pareció influir negativamente. Los neuroblastomas presentaron los peores resultados respecto a otro tipo de tumores. Es necesario hacer un seguimiento respiratorio funcional en estos niños a fin de prever posibles secuelas restrictivas (AU)

Single-centre experience of peripheral blood stem cell transplantation using cryopreservation by immersion in a methanol bath.

A simplified method to remove and cryopreserve peripheral blood stem cells (PBSC) was utilised to restore the bone marrow in 31 patients with haematological or solid neoplasms after ablative chemotherapy. Mobilization was performed with subcutaneous G-CSF, starting 4 days before the first PBSC harvest and continuing to the last day of harvest. Cryopreservation was carried out by freezing cells to -80 degrees C after addition of autologous fresh plasma with DMSO, in a methanol bath and non-programmed freezer. The PBSC were reinfused in all cases. The mean quantity of CD34 cell (x 10(6)/kg) infused was 6.5 +/- 6.7. The mean number of procedures needed to harvest an appropriate number of PBSC was 3.6 +/- 1.3. The mean times necessary to recover more than 0.5 x 10(9)/l granulocytes were 11 +/- 4 (8-30) days and 23 +/- 13 (8-55) days to obtain more than 20 x 10(9)/l platelets. These results confirm our method as very effective in achieving a high-quality harvest, and it was used in paediatric and adult patients without problems. This procedure, using a non-programmed freezer, simplifies and reduces enormously the cost of the technical measures currently used, enabling their adoption in almost any clinical oncological institution.

Balanced reciprocal translocation (X;20) limited to Wilms' tumor in a Wiedemann-Beckwith syndrome.

A girl aged 4 years 3 months with sporadic unilateral Wilms' tumor associated with Wiedemann-Beckwith syndrome, but without aniridia, was found to have a t(X;20) in the tumor cells. Karyotypes of peripheral blood of the patient and her parents were normal. This translocation was confined to the tumor and not been previously reported either in nephroblastoma or any other neoplastic processes. Although there is no microscopic deletion on chromosome 11 and catalase activity was not decreased, we cannot rule out the possibility of a point mutation or a submicroscopic deletion.

[Sacrococcygeal tumors. Experience of 42 cases]. / Tumores sacrocoxígeos. Experiencia en 42 pacientes.

From 1966 to 1981, at "La Paz" Children's Hospital, Madrid, 42 patients with ST were studied. We exclude 4 stillborn, the remaining 38 underwent 40 surgical operations, 69% in the first week of life. The aim of the study is to classify and organize from the clinical and pathological points of view the usual equivocal terminology. From a clinical point of view, sex, localization and age at diagnosis were the usually described for these tumors. The relationship of age with malignancy is pointed out. Special emphasis is made on the pathological information about undifferentiated blastodermic tissues, like neuroblasts pointing out their low influence in prognosis. 79% were mature teratomas and 21% malignant tumors (Yolk sac and embrional carcinoma).

[Rhabdomyosarcoma in childhood. Report of 27 cases (author's transl)]. / Rabdomiosarcoma en la edad infantil. A propósito de 27 casos.

Twenty-seven cases of rhabdomyosarcoma in children between one month and 14 years of age are reported. The most frequent histologic type was the embryonal sarcoma and almost half of the cases were diagnosed in advanced clinical stages. Treatment consisted on the combination of surgery, radiotherapy and chemotherapy (vincristine, cyclophosphamide and actinomicin-D in non metastatic cases, plus adriamycin when metastases were present). The survival rate is 57.7% and the survival with no evidence of disease 53.8%, with observation periods from eight months to four years.

[Hodgkin's disease in small children (author's transl)]. / La enfermedad de Hodgkin en el niño en edad preescolar

17 cases of Hodgkin's disease are reported, seen in a ten year period. Age of patients oscillated between three and seven years. There was a predominance of mixed cellularity type in histologic pattern. Most of patients were diagnosed in advanced clinical stages. Primary sign of disease was presence of cervical adenopathies and there were few cases of thoracic disease. Treatment consisted in radiotherapy and chemotherapy, according to histological type and clinical stage. Survival in our serie is of 53.3% with periods of observation from six months to seven years.