[Unusual imaging findings of Ewing sarcoma in the childhood]. / Manifestaciones radiológicas infrecuentes del sarcoma de Ewing en la infancia.

OBJECTIVES: To review the cases of Ewing's sarcoma (ES) diagnosed in children at our hospital from 1995 to 2005 and to analyze uncommon imaging signs at diagnosis or during the course of the disease. MATERIAL AND METHODS: We reviewed the cases of 21 patients with ES (12 boys and 9 girls) diagnosed between the ages of 2 and 14 years. We analyzed the types of presentation, the imaging findings (plain-films, CT, and MRI), and the patients' evolution. All cases were confirmed at histological study. RESULTS: The following uncommon manifestations were found in 11 patients: 2 vertebral, one cervical, and one dorsal ES that initially manifested as spinal cord compression and as a mediastinal mass, respectively; 2 sacral ES with ascending epidural masses; 1 mandibular ES; 1 costal ES with hemorrhagic cerebral metastases; 3 extraskeletal (thigh, buttocks, and pelvis minor) ES with bone metastases at diagnosis: the first two had spinal cord and nerve compression due to vertebral metastases; 2 ES had benign-appearing radiological findings (cortical lesion of the radius with osseous remodeling of the ulna and an expanding multilocular lesion of the fibula). CONCLUSIONS: Although ES is the second most common malignant bone tumor in children, its unusual imaging findings are less well known. More than half (52.4 %) of our last 21 patients presented some of these manifestations at diagnosis or during follow-up, so radiologists should be aware of them and include ES in the prebiopsy diagnostic possibilities.

Manifestaciones radiológicas infrecuentes del sarcoma de Ewing en la infancia / Unusual imaging findings of Ewing sarcoma in the childhood

Objetivos: revisión de los sarcomas de Ewing (SE) infantiles diagnosticados desde 1995 hasta el 2005 en nuestro hospital, analizando las manifestaciones infrecuentes en el momento del diagnóstico o en su evolución. Material y métodos: estudio retrospectivo de 21 pacientes con SE (12 varones y 9 mujeres) diagnosticados entre los 2 y 14 años. Análisis de sus formas de presentación, hallazgos radiológicos (radiografía, tomografía computarizada y resonancia magnética) y evolución. Todos los casos tienen confirmación anatomopatológica. Resultados: 11 pacientes tuvieron las manifestaciones infrecuentes siguientes: 2 SE vertebrales, 1 cervical y 1 dorsal que se iniciaron con compresión medular y masa mediastínica, respectivamente; 2 SE sacros con masa epidural ascendente; 1 SE de localización mandibular; 1 SE costal con metástasis cerebral hemorrágica; 3 SE extraesqueléticos (muslo, glúteo y pelvis menor) con metástasis óseas al diagnóstico, los 2 primeros con compresión medular y radicular por metástasis vertebrales; 2 SE con manifestaciones radiológicas de benignidad (lesión cortical del radio incurvando al cúbito y aspecto insuflante multiloculado del peroné). Conclusiones: aunque el SE es el segundo tumor óseo maligno más frecuente de la infancia, sus manifestaciones infrecuentes son menos conocidas. Más de la mitad (52,4 %) de nuestros últimos 21 casos han presentado alguna de estas manifestaciones al diagnóstico o en su evolución, que deben conocerse para incluir al SE como posibilidad diagnóstica prebiopsia (AU)

Objectives: to review the cases of Ewing's sarcoma (ES) diagnosed in children at our hospital from 1995 to 2005 and to analyze uncommon imaging signs at diagnosis or during the course of the disease. Material and methods: we reviewed the cases of 21 patients with ES (12 boys and 9 girls) diagnosed between the ages of 2 and 14 years. We analyzed the types of presentation, the imaging findings (plain-films, CT, and MRI), and the patients' evolution. All cases were confirmed at histological study. Results: the following uncommon manifestations were found in 11 patients: 2 vertebral, one cervical, and one dorsal ES that initially manifested as spinal cord compression and as a mediastinal mass, respectively; 2 sacral ES with ascending epidural masses; 1 mandibular ES; 1 costal ES with hemorrhagic cerebral metastases; 3 extraskeletal (thigh, buttocks, and pelvis minor) ES with bone metastases at diagnosis: the first two had spinal cord and nerve compression due to vertebral metastases; 2 ES had benign-appearing radiological findings (cortical lesion of the radius with osseous remodeling of the ulna and an expanding multilocular lesion of the fibula). Conclusions: although ES is the second most common malignant bone tumor in children, its unusual imaging findings are less well known. More than half (52.4 %) of our last 21 patients presented some of these manifestations at diagnosis or during follow-up, so radiologists should be aware of them and include ES in the prebiopsy diagnostic possibilities (AU)

[Pleuropulmonary blastoma in children: imaging findings and clinical patterns]. / Blastoma pleuropulmonar en niños: manifestaciones clínicas y radiológicas.

PURPOSE: To describe the imaging findings, clinical presentation and follow up of pleuropulmonary blastoma (PPB) in children. MATERIALS AND METHODS: Authors present a retrospective review of three young children with pathologically proven PPB seen from 1992 to 2006 in a pediatric hospital. The imaging findings on simple views, CT and MRI are presented. RESULTS: The first patient showed two bilateral well-defined solid lung lesions on chest X-rays, with homogeneous low attenuation on CT. Patient is free of disease following chemotherapy and surgical treatment. The second patient displayed a right tension pneumothorax. After drainage, he presented on chest X-rays and CT an underlying multicystic lesion. Following chemotherapy and surgical treatment, he presented two pulmonary metastases, which were treated with chemotherapy and surgery. The patient is now free of disease. The third patient showed a complete opacification of the left hemithorax due to a massive pleural effusion; a pleuropulmonary solid mass was seen on US, CT and MRI. The disease progressed with mediastinal, orbital and abdominal metastasis. The patient eventually died. CONCLUSION: PPB is a rare chest tumor seen in young children that can present with diverse radiological findings, and sometimes can arise in congenital cystic lung lesions. CT is the gold standard technique both for diagnosis and follow-up of these tumors.

Blastoma pleuropulmonar en niños: manifestaciones clínicas y radiológicas / Pleuropulmonary blastoma in children: imaging findings and clinical patterns

Objetivos. Describir los hallazgos por imagen del blastoma pleuropulmonar (BPP) en niños, así como su presentación clínica y evolución. Material y métodos. Estudio retrospectivo de tres pacientes consecutivos con confirmación anatomopatológica de BPP, recogidos entre 1992 y 2006 en un hospital de referencia pediátrico. Se analizan los hallazgos en la radiografía (Rx) de tórax, la tomografía computarizada (TC) y la resonancia magnética (RM). Resultados. Un paciente presentó en la Rx de tórax dos masas pulmonares sólidas bilaterales bien delimitadas, que en la TC se mostraban hipocaptantes y homogéneas. Tras tratamiento quimioterápico y cirugía, el paciente permanece libre de enfermedad. El segundo paciente comenzó con neumotórax a tensión derecho. Tras el drenaje del mismo se observó en la Rx de tórax y en la TC la presencia de una lesión multiquística subyacente. Posterior a la cirugía y al tratamiento quimioterápico presentó dos metástasis pulmonares que fueron tratadas con quimioterapia (QT) y cirugía. Actualmente está libre de enfermedad. El tercer caso presentó una opacificación completa del hemitórax izquierdo por derrame pleural masivo y masa sólida pleuropulmonar vista en la ecografía, la TC y la RM. La enfermedad progresó con extensión al mediastino, la órbita y el abdomen, y el paciente falleció. Conclusiones. El BPP es un tumor pulmonar infrecuente que se da en niños, con manifestaciones radiológicas diversas, que en ocasiones se asocia a una patología pulmonar quística congénita. La TC es la técnica de elección (patrón oro) tanto para el diagnóstico como para el seguimiento de estos tumores

Purpose. To describe the imaging findings, clinical presentation and follow up of pleuropulmonary blastoma (PPB) in children. Materials and methods. Authors present a retrospective review of three young children with pathologically proven PPB seen from 1992 to 2006 in a pediatric hospital. The imaging findings on simple views, CT and MRI are presented. Results. The first patient showed two bilateral well-defined solid lung lesions on chest X-rays, with homogeneous low attenuation on CT. Patient is free of disease followingchemotherapy and surgical treatment. The second patient displayed a right tension pneumothorax. After drainage, he presented on chest X-rays and CT an underlying multicystic lesion. Following chemotherapy andsurgical treatment, he presented two pulmonay metastases,which were treated with chemotherapy and surgery. The patient is now free of disease. The third patient showed a complete opacification of the left hemithorax due to a massive pleural effusion; a pleuropulmonary solid mass was seen on US, CT and MRI. The disease progressed with mediastinal, orbital and abdominal metastasis. The patient eventually died. Conclusion. PPB is a rare chest tumor seen in young children that can present with diverse radiological findings, and sometimes can arise in congenital cystic lung lesions. CT is the gold standard technique both for diagnosis and follow-up of these tumors (AU)