Calidad de vida y funcionalidad en pacientes pediátricos intervenidos de tumores óseos en miembros inferiores: cirugía reconstructiva versus amputación / Quality of life and functionality in pediatric patients operated on for lower limb bone tumors: limb salvage surgery versus amputation

Objetivo. Analizar las diferencias en cuanto a funcionalidad y calidad de vida relacionadas con la salud en pacientes intervenidos de un tumor óseo maligno en edad infantil según el tipo de cirugía practicada. Material y método. Estudio descriptivo transversal de pacientes mayores de 14 años tratados por osteosarcoma o sarcoma de Ewing en miembro inferior y que recibieron tratamiento quirúrgico en edad infantil tras una evolución mínima de 2 años. Se realizó una evaluación de funcionalidad y calidad de vida utilizando las escalas de Enneking y Medical Outcomes Study Short-Form 36. Comparamos los resultados funcionales según la técnica quirúrgica. Resultados. Nuestra muestra quedó formada por 17 pacientes (8 hombres y 9 mujeres) con una edad media actual de 22 años. El tumor más frecuente fue el osteosarcoma, y la localización, el tercio distal del fémur y proximal de la tibia. En relación a la técnica quirúrgica, en 8 pacientes se colocó endoprótesis, 4 se trataron mediante injerto y/u osteosíntesis y 5 fueron amputados en cirugía inicial. Cinco pacientes tuvieron que ser amputados en un segundo momento debido a complicaciones. La puntuación expresada en mediana y percentiles obtenidos en la escala de Enneking sobre la totalidad de la muestra fue del 83,33% (66,66-88,33%). En pacientes no amputados fue del 83,33% (73,33-86,66%), frente al 90% (80-93,33%) en pacientes amputados inicialmente. En los que se realizó amputación posterior tras complicación, la puntuación fue de 56,66% (51,66-73,33%). Conclusiones. En nuestra muestra no encontramos diferencias significativas en cuanto a funcionalidad y calidad de vida de los pacientes tratados mediante cirugía reconstructiva en comparación con amputación inicial, aunque sí peores resultados en los pacientes que sufren una amputación tardía debido a una complicación tras cirugía de salvamento inicial (AU)

Objective. To analyze the differences in functionality and quality of life related with outcome of a malignant bone tumor intervention in childhood age according to type of surgery performed. Material and methods. A descriptive, cross-sectional study of patients over 14 years treated for osteosarcoma or Ewing's sarcoma in lower limb during childhood after a minimum of two years evolution. Functionality and quality of life were evaluated with the Enneking and Medical Outcomes Study Short-Form 36 scales. The functional results were compared according to surgical technique. Results. Our sample consisted of 8 men and 9 women, with a current average age of 22 years. The most common type of tumor was osteosarcoma and most common location was distal third of the femur and proximal tibia. In relation to the surgical technique, stent was placed in 8 patients, 4 were treated by graft and/or osteosynthesis and 5 patients underwent amputation as initial surgery. Five more patients had to under amputation at a second time due to complications. The score, expressed as median and percentiles obtained in the scale of Enneking on the entire sample, was 83.33% (66.66%-88.33%). In non-amputees, it was 83.33% (73.33%-86.66%), compared with 90% (80%-93.33%) in patients who were initially amputees. In those in whom the amputation was performed later after a complication, the median Enneking score was 56.66% (51.66%-73.33%). Conclusions. In our sample, we did not find significant differences in terms of functionality and quality of life of patients treated by reconstructive surgery compared with initial amputation although there were worse results in those patients who underwent a later amputation due to a complication after the initial life-saving surgery (AU)

Successful treatment of orbital rhabdomyosarcoma in two infants using chemotherapy alone.

Two infants, 2 months and 13 months of age, were found to have orbital embryonal rhabdomyosarcomas (ERMS). Because of the adversities associated with either surgical exenteration or curative doses of radiation therapy, they were treated with chemotherapy alone. They survive disease-free 5 and 9 years after diagnosis with excellent cosmesis and normal vision. This approach may be suitable for similarly small (< 2 cm in diameter) ERMS in other sites.

[Ewing's sarcoma with primary localization in the skull: apropos of two cases]. / Sarcoma de Ewing de localización primaria en calota craneal: a propósito de dos observaciones.

Two cases of Ewing's sarcoma primary found in the skull are presented. Both started with neurologic symptoms, the first case with intracraneal hypertension syndrome, and the second case with Jackson-like crisis. We emphasize the rareness of the initial localization, as well as the favorable evolution of both cases after surgery and radiotherapy and chemotherapy treatment.

Preservation of anaplastic features in Wilms' tumors after preoperative chemotherapy.

In 4 of 35 childhood Wilms' tumors on record in the Hospital Infantil Virgen de Rocio de Sevilla, it was possible to identify diffuse anaplasia despite the fact these children had received cytoreductive chemotherapy preoperatively.

Maturation of Wilms' tumor pulmonary metastases to benign fibromas after therapy.

A 3-year-old girl with Wilms' tumor developed pulmonary metastases 5 months after diagnosis. All but one of the visible lesions disappeared after thoracic irradiation and multiple-agent chemotherapy. Subsequent thoracotomy revealed 17 small nodules in addition to the visible lesion, and all of them proved to be fibromas. It would appear that the Wilms' tumor metastases to the lung were made to mature to benign neoplastic processes after the treatments given.

[Treatment of rhabdomyosarcoma of the bladder without radical surgery]. / Tratamiento del rabdomiosarcoma de vejiga sin cirugía radical.

Authors present two cases of rhabdomyosarcoma of the bladder treated previously to surgery with chemotherapy (protocol T-2) and radiation therapy. In one of these patients surgery consisted only in a partial cystectomy, and in the other only a few biopsies were taken from the most suspicious zones, and these were all negative. Three years and four months after diagnosis and 15 months after completion of treatment, both children are alive and free of detectable disease.

[Tumor committee and pediatric oncology. Importance for management of infantile neoplasias (author's transl)]. / Importancia de la Sección de Oncología Pediátrica y Ccomisión de Tumores en el tratamiento de las neoplasias infantiles

The organization for the diagnosis, treatment and follow-up of the children with malignancies at the Hospital Infantil "Virgen del Rocio", are described. There is a Tumor Committee made up by two pediatrics oncologist, two pediatrics surgeons, one pathologist, one radiotherapist, one pediatric psiquiatrist and one social worker. The Oncology Section of the Hospital is made up by one pediatric oncologist and one registerers nurse (A.T.S.) and several clinical assistants.

[Correlation between scanning, Radiological images and clinical findings in tumoral processes (author'a transl)]. / Correlación entre la gammagrafía ósea, la radiología y la clínica en los niños con patología tumoral

During last years bone scanning has shown a great accuracy in the diagnosis of primitive bone tumors and bone metastasis in adult patients. With the use of short lived tracers this technique has also been used in Pediatrics. The authors present their experience in the evaluation of bone scanning in 31 patients with different tumoral processes doing a correlation between scanning, radiological images and clinical findings. The technique is presented for discussion showing its possibilities, limitations and future of its application in Pediatrics.

[Histiocytosis management (author's transl)]. / Tratamiento de la histiocitósis.

Seven cases of Histiocytosis have been treated with VB and PR. Five of them that belong to grup C (according to Oberman's classification) experimented favorable resuts. Likewise, we have been able to prove that prognosis of these children depend on: their age at the onset of the disease; the original site of the disease; the extension of the lesions and the maintenance of prolonged therapy.