Sarcoma de Ewing extraóseo / Extraosseous Ewing sarcoma

Introducción. La familia de sarcomas de Ewing incluye el sarcoma de Ewing óseo, el tumor neuroectodérmico primitivo, el sarcoma de Ewing extraóseo y el tumor de Askim (sarcoma de Ewing de la pared torácica). Los sitios más comunes de estos tumores son tórax y extremidades. Estos tumores malignos surgen del hueso o de tejidos blandos. Histológicamente, se caracterizan por alta celularidad de pequeño tamaño, redondas, azules y con tendencia a formar estructuras rosetoides. Caso clínico. Se presentó el caso de un adolescente de 14 años de edad que, tres meses previos a su ingreso, inició con cefalea fronto-parietal izquierda con irradiación hacia el globo ocular. Al examen neurológico se encontró proptosis y disminución de la agudeza visual, además de atrofia papilar. Se realizó una tomografía computarizada de cráneo, donde se identificó una lesión intraorbitaria izquierda y otra parietotemporal ipsilateral. Se realizó la exéresis tumoral, reportando positividad para marcadores de tumor neuroectodérmico primitivo, vimentina y CD99. Conclusiones. El sarcoma de Ewing es extremadamente raro a nivel de la órbita. Sin embargo, es prudente considerarlo como diagnóstico diferencial en lesiones de esta región, ya que el pronóstico depende de la precisión diagnóstica temprana.

Background. The Ewing sarcoma family includes Ewing bone sarcoma, primitive neuroectodermal tumor, extraosseous Ewing's sarcoma, and Askim tumor (Ewing sarcoma of the chest wall). The most common sites of these tumors are chest and limbs. These malignancies arise from bone or soft tissues. Histologically, it is characterized by high cellularity, small, round, blue and with a tendency to form rosette structures. Case report. We report the case of a 14-year-old patient whose evolution began 3 months before admission with left frontoparietal headache radiating to the eyeball. During neurological examination, proptosis and decreased visual acuity were found, as well as papillary atrophy. Cranial computed tomography identifed a left intraorbital injury and another ipsilateral parietotemporal. Tumor exeresis was performed, reporting positive for primitive neuroectodermal tumor markers, vimentin and CD 99. Conclusions. Ewing sarcoma is extremely rare at the orbital level: however, it is prudent to consider this tumor as a differential diagnosis in lesions of this region. Prognosis depends on early diagnostic accuracy.

Aneurismas del ápex carotideo en la edad pediátrica: Reporte de un caso / Carotid apex aneurysms in children: Case report

Introducción. Las malformaciones aneurismáticas del polígono de Willis comprenden un raro grupo de anomalías congénitas que se presentan en edades pediátricas. Son causa importante de morbimortalidad en este grupo de edad; en consecuencia, el manejo de estas lesiones es complejo y potencialmente letal, y puede plantear problemas para el paciente y su tratamiento. Caso clínico. Presentamos el caso de una niña de 10 años que inició de manera súbita con síncope neurogénico y crisis convulsivas tónico-clónicas generalizadas. Al llegar a urgencias, se le realizó una tomografía donde se identificó hemorragia subaracnoidea Fisher II. En la angiografía cerebral de 4 vasos se observó una lesión sacular del ápex carotideo, de aproximadamente 5.1 x 7.2 mm, con cuello visible y domo dirigido hacia la parte cefálica y hacia la parte frontal. Se le realizó craneotomía fronto-pterional derecha. Durante el transquirúrgico, se llevaron a cabo un ultrasonido pre-clipaje, que identificó la lesión aneurismática, y uno post-clipaje, para verificar la permeabilidad de las ramas distales al clipaje. Conclusiones. Las malformaciones aneurismáticas en la edad pediátrica son extremadamente raras. Por ello, métodos como la endoscopia y la sonografía transoperatoria y postoperatoria son de gran utilidad, ya que evidencian detalles que en ocasiones los estudios de imagen convencionales no revelan. Todo esto condiciona un procedimiento con menor riesgo de morbimortalidad para este tipo de pacientes.

Background. Aneurysm malformations of the circle of Willis comprise a rare group of congenital anomalies that occur during the pediatric age and are a major cause of morbidity and mortality in this age group. Consequently, management of these lesions is complex and potentially lethal and may pose problems for the patient and affect their treatment. Case report. We report the case of a 10-year-old girl who had a sudden onset of neurogenic syncope and tonic-clonic seizures. After arriving at the emergency room, she underwent a CT scan, identifying a Fisher II subarachnoid hemorrhage. Four-vessel cerebral angiography identified apex saccular carotid injury ~5.1 x 7.2 mm with visible neck and dome partially cephalic and partially frontal. Right frontopterional craniotomy was performed and preclipping during trans-surgical ultrasound was performed, identifying the aneurysmal lesion and post-clipping to verify patency of the distal branches to clipping. Conclusions. Aneurysmal malformations in children are extremely rare. For this reason, methods such as endoscopy, trans- and postoperative ultrasound are very useful. These procedures sometimes reveal details that conventional imaging studies do not, determining use of a procedure with less risk of morbidity and mortality for these patients.

An expandable prosthesis with dual cage-and-plate function in a single device for vertebral body replacement: clinical experience on 14 cases with vertebral tumors.

An expandable vertebral body prosthesis with dual cage-and-plate function in a single device (JR prosthesis) was designed to test the hypothesis that this modular system can provide the biomechanical requirements for immediate and durable spine stabilization after corpectomy. Cadaver assays were performed with a stainless steal device to test fixation and adequacy to the human spine anatomy. Then, 14 patients with vertebral tumors (eight metastatic) underwent corpectomy and vertebral body replacement with a titanium-made JR prosthesis. All patients had neurological deficit, severe pain and spine instability prior to surgery. Mean pain score before surgery on a visual analog scale decreased from 7.6-3.0 points after operation (p = 0.002). All patients achieved at least one grade of improvement in the Frankel score (p = 0.003), excepting the three patients with Frankel grade A before surgery. Two patients with renal cell carcinoma died during the following 4 days after surgery. The remaining patients attained a painless and stable spine immediately, which was maintained for long periods (mean follow-up: 25.4 months). No significant infections or implant failures were registered. A nonfatal case of inferior vena cava surgical injury was observed (repaired during surgery without further complications). In conclusion, the JR prosthesis stabilizes the spine immediately after surgery and for the rest of the patients' life. To our knowledge, this is the first report on the clinical experience of any expandable vertebral body prosthesis with dual cage-and-plate function in a single device.