RESUMEN
OBJECT Malignant tumors of the brainstem, excluding classic diffuse intrinsic pontine gliomas (DIPGs), are a very rare, heterogeneous group of neoplasms that have been infrequently described in the literature. In this paper, the authors present their experiences with treating these unique cancers. METHODS A retrospective chart review was conducted to identify eligible cases over a 15-year period. All tumors involving the pons were, by consensus, felt not to be DIPGs based on their neuroimaging features. Demographic information, pathological specimens, neuroimaging characteristics, surgical and nonsurgical management plans, and survival data were gathered for analysis. RESULTS Between January 2000 and December 2014, 29 patients were identified. The mean age at diagnosis was 8.4 years (range 2 months to 25 years), and 17 (59%) patients were male. The most common presenting signs and symptoms were cranial neuropathies (n = 24; 83%), hemiparesis (n = 12; 41%), and ataxia or gait disturbance (n = 10; 34%). There were 18 glial and 11 embryonal tumors. Of the glial tumors, 5 were radiation-induced and 1 was a malignant transformation of a previously known low-grade tumor. Surgical intervention consisted of biopsy alone in 12 patients and some degree of resection in another 15 patients. Two tumors were diagnosed postmortem. The median overall survival for all patients was 196 days (range 15 to 3999 days). There are currently 5 (17%) patients who are still alive: 1 with an anaplastic astrocytoma and the remaining with embryonal tumors. CONCLUSIONS In general, malignant non-DIPG tumors of the brainstem carry a poor prognosis. However, maximal cytoreductive surgery may be an option for select patients with focal tumors. Long-term survival is possible in patients with nonmetastatic embryonal tumors after multimodal treatment, most importantly maximal resection.
Asunto(s)
Neoplasias del Tronco Encefálico , Glioma , Neoplasias de Células Germinales y Embrionarias , Adolescente , Adulto , Astrocitoma/mortalidad , Astrocitoma/patología , Astrocitoma/terapia , Neoplasias del Tronco Encefálico/mortalidad , Neoplasias del Tronco Encefálico/patología , Neoplasias del Tronco Encefálico/terapia , Niño , Preescolar , Femenino , Estudios de Seguimiento , Glioma/mortalidad , Glioma/patología , Glioma/terapia , Humanos , Lactante , Masculino , Neoplasias de Células Germinales y Embrionarias/mortalidad , Neoplasias de Células Germinales y Embrionarias/patología , Neoplasias de Células Germinales y Embrionarias/terapia , Estudios Retrospectivos , Adulto JovenRESUMEN
OBJECT: Pediatric brain tumors may be associated with medically intractable epilepsy for which surgery is indicated. The authors sought to evaluate the efficacy of epilepsy surgery for seizure control in pediatric patients with brain tumors. METHODS: The authors performed a retrospective review of consecutive patients undergoing resective epilepsy surgery related to pediatric brain tumors at Miami Children's Hospital between June 1986 and June 2014. Time-to-event analysis for seizure recurrence was performed; an "event" was defined as any seizures that occurred following resective epilepsy surgery, not including seizures and auras in the 1st postoperative week. The authors analyzed several preoperative variables to determine their suitability to predict seizure recurrence following surgery. RESULTS: Eighty-four patients (47 males) with a mean age (± standard deviation) of 8.7 ± 5.5 years (range 0.5-21.6 years) were included. The study included 39 (46%) patients with gliomas, 20 (24%) with dysembryoplastic neuroepithelial tumors (DNETs), 14 (17%) with gangliogliomas, and 11 (13%) with other etiologies. Among the patients with gliomas, 18 were classified with low-grade glioma, 5 had oligodendroglioma, 6 had uncategorized astrocytoma, 3 had pilocytic astrocytoma, 3 had pleomorphic xanthoastrocytoma, 3 had glioblastoma, and 1 had gliomatosis cerebri. Seventy-nine (94.0%) resections were guided by intraoperative electrocorticography (ECoG). The mean time (± standard deviation) to seizure recurrence was 81.8 ± 6.3 months. Engel Class I outcome was achieved in 66 (78%) and 63 (75%) patients at 1 and 2 years' follow-up, respectively. Patients with ganglioglioma demonstrated the highest probability of long-term seizure freedom, followed by patients with DNETs and gliomas. In univariate analyses, temporal location (HR 1.75, 95% CI 0.26-1.27, p = 0.171) and completeness of resection (HR 1.69, 95% CI 0.77-3.74, p = 0.191) demonstrated a trend toward a longer duration of seizure freedom. CONCLUSIONS: ECoG-guided epilepsy surgery for pediatric patients with brain tumors is highly effective. Tumors located in the temporal lobe and those in which a complete ECoG-guided resection is performed may result in a greater likelihood of long-term seizure freedom.
Asunto(s)
Neoplasias Encefálicas/cirugía , Epilepsia/cirugía , Glioma/cirugía , Procedimientos Neuroquirúrgicos/métodos , Lóbulo Temporal/patología , Adolescente , Astrocitoma/cirugía , Neoplasias Encefálicas/complicaciones , Neoplasias Encefálicas/patología , Niño , Preescolar , Electroencefalografía , Epilepsia/etiología , Femenino , Florida , Estudios de Seguimiento , Ganglioglioma/cirugía , Glioblastoma/cirugía , Glioma/complicaciones , Humanos , Lactante , Estimación de Kaplan-Meier , Masculino , Neoplasias Neuroepiteliales/cirugía , Valor Predictivo de las Pruebas , Recurrencia , Estudios Retrospectivos , Factores de Riesgo , Índice de Severidad de la Enfermedad , Lóbulo Temporal/fisiopatología , Lóbulo Temporal/cirugía , Factores de Tiempo , Resultado del Tratamiento , Adulto JovenRESUMEN
Embryonal tumor with multilayered rosettes (ETMR) is a recently described pathological entity. These primitive central nervous system tumors harbor amplification of the 19q13.42 locus and resultant overexpression of the LIN28A protein. Although the WHO currently recognizes 3 distinct histopathological entities-embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma, and medulloepithelioma-recent studies indicate that these tumors have a common molecular profile and clinical course and that they are now classified as a single entity. Here the authors present a case of ETMR located in the fourth ventricle in a 12-month-old boy. The histopathology featured areas of neuropil-like stroma and highly cellular foci with characteristic multilayered rosettes. The authors discuss the clinical, radiological, and histopathological findings in this case and compare them with data in previously published cases in the literature. A review of studies assessing the molecular mechanisms underlying these tumors is also presented.
RESUMEN
Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is rarely seen in the brainstem, and there are few case reports of brainstem ETANTR in the literature. Accordingly, the characteristics and the role of surgical treatment of this rare entity remain unclear. The authors present a case of brainstem ETANTR involving a 33-month-old boy along with a review of the literature and discuss the role of surgical removal in the treatment of this entity. In the authors' case, the tumor was surgically treated with subtotal resection, which resulted in improvement of the patient's preoperative symptoms. Chemotherapy was initiated but did not appear to be effective, radiotherapy was declined, and the boy died 6 months after the operation. Based on their analysis of 10 previously reported cases and their own case, the authors conclude that, with respect to survival, surgery may be beneficial even in cases of ETANTR in the brainstem. They note, however, that further studies with a large number of cases are needed to validate the role of surgical treatment in brainstem ETANTR.
Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias del Tronco Encefálico/patología , Neoplasias del Tronco Encefálico/terapia , Neoplasias de Células Germinales y Embrionarias/patología , Neoplasias de Células Germinales y Embrionarias/terapia , Neurópilo/patología , Procedimientos Neuroquirúrgicos , Ataxia/etiología , Neoplasias del Tronco Encefálico/complicaciones , Quimioterapia Adyuvante , Preescolar , Ciclofosfamida/administración & dosificación , Imagen de Difusión por Resonancia Magnética , Doxorrubicina/administración & dosificación , Humanos , Hibridación Fluorescente in Situ , Imagen por Resonancia Magnética , Masculino , Neoplasias de Células Germinales y Embrionarias/complicaciones , Procedimientos Neuroquirúrgicos/métodos , Tomografía de Emisión de Positrones , Vincristina/administración & dosificaciónRESUMEN
OBJECT: Because of their large size and high vascularity, complete removal of brain tumors in infants and young children is often difficult. In most cases the degree of resection is associated with prognosis. Neoadjuvant chemotherapy may facilitate resection by reducing the vascularity of the tumor. The authors evaluated the effectiveness of neoadjuvant chemotherapy in the management of these tumors. METHODS: The authors performed a retrospective review of infants and young children who underwent tumor removal after neoadjuvant chemotherapy. RESULTS: Nine consecutive patients underwent resection after neoadjuvant chemotherapy during the period February 2004 to December 2012. The mean age at diagnosis was 18 months (range 2-50 months). The average largest tumor diameter was 71 mm (range 30-130 mm) at initial surgery. Five patients underwent partial resection, and 4 underwent biopsy as the initial surgery. The histopathological diagnoses were ependymoma in 2 patients, anaplastic ependymoma in 1, primitive neuroectodermal tumor (PNET) in 2, choroid plexus carcinoma in 1, atypical teratoid/rhabdoid tumor (AT/RT) in 1, glioblastoma in 1, and embryonal tumor with abundant neuropil and true rosettes in 1. After 2-4 courses of multiagent chemotherapy (mainly with vincristine, cyclophosphamide, etoposide, and cisplatin), the second-look surgery was performed. In 1 patient with a PNET, intratumoral hemorrhage was observed after 2 courses of chemotherapy. The mean interval between the initial and the second-look surgery was 3 months. The tumor volume was reduced to varying degrees in 5 patients (56%) after chemotherapy. Intraoperatively, the vascularity of the tumor was considerably reduced, and the tumor was more circumscribed in all cases. Gross-total resection was achieved in 8 patients (89%) and neartotal resection in 1 (11%). Histopathological examination demonstrated fibrotic tissue circumscribing the tumor in 6 of 9 cases (67%). The average blood loss was 20% of the estimated blood volume, and 3 patients (33%) required a blood transfusion. There was no surgical mortality. One patient had transient dysphasia postoperatively. The mean follow-up period was 28 months. At the last follow-up, 2 patients (22%) had died (1 died of tumor progression and 1 of sepsis), and 4 patients (44%) had no tumor recurrence. CONCLUSIONS: Neoadjuvant chemotherapy for brain tumors in infants and young children was effective in reduction of tumor vascularity and clarification of the tumor-brain interface, which significantly facilitated maximal tumor resection.