Ganglioglioma espinal intramedular: caso clínico y revisión comparativa de la literatura / Intramedullary spinal cord ganglioglioma: Case report and comparative literature review

Introducción Los gangliogliomas espinales intramedulares (GGEI) representan el 35-40% de todos los tumores intramedulares en niños. Estos tumores presentan un algoritmo terapéutico y pronóstico diferente con respecto a otros tumores medulares, tales como los astrocitomas o los ependimomas espinales. El objetivo del estudio es revisar la literatura para esclarecer una vía diagnóstica y terapéutica de dicho tumor en base a un caso clínico de GGEI diagnosticado en nuestro centro. Método Se ha realizado una revisión literaria exhaustiva de los GGEI publicados desde 1911 hasta 2018 a través de la plataforma PubMed-NCBI, adjuntando de cada paciente, las características epidemiológicas, la localización, la clínica, el diagnóstico radiológico y el tratamiento. Resultados Se encontraron un total de 167 casos de GGEI, incluyendo nuestro caso. En nuestra muestra, el 52% de los pacientes pertenece al sexo femenino, siendo la década de edad más afectada la que va de los 0 a los 9 años (35% de los pacientes). El déficit motor se establece como síntoma principal en pacientes adultos en mayor proporción que en pacientes pediátricos. Radiológicamente, este tumor se presenta con mayor hiperintensidad y captación de contraste que los astrocitomas y los ependimomas, así como un mayor porcentaje de quistes intratumorales. La mutación BRAFV600E es menos frecuente en los gangliogliomas espinales que en los supratentoriales. La cirugía con resección total es el tratamiento de elección. Solamente el 19% de los pacientes de la muestra recibieron radioterapia y solo el 9% quimioterapia como única vía de tratamiento. Conclusiones Los GGEI son frecuentes en la población pediátrica y requieren de un alto nivel de sospecha para su correcto diagnóstico y tratamiento, ya que tienen 3 veces más riesgo de recurrencia que sus homólogos supratentoriales (AU)

Introduction Intramedullary spinal cord gangliogliomas (ISCGGs) account for 35-40% of all intramedullary tumors in children. These tumors have a different algorithm for treatment and prognosis than other medullary tumors, such as astrocytomas and spinal ependymomas. The objective of the study was to review the literature and examine an approach to diagnosing and treating this tumor based on a case report of ISCGG diagnosed at our center. Method An exhaustive review of cases of ISCGG published via the PubMed-NCBI platform between 1911 and 2018 was performed, and each patient's epidemiological characteristics, tumor location, symptoms, radiological diagnosis and treatment were appended. Results A total of 167 cases of ISCGG were found, including our own. In our sample, 52% of patients were female and the most commonly affected age group was 0-9 years of age (35% of patients). Motor deficit has been found to be the main symptom in a larger proportion of adults patients versus pediatric patients. On X-ray, this tumor shows greater hyperintensity and contrast uptake than astrocytomas and ependymomas, as well as a higher percentage of intratumoral cysts. The BRAFV600E mutation is less common in spinal as opposed to supratentorial gangliogliomas. Surgery with complete resection is the treatment of choice. Only 19% of the patients in the sample received radiotherapy, and only 9% received chemotherapy as their only line of treatment. Conclusions ISCGGs are common in the pediatric population and require strong suspicion for proper diagnosis and treatment, as the risk of recurrence of ISCGGs is 3 times greater than that of supratentorial gangliogliomas (AU)

Intramedullary spinal cord ganglioglioma: Case report and comparative literature review. / Ganglioglioma espinal intramedular: caso clínico y revisión comparativa de la literatura.

INTRODUCTION: Intramedullary spinal cord gangliogliomas (ISCGGs) account for 35-40% of all intramedullary tumors in children. These tumors have a different algorithm for treatment and prognosis than other medullary tumors, such as astrocytomas and spinal ependymomas. The objective of the study was to review the literature and examine an approach to diagnosing and treating this tumor based on a case report of ISCGG diagnosed at our center. METHOD: An exhaustive review of cases of ISCGG published via the PubMed-NCBI platform between 1911 and 2018 was performed, and each patient's epidemiological characteristics, tumor location, symptoms, radiological diagnosis and treatment were appended. RESULTS: A total of 167 cases of ISCGG were found, including our own. In our sample, 52% of patients were female and the most commonly affected age group was 0-9 years of age (35% of patients). Motor deficit has been found to be the main symptom in a larger proportion of adults patients versus pediatric patients. On X-ray, this tumor shows greater hyperintensity and contrast uptake than astrocytomas and ependymomas, as well as a higher percentage of intratumoral cysts. The BRAFV600E mutation is less common in spinal as opposed to supratentorial gangliogliomas. Surgery with complete resection is the treatment of choice. Only 19% of the patients in the sample received radiotherapy, and only 9% received chemotherapy as their only line of treatment. CONCLUSIONS: ISCGGs are common in the pediatric population and require strong suspicion for proper diagnosis and treatment, as the risk of recurrence of ISCGGs is 3 times greater than that of supratentorial gangliogliomas.

Fascitis craneal: caso clínico y revisión de la literatura / No disponible

La fascitis craneal es una lesión miofibroblástica benigna infrecuente, de rápido crecimiento, localizada en el cráneo y de aparición preferente en niños durante el primer año de vida. Histológicamente es similar a la fascitis nodular o a la fascitis seudosarcomatosa. Puede mimetizar patologías más agresivas, como histiocitosis o sarcomas, por su rápido crecimiento de forma nodular en el tejido subcutáneo. La resección completa se considera curativa y tiene bajo riesgo de metástasis o de malignización. En el presente trabajo se presenta el caso de un varón de 4 años con fascitis en tejido celular subcutáneo craneal con erosión de la tabla externa y reacción perióstica circundante, respetando las meninges. El objetivo de este trabajo es destacar la ausencia de necesidad de tratamiento adyuvante con radioterapia o quimioterapia. Se lleva a cabo, además, una revisión de la literatura (AU)

[Cranial fasciitis: a case report and review of the literature]. / Fascitis craneal: caso clínico y revisión de la literatura.

Cranial fasciitis is an uncommon, rapidly-growing, benign, non-tumoural, myofibroblastic lesion of the skull, found mainly among young children in their first year of life. It is histologically similar to nodular fasciitis and pseudosarcomatous fasciitis. It may mimic more aggressive pathologies, such as sarcomatosis or histiocytosis, due to its rapid, nodular growth in subcutaneous tissue. Complete resection is considered curative and, therefore, entails a low risk of metastases or malignant recurrences. We present the clinical, radiological and pathological findings in a 4-year-old boy with cranial fasciitis in the deep, subcutaneous, soft tissue, with erosion of the outer table of the cranium, which also produced periosteal reaction, while respecting the inner table and meninges. The objective of this article is to highlight the absence of radiotherapeutic or chemotherapeutic adjuvant treatment. In addition, an exhaustive review of the literature is also presented.

Ciatalgia secundaria a endometriosis extrapélvica del músculo piriforme. A propósito de un caso / No disponible

Objetivos Mostrar un caso de compresión sintomática del nervio ciático derecho a nivel de la escotadura ciática secundaria a una endometriosis en el músculo piriforme y realizar una revisión de los casos publicados en la literatura. Material y métodos Se presenta el caso de una paciente de 29 años con un cuadro de ciática derecha crónica de 2 años de evolución: el primer año episódica y coincidente con la menstruación, el segundo año constante y con un trastorno de la marcha por debilidad de la musculatura dependiente del músculo ciático. Como causa de la mononeuropatía (demostrada por estudio neurofisiológico) se objetivó en RM y PET una masa a nivel de la escotadura ciática con un aumento del metabolismo de las estructuras adyacentes. Con la finalidad de liberar el nervio y obtener muestra para diagnóstico histológico se decidió intervenir quirúrgicamente a la paciente. Resultados Se procedió a un abordaje transglúteo, neurólisis externa del nervio ciático y resección de un (..) (AU)

[Sciatica secondary to extrapelvic endometriosis affecting the piriformis muscle. Case report]. / Ciatalgia secundaria a endometriosis extrapélvica del músculo piriforme. A propósito de un caso.

OBJECTIVE: We present a case report of symptomatic compression of the right sciatic nerve notch, secondary to piriformis muscle endometriosis, as well as a literature review. MATERIAL AND METHODS: We report the case of a 29-year-old woman with 2-year evolution of right chronic sciatica. During the first year, symptoms were episodic and associated with menstruation. During the second year, sciatica was constant and associated with gait disorder due to sciatic musculature weakness. Mononeuropathy was proved by a neurophysiological study, with MRI and PET studies revealing a mass in the sciatic notch and regional pathological increase in metabolic activity. Surgical treatment was performed in order to release the nerve and obtain a histological sample. RESULTS: The patient was treated by a transgluteal approach, with external neurolysis of the sciatic nerve and resection of an old-blood cyst at the level of the piriformis muscle. This was subsequently reported as endometriosis by histological examination. The sciatica was resolved after surgery. CONCLUSIONS: Extrapelvic sciatic nerve compression by adjacent endometriosis is very infrequent. Muscle denervation and lack of a histological diagnosis led to surgical exploration of the compression area in order to release the nerve, resect the cause of compression and obtain a definitive diagnosis. The procedure improved all symptoms.