Caracterización de los pacientes con ependimoma intracraneal en el hospital pediátrico Juan Manuel Márquez. 2012-2017 / Characterization of patients with intracranial ependymoma in the pediatric hospital Juan Manuel Márquez. 2012-2017

RESUMEN Introducción: los ependimomas constituyen aproximadamente del 3-5 % de los tumores intracraneales y del 5-10 % de los tumores cerebrales, en la edad pediátrica. Objetivo: caracterizar los pacientes con ependimomas intracraneales intervenidos quirúrgicamente, en el Hospital Pediátrico ¨Juan Manuel Márquez. ¨ Materiales y método: estudio descriptivo, retrospectivo, a pacientes en edad pediátrica con diagnóstico histológico de ependimoma de localización intracraneal. En el período de enero 2012 a diciembre 2017. El universo quedó conformado por todos los pacientes en edad pediátrica operados con diagnóstico histológico de ependimoma intracraneal en el lugar y período antes mencionado (N=22). Resultados: la edad media fue 2,75 años con límites entre 1 y 17 y una desviación estándar de 3,65. Los pacientes del sexo masculino representaron el 63,64 %, la relación con el sexo femenino en los primeros 4 años fue de 1:1. En cuanto al cuadro clínico, se observó predominio de la hidrocefalia en el 72,73 % de los pacientes. Los ependimomas intracraneales de localización infratentorial, (63,64 %) predominaron. El 45,45 % de las lesiones estudiadas se correspondían con el subtipo histológico de ependimoma anaplásico. Conclusiones: la combinación de cirugía, radioterapia y quimioterapia se empleó en la mayoría de los casos. Predominó el abordaje directo de la lesión a través de craneotomía y exéresis adecuada a la localización del ependimoma, sin embargo, en la mayoría solo se logró resección entre el 50 y 90 %. En la mayoría de los pacientes la evolución luego del diagnóstico, evidenció una tendencia hacia la estabilidad (AU).

ABSTRACT Introduction: ependymoma are almost 3-5 % of the intracranial tumors and 5-10 % of the brain tumors in pediatric age. Objective: to characterize the patients with intracranial ependymoma who underwent surgery in the Pediatric Hospital ¨Juan Manuel Márquez.¨ Materials and method: retrospective, descriptive study of patients in pediatric age with histological diagnosis of ependymoma of intracranial location in the period January 2012-December 2017. The universe was formed by all patients of pediatric age who underwent surgery with histological diagnosis of intracranial ependymoma in the before-mentioned place and period (N=22). Results: the average age was 2.75 years with limits between 1 and 17 years old. Male patients represented 63.64 %; the relation with female sex during the first 4 years was 1:1. Regarding the clinical characteristics, hydrocephaly predominated in 72.73 % of patients. Intracranial ependymoma of infratentorial location (63.64 %) predominated. 45.45 % of the studied lesions corresponded to the histological subtype of anaplastic ependymoma. Conclusions: the combination of surgery, radiotherapy and chemotherapy was used in most of the cases. The direct approach of the lesion through craniotomy and a removal adequate to ependymoma location predominated. However, in most of them just the resection of 50-90 % was achieved. The evolution of most of patients after the diagnosis evidenced a tendency to the stability (AU).

Ependimomas intracraneanos en el Instituto Nacional de Oncología y Radiobiología / Intracranial ependymomas at the National Institute of Oncology and Radiobiology

Introducción: los ependimomas representan del 2 al 3 por ciento de todos los tumores cerebrales primarios. Aproximadamente el 30 por ciento de los ependimomas pediátricos son diagnosticados en niños menores de tres años. La ubicación de los ependimomas difiere con la edad; en los adultos, el 60 por ciento se encuentra en la médula espinal, y en los niños, el 90 por ciento se encuentra en el cerebro, en su mayoría, en la fosa posterior. Objetivo: describir las características clínicas, tratamiento y supervivencia de los pacientes con diagnóstico de ependimomas tratados en el servicio de Oncopediatría en el Instituto Nacional de Oncología y Radiobiología. Métodos: se realizó un estudio descriptivo, longitudinal y retrospectivo desde el 1� de enero de 2006 al 31 de diciembre de 2016, según variables demográficas, clínicas y terapéuticas. Se identificaron los pacientes a partir de las bases de datos del registro hospitalario del Instituto Nacional de Oncología y Radiobiología. Se seleccionaron todos los pacientes con diagnóstico histológico de esta enfermedad. Resultados: se identificaron 20 pacientes, con ligero predominio del sexo masculino (45 por ciento), con una edad media de 5,3 años (rango entre 0 y 9 años). El tratamiento más utilizado fue la combinación de la cirugía más la radioterapia y la quimioterapia (45 por ciento), seguida de la cirugía más la radioterapia (30 por ciento). En estos momentos se cuenta con 47 por ciento de supervivencia. Conclusiones: los ependimomas son tumores infrecuentes. El tratamiento de elección es la resección total, acompañado de radioterapia y quimioterapia, dependiendo del tipo histológico y grado de resección(AU)

Introduction: ependymomas account for 2 to 3 percent of all primary brain tumors. About 30 percent of the pediatric ependymomas are diagnosed in children under three years of age. The location of ependymomas varies with age. In adults, 60 percent occur in the spinal cord, whereas in children they occur in the brain, mainly in the posterior cranial fossa. Objective: describe the clinical characteristics, treatment and survival of patients diagnosed with ependymoma cared for at the Oncopediatrics service of the National Institute of Oncology and Radiobiology. Methods: a retrospective longitudinal descriptive study was conducted from 1 January 2006 to 31 December 2016 based on demographic, clinical and therapeutic variables. The patients were identified using data obtained from databases from hospital records at the National Institute of Oncology and Radiobiology. All the patients with a histological diagnosis of the disease were selected. Results: twenty patients were selected, with a slight predominance of the male sex (45 percent) and a mean age of 5.3 years (range 0-9 years). The most common treatment was the combination of surgery plus radiotherapy and chemotherapy (45 percent), followed by surgery plus radiotherapy (30 percent). Current survival is 47 percent. Conclusions: ependymomas are infrequent tumors. The treatment of choice is total resection, accompanied by radiotherapy and chemotherapy, depending on the histological type and resection grade(AU)

Multi-Disciplinary Treatment of a Rare Pelvic Cavity Ependymoma

Ependymomas usually develop from neuroectodermal organs. Here, we present an ependymoma arising from the pelvic cavity. A 27-year-old Korean female was admitted to the hospital with a sensation of abdominal fullness. Imaging studies revealed a huge heterogeneous nodular mass in the pelvis and lower abdomen. Laparotomy showed that two large masses with multiple nodules were located between the uterus and rectum and uterus and bladder, respectively. Histologically, the tumor was characterized by compact columnar neoplastic cells divided by fibrovascular septae. The neoplastic cells formed true ependymal rosettes and perivascular pseudorosettes. Immunohistochemical staining showed a strong positive reaction for glial fibrillary acidic protein (GFAP) and vimentin and a partial positive reaction for S100 and EMA. The tumor was thus diagnosed as an ependymoma arising from the pelvic cavity. The patient was treated with a debulking operation and chemotherapy based upon the in vitro chemosensitivity test results. The patient was free of cancer for 4 years following surgery. This is a rare case of extraneural ependymoma for which an in vitro chemosensitivity test was critical in determining the multidisciplinary approach for treatment.

Multi-Disciplinary Treatment of a Rare Pelvic Cavity Ependymoma

Ependymomas usually develop from neuroectodermal organs. Here, we present an ependymoma arising from the pelvic cavity. A 27-year-old Korean female was admitted to the hospital with a sensation of abdominal fullness. Imaging studies revealed a huge heterogeneous nodular mass in the pelvis and lower abdomen. Laparotomy showed that two large masses with multiple nodules were located between the uterus and rectum and uterus and bladder, respectively. Histologically, the tumor was characterized by compact columnar neoplastic cells divided by fibrovascular septae. The neoplastic cells formed true ependymal rosettes and perivascular pseudorosettes. Immunohistochemical staining showed a strong positive reaction for glial fibrillary acidic protein (GFAP) and vimentin and a partial positive reaction for S100 and EMA. The tumor was thus diagnosed as an ependymoma arising from the pelvic cavity. The patient was treated with a debulking operation and chemotherapy based upon the in vitro chemosensitivity test results. The patient was free of cancer for 4 years following surgery. This is a rare case of extraneural ependymoma for which an in vitro chemosensitivity test was critical in determining the multidisciplinary approach for treatment.