Hematoma intraparenquimatoso asociado a vasculitis ANCA positiva / Intraparenchymal haematoma associated with an ANCA-positive vasculitis

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Supratentorial neuroenteric cyst in children: a case report and brief literature review.

Neurenteric cysts are rare developmental lesions typically found outside the central nervous system but when they do, we most likely find them in a spinal (cervical or dorsal) intradural extramedular location, often associated with dysraphism. The more unusual intracranial cases have been published because of its rarity, occurring mostly as a posterior fossa extra-axial cyst, in adults. Supratentorial cases are distinctly infrequent, especially in children, resulting in few case reports and even fewer case reviews. We describe a case of a child with a supratentorial neurenteric cyst and present a brief review of the literature about these cysts in children, a noticeable gap in the literature.

Seno dérmico lumbar: consecuencias de un retraso diagnóstico / Lumbar dermal sinus: Consequences of a delayed diagnosis

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Vanishing diffuse leptomeningeal contrast enhancement in an infant with choroid plexus papilloma.

Choroid plexus tumors (CPT) can present in the baseline magnetic resonance imaging (MRI) with lesions compatible with leptomeningeal dissemination. Therapeutic strategy in this condition is controversial. We present a case of an infant with CPP and significant diffuse leptomeningeal contrast enhancement at diagnosis, which spontaneously resolved after removal of the primary tumor. In these challenging cases, several aspects, such as histopathological/molecular diagnosis and close radiological follow-up, should be taken into account to avoid unnecessary treatments.

Hidrocefalia por hiperplasia de plexos coroideos en un paciente con mosaicismo de trisomía 9. Un verdadero reto diagnóstico y terapéutico / Hydrocephalus due to hyperplasia of the choroid plexuses in a patient with trisomy 9 mosaicism. A real diagnostic and therapeutic challenge

Introducción. La trisomía 9 es una cromosomopatía inusual en pacientes nacidos vivos, que frecuentemente se acompaña de anomalías funcionales y estructurales del sistema nervioso central. Entre otras muchas alteraciones, varios trabajos en la bibliografía anglosajona demuestran una asociación entre cromosomopatía 9 y patología de los plexos coroideos. Caso clínico. Varón de 4 meses de vida con mosaicismo de trisomía 9 asociado a hidrocefalia secundaria a hiperplasia de los plexos coroideos, que fue remitido por clínica de hipertensión intracraneal. El procedimiento derivativo de líquido cefalorraquídeo por el que optamos inicialmente provocó una ascitis masiva debida a la producción desmesurada de líquido cefalorraquídeo, y desembocó en una cascada de múltiples intervenciones quirúrgicas, entre las que se incluyeron procedimientos endoscópicos y derivativos. Conclusiones. Se trata de un ejemplo más de asociación entre patología de los plexos coroideos y cromosomopatía 9. Debido a su escasa incidencia, es difícil establecer el diagnóstico de hidrocefalia secundaria a hiperplasia de los plexos y, por tanto, el tratamiento más adecuado. En este tipo de hidrocefalia existe un doble mecanismo fisiopatológico, que implica un aumento de producción de líquido cefalorraquídeo y una disminución de su reabsorción. A pesar de tener en cuenta dicha consideración, el tratamiento de la hidrocefalia secundaria a hiperplasia de los plexos supone un verdadero reto que habitualmente pasa por múltiples procedimientos quirúrgicos, desde la plexectomía o coagulación de los plexos coroideos hasta la implantación de dispositivos de derivación de líquido cefalorraquídeo (AU)

Introduction. Trisomy 9 is an unusual chromosome abnormality in live-born patients, which is frequently accompanied by functional and structural anomalies of the central nervous system. Among many other alterations, several studies have been published in the English-speaking literature that show an association between chromosome 9 abnormality and pathologies affecting the choroid plexuses. Case report. We report the case of a 4-month-old male with trisomy 9 mosaicism associated to hydrocephalus secondary to choroid plexus hyperplasia, who was referred due to a clinical picture of intracranial hypertension. The cerebrospinal fluid (CSF) drainage procedure that was initially chosen caused massive ascites due to an excessive production of CSF, and led to a cascade of multiple surgical interventions, which included endoscopic and drainage procedures. Conclusions. This is another example of an association between choroid plexus pathologies and chromosome 9 abnormality. Due to its scarce incidence, diagnosis of hydrocephalus secondary to plexus hyperplasia is difficult, as is selecting its most suitable treatment. In this type of hydrocephalus there is a double pathophysiological mechanism, which involves an increase in CSF production and a decrease in its reabsorption. Despite taking these considerations into account, the treatment of hydrocephalus secondary to plexus hyperplasia is a real challenge that usually leads to multiple surgical interventions ranging from plexectomy or coagulation of the choroid plexuses to the implantation of CSF drainage devices (AU)

Second re-irradiation for DIPG progression, re-considering "old strategies" with new approaches.

Diffuse intrinsic pontine glioma (DIPG) is an aggressive infiltrative glioma for which no curative therapy is available. Radiation therapy (RT) is the only potentially effective intervention in delaying tumor progression, but only transiently. At progression, re-irradiation is gaining popularity as an effective palliative therapy. However, at second progression, exclusive symptomatic treatment is usually offered. Here we report two patients with DIPG at second progression who were treated with a second re-irradiation course with good response. Importantly, treatment was well tolerated with no irradiation associated acute toxicity identified.

Plexopapiloma metastásico en la edad pediátrica: caso clínico y revisión de la bibliografía / Metastatic plexus papilloma in the paediatric age: a case report and review of the literature

Introducción. Los tumores del plexo coroideo son tumores poco frecuentes, con un pico de incidencia en los primeros años de vida. Clínicamente suelen producir hipertensión intracraneal. Histológicamente se dividen en papilomas y carcinomas. Es rara la existencia de metástasis en los papilomas. Caso clínico. Niña de 11 años con lesión intracraneal en el IV ventrículo y lesión intradural sacra. Se practica craneotomía suboccipital con resección total de la lesión ventricular, y en un segundo tiempo exéresis parcial de la lesión espinal. El estudio histológico determina el diagnóstico de papiloma típico del plexo coroideo en la lesión intracraneal y de metástasisde papiloma del plexo coroideo en la lesión espinal. Al revisar la bibliografía, se comprueba que en la edad pediátrica es muy raro encontrar papilomas del plexo coroideo con lesiones metastásicas. No existe consenso en el tratamiento de las metástasis de plexopapilomas en la edad pediátrica, adoptándose una conducta expectante en dos casos y tratamiento quirúrgico mediante laminectomía en otro. Conclusiones. Los papilomas del plexo coroideo son lesiones raras, benignas y con buen pronóstico. Es poco frecuente la existencia de metástasis. El tratamiento de elección es la resección total de la lesión. Para el tratamiento de las metástasis no hay evidencias de cuál es la mejor actitud, y se han propuesto distintas alternativas (AU)

Introduction. Tumours of the choroid plexus are uncommon, with a peak incidence in the early years of life. Clinically they usually produce intracranial hypertension. Histologically, they can be divided into papillomas and carcinomas. Metastasis rarely occurs in the case of papillomas. Case report. An 11-year-old female with an intracranial lesion in the 4th ventricle and a sacral intradural lesion. A suboccipital craniotomy with total resection of the ventricular lesion was performed and this was followed later by partial exeresis of the spinal lesion. The histological study provides the diagnosis of typical choroid plexus papilloma in the intracranial lesion and choroid plexus papilloma metastasis in the spinal lesion. A review of the literature showed that choroid plexus papillomas with metastatic lesions are very rarely found at the paediatric age. There is no general agreement on the treatment of plexus papilloma metastasis at the paediatric age, expectant management being adopted in two cases and surgical treatment involving a laminectomy in the other. Conclusions. Choroid plexus papillomas are rare, benign lesions with a good prognosis. Metastasis seldom exists. Preferred treatment is total resection of the lesion. For the treatment of metastases, there is no evidence as to which is the best approach and different alternatives have been suggested (AU)

[Metastatic plexus papilloma in the paediatric age: a case report and review of the literature]. / Plexopapiloma metástasico en la edad pediátrica: caso clínico y revisión de la bibliografía.

INTRODUCTION: Tumours of the choroid plexus are uncommon, with a peak incidence in the early years of life. Clinically they usually produce intracranial hypertension. Histologically, they can be divided into papillomas and carcinomas. Metastasis rarely occurs in the case of papillomas. CASE REPORT: An 11-year-old female with an intracranial lesion in the 4th ventricle and a sacral intradural lesion. A sub-occipital craniotomy with total resection of the ventricular lesion was performed and this was followed later by partial exeresis of the spinal lesion. The histological study provides the diagnosis of typical choroid plexus papilloma in the intracranial lesion and choroid plexus papilloma metastasis in the spinal lesion. A review of the literature showed that choroid plexus papillomas with metastatic lesions are very rarely found at the paediatric age. There is no general agreement on the treatment of plexus papilloma metastasis at the paediatric age, expectant management being adopted in two cases and surgical treatment involving a laminectomy in the other. CONCLUSIONS: Choroid plexus papillomas are rare, benign lesions with a good prognosis. Metastasis seldom exists. Preferred treatment is total resection of the lesion. For the treatment of metastases, there is no evidence as to which is the best approach and different alternatives have been suggested.