Tentorial dural arteriovenous fistula with perimedullary venous drainage-associated cervical myelopathy: illustrative case.

BACKGROUND: Tentorial dural arteriovenous fistulas (DAVFs) with perimedullary venous drainage causing cervical myelopathy are very uncommon conditions with an extremely aggressive behavior. When the characteristic radiological clues are missing, the unspecific clinical picture may cause delay and make the diagnosis challenging. OBSERVATIONS: Here the authors report a case of a 58-year-old man who developed progressive spastic tetraparesis and dyspnea with an extensive mild enhancing cervical cord lesion initially oriented as a neurosyphilis-associated transverse myelitis. Acute worsening after steroid administration redirected the diagnosis, and a tentorial Cognard type V DAVF was elicited. The microsurgical disconnection process is described, and previously documented cases in the literature are reviewed. LESSONS: If a DAVF is highly suspected, it is important to consider the possibility of its intracranial origin, and spinal as well as cerebral arteriography must be performed.

Ganglioglioma anaplásico: diseminación leptomeníngea cervico-dorso-lumbar. A propósito de un caso / Anaplasic ganglioglioma: Cervical-dorso-lumbar leptomeningeal dissemination. About a case

Los ganglioglioma son tumores bien diferenciados, de crecimiento lento, compuestos por una mezcla de células ganglionares maduras y gliales. La mayoría son de grado I de la OMS. Aparecen predominantemente en niños y adultos jóvenes. La mayoría se localizan a nivel del lóbulo temporal, y la sintomatología más frecuente son las crisis epilépticas de difícil control farmacológico. En general tienen buen pronóstico tras la resección quirúrgica. La variante anaplásica, considerada grado III de la OMS, presenta mayor agresividad clínica y radiológica. La diseminación leptomeníngea es excepcional en estos tipos de tumores, pero cuando es diagnosticada presenta un curso rápidamente progresivo y fatal para el paciente (AU)

Gangliogliomas are well-differentiated, slow-growing tumors. The majority are grade I of WHO. It appears predominantly in children and young adults. Most are located at the temporal lobe, and as symptomatology more frequent epileptic seizures of difficult pharmacological control. In general, they have a good prognosis after surgical resection. The anaplasic variant, considered to be grade III of the WHO, presents greater clinical and radiological aggressiveness. Leptomeningeal dissemination is exceptional in these types of tumors, but when diagnosed it presents a rapidly progressive and fatal course for the patient (AU)

Anaplasic ganglioglioma: Cervical-dorso-lumbar leptomeningeal dissemination. About a case. / Ganglioglioma anaplásico: diseminación leptomeníngea cervico-dorso-lumbar. A propósito de un caso.

Gangliogliomas are well-differentiated, slow-growing tumors. The majority are gradeI of WHO. It appears predominantly in children and young adults. Most are located at the temporal lobe, and as symptomatology more frequent epileptic seizures of difficult pharmacological control. In general, they have a good prognosis after surgical resection. The anaplasic variant, considered to be gradeIII of the WHO, presents greater clinical and radiological aggressiveness. Leptomeningeal dissemination is exceptional in these types of tumors, but when diagnosed it presents a rapidly progressive and fatal course for the patient.

Anterior sacral meningocele presenting as intracystic bleeding.

PURPOSE: To report a case of anterior sacral meningocele with intralesional bleeding secondary to sacrococcygeal trauma. Likewise, there is a discussion about the physiopathology and the surgical approach to these types of lesions. METHODS: A 43-year-old man diagnosed with Marfan syndrome suffered sacrococcygeal trauma. He was admitted to the emergency room due to symptoms of headache, nausea, and lower limb subjective weakness. CT and MRI showed a large retroperitoneal mass with hemorrhagic content close to the sacrum. Likewise, the MRI showed an image compatible with subarachnoid hemorrhage in the thoracic spinal area, cerebral convexity, and the basal cisterns. The patient went into surgery for an anterior abdominal approach in the midline to reduce the content of the lesion, and subsequently, in the same act, a posterior approach was done with an S1-S2 laminectomy and obliteration of the pedicle. Postoperative MRI 5 months later showed resolution of the ASM. RESULTS: Anterior sacral meningocele is characterized by herniation of the dura mater and the arachnoid mater outside the spinal canal through a defect of the sacrum. We add the risk of bleeding after trauma-never seen in the literature-as one of the possible inherent complications of this lesion. CONCLUSIONS: This report highlights a complication never seen in the literature of a relatively rare condition. In our case, the combined approach was effective for both clinical control and lesion regression.

Fluorescence guided resection with 5-aminolevulinic acid of a pilomyxoid astrocytoma of the third ventricle / Resección guiada por fluorescencia con ácido 5-aminolevulínico de un astrocitoma pilomixoide del tercer ventrículo

Fluorescence-guided resection with 5-aminolevulinic acid has been shown to be useful in the resection of certain brain tumors other than high grade gliomas, facilitating the intraoperative differentiation of neoplastic tissue. The technique enables the surgeon to ensure that no tumor fragments remain, thereby achieving higher rates of complete resection. Tihan first described pilomyxoid astrocytomas in 1999. They are currently classified as grade II astrocytoma according to the WHO classification system and, because of their tendency to recur and their dissemination through the cerebrospinal fluid pathways, they are considered to be more aggressive than pilocytic astrocytoma. As a result, management of these tumors must be more aggressive, always aiming for complete macroscopic resection whenever possible. In this article, we present a case of pilomyxoid astrocytoma of the third ventricle in which the use of fluorescence-guided resection with 5-ALA facilitated complete resection. Imaging tests performed after five years revealed no signs of recurrence and no adjuvant radiotherapy or chemotherapy was required. This article also comprises a review of the literature concerning the characteristics and management of this tumor, which was recently considered to be a different histopathological entity

La resección guiada por fluorescencia con 5-ALA se ha mostrado útil en tumores diferentes a los gliomas de alto grado, permitiendo la diferenciación intraoperatoria del tejido tumoral. La técnica permite revisar el lecho quirúrgico para comprobar que no quedan fragmentos tumorales, consiguiéndose así mejorar las tasas de resección completa. El astrocitoma pilomixoide, descrito en 1999 por Tihan, se clasifica actualmente como un astrocitoma grado II en la clasificación de la OMS y es considerado como una variante con mayor agresividad que el astrocitoma pilocítico por su tendencia a la recidiva y a la diseminación por el líquido cefalorraquídeo. Por ello el tratamiento debe ser más agresivo, fundamentado en una resección macroscópicamente completa siempre que se pueda. En este artículo presentamos el caso de un astrocitoma pilomixoide del tercer ventrículo en el que la fluorescencia con 5-ALA permitió una resección completa, sin signos de recidiva en pruebas de imagen a los 5 años, sin haber precisado tratamiento complementario con radioterapia ni quimioterapia. Se hace además una revisión de la literatura acerca de las características y el manejo de este tumor recientemente considerado como una entidad histopatológica diferente

Fluorescence guided resection with 5-aminolevulinic acid of a pilomyxoid astrocytoma of the third ventricle.

Fluorescence-guided resection with 5-aminolevulinic acid has been shown to be useful in the resection of certain brain tumors other than high grade gliomas, facilitating the intraoperative differentiation of neoplastic tissue. The technique enables the surgeon to ensure that no tumor fragments remain, thereby achieving higher rates of complete resection. Tihan first described pilomyxoid astrocytomas in 1999. They are currently classified as grade II astrocytoma according to the WHO classification system and, because of their tendency to recur and their dissemination through the cerebrospinal fluid pathways, they are considered to be more aggressive than pilocytic astrocytoma. As a result, management of these tumors must be more aggressive, always aiming for complete macroscopic resection whenever possible. In this article, we present a case of pilomyxoid astrocytoma of the third ventricle in which the use of fluorescence-guided resection with 5-ALA facilitated complete resection. Imaging tests performed after five years revealed no signs of recurrence and no adjuvant radiotherapy or chemotherapy was required. This article also comprises a review of the literature concerning the characteristics and management of this tumor, which was recently considered to be a different histopathological entity.

Meningioma intraóseo primario osteolítico del hueso occipital / Primary osteolytic intraosseous meningioma of the occipital bone

Los meningiomas intraóseos primarios son considerados meningiomas extradurales siempre que no tengan relación con la duramadre. La mayoría crecen de huesos del cráneo, pudiendo ser osteoblásticos u osteolíticos. Estos últimos son los más raros, habiendo muy pocos casos descritos en la literatura. Con mayor frecuencia tienen signos de malignidad, por lo que es importante el diagnóstico histológico precoz de una lesión osteolítica craneal para su correcto tratamiento. El tratamiento debe ser la cirugía, con resección completa siempre que se pueda. Presentamos el caso de un gran meningioma intraóseo primario osteolítico del hueso occipital tratado quirúrgicamente mediante craniectomía y sin signos de recidiva a los 5 años de seguimiento

Primary intraosseous meningiomas are considered extradural meningiomas when no dural attachment is present. Most of them arise from the cranial bones and can present either as an osteoblastic or an osteolytic lesion. Osteolytic intraosseous meningiomas are the rarest and very few cases have been reported. Given that many of these may develop signs of malignancy, early histological confirmation is important in order to ensure appropriate treatment. The recommended therapy is surgery, with complete resection whenever possible. We present the case of a large primary intraosseous osteolytic meningioma within the occipital bone, which was completely excised five years ago, currently presenting no signs of recurrence

Sarcoma de Ewing extraesquelético epidural. Caso clínico y revisión de la literatura / Epidural extraskeletal Ewing sarcoma. Case report and literature review

El sarcoma de Ewing es un tumor óseo maligno que en ocasiones presenta afectación extraesquelética, siendo rara la localización epidural. Presentamos el caso de una mujer de 45 años que presentó parestesias, debilidad progresiva y retención urinaria. La resonancia magnética mostró una masa epidural desde C6 a D3. Se realizó una laminectomía de C7 a D2 y exéresis parcial de la lesión. El estudio anatomopatológico fue compatible con sarcoma de Ewing. La paciente recibió quimioterapia y radioterapia, no existiendo evidencia de enfermedad a los 8 meses de seguimiento. Presentamos una revisión de la literatura de todos los casos publicados de sarcoma de Ewing extraesquelético con afectación epidural

Ewing sarcoma is a malignant tumour of the bone that sometimes presents extraskeletal involvement, with the epidural location being rare. We report the case of a 45-year-old woman with paresthesia, paresis and urinary retention. Magnetic resonance imaging showed an epidural mass from C6 to D3. Laminectomy from C7 to D2 and partial resection of the lesion was performed. Pathological analysis was consistent with Ewing sarcoma. The patient received chemotherapy and radiotherapy, without evidence of disease at 8 months follow-up. A review of the literature on all published cases of extraskeletal Ewing sarcoma with epidural involvement is presented

Fluorescence-guided resection with 5-aminolevulinic Acid of subependymomas of the fourth ventricle: report of 2 cases: technical case report.

BACKGROUND AND IMPORTANCE: The usefulness of 5-aminolevulinic acid (5-ALA) for resection of malignant astrocytomas has been established in recent years. In addition to these tumors, it has been reported that 5-ALA fluorescence could be elicited in other tumors such as intracranial and spinal meningiomas or posterior fossa and spinal cord ependymomas, resulting in improved resections. Here, we present 2 cases of subependymomas of the fourth ventricle that showed intense fluorescence after 5-ALA administration. To the best of our knowledge, these are the first reported cases of subependymomas in this location in which 5-ALA elicited useful fluorescence. CLINICAL PRESENTATION: Case 1 was a 61-year-old woman with a history of headaches accompanied by vomiting in the last month. Magnetic resonance imaging (MRI) revealed a tumor occupying the fourth ventricle with slight irregular enhancement. She was operated on after administration of 5-ALA. The tumor emitted intense red fluorescence when illuminated with blue light. An MRI performed 48 hours after surgery confirmed complete resection of the tumor. The pathological diagnosis was subependymoma. Case 2 was a 35-year-old man with a history of several months of headaches and vomiting. An MRI revealed a tumor occupying the caudal part of the fourth ventricle with moderate and irregular enhancement. He was operated on after administration of 5-ALA. The tumor showed intense fluorescence. An MRI performed 48 hours after surgery confirmed a complete resection of the tumor. The pathological diagnosis was subependymoma. CONCLUSION: Fluorescence-guided resection with 5-ALA may be useful for resection of subependymomas of the fourth ventricle. However, further studies are needed.

Fluorescence-guided resection with 5-aminolevulinic acid of meningeal sarcoma in a child.

BACKGROUND: The usefulness of fluorescence-guided resection with 5-aminolevulinic acid for malignant brain gliomas was demonstrated by Stummer. However, there are several articles in the literature showing the usefulness of the technique for other types of tumors, including benign tumors, such as ependymomas or meningiomas. Meningeal sarcomas are rare, highly aggressive malignant tumors, predominately affecting the pediatric population and have a poor prognosis in spite of treatment. Surgical treatment thereof should consist of the most complete resection possible. METHODS: In this article, we present the case of a seven-year-old boy who received surgical treatment for a left frontal tumor after oral administration of 5-aminolevulinic acid (5-ALA); the definitive histological diagnosis of which was meningeal sarcoma. The technique was useful for achieving a complete resection, as the lesion emitted intense fluorescence, and after resection of the lesion with the usual technique, intraoperative fluorescent spots were observed in the resection bed that were also tumor. Imaging tests performed 5 years after surgery ruled out recurrence of the tumor. CONCLUSION: To our knowledge, this is the first case published in the literature of meningeal sarcoma in a child in which intraoperative fluorescence with 5-ALA was used to achieve a complete resection.

[Primary osteolytic intraosseous meningioma of the occipital bone]. / Meningioma intraóseo primario osteolítico del hueso occipital.

Primary intraosseous meningiomas are considered extradural meningiomas when no dural attachment is present. Most of them arise from the cranial bones and can present either as an osteoblastic or an osteolytic lesion. Osteolytic intraosseous meningiomas are the rarest and very few cases have been reported. Given that many of these may develop signs of malignancy, early histological confirmation is important in order to ensure appropriate treatment. The recommended therapy is surgery, with complete resection whenever possible. We present the case of a large primary intraosseous osteolytic meningioma within the occipital bone, which was completely excised five years ago, currently presenting no signs of recurrence.

[Epidural extraskeletal Ewing sarcoma. Case report and literature review]. / Sarcoma de Ewing extraesquelético epidural. Caso clínico y revisión de la literatura.

Ewing sarcoma is a malignant tumour of the bone that sometimes presents extraskeletal involvement, with the epidural location being rare. We report the case of a 45-year-old woman with paresthesia, paresis and urinary retention. Magnetic resonance imaging showed an epidural mass from C6 to D3. Laminectomy from C7 to D2 and partial resection of the lesion was performed. Pathological analysis was consistent with Ewing sarcoma. The patient received chemotherapy and radiotherapy, without evidence of disease at 8 months follow-up. A review of the literature on all published cases of extraskeletal Ewing sarcoma with epidural involvement is presented.

Multiple filum terminale hemangioblastomas symptomatic during pregnancy. Case report.

Hemangioblastomas are low-grade, highly vascular tumors commonly associated with von Hippel-Lindau (VHL) syndrome and most often appearing in the cerebellum. They very rarely occur in the spinal nerve roots, and an origin in the filum terminale is exceptional with no instances of multiple hemangioblastomas of the filum terminale reported in the literature. Because of their vascular nature, these lesions can enlarge and become symptomatic in the context of the changes that take place during pregnancy, as has been noted with cerebellar hemangioblastomas. In any case, the evolution of spinal hemangioblastomas during pregnancy is not well known given its rarity. The conjunction of both processes--that is, multiple hemangioblastomas arising in the filum terminale and pregnancy--is unique. The authors describe the case of a 41-year-old woman with multiple hemangioblastomas of the filum terminale and no other evidence of VHL syndrome, in whom pregnancy precipitated symptoms. The interruption of gestation led to a remission of the symptoms. The literature concerning filum terminale hemangioblastomas and pregnancy is also reviewed.