Intracranial Inflammatory Myofibroblastic Tumor: A Review of 49 cases.

Inflammatory Myofibroblastic Tumor (IMT) is a rare pathologic entity that was first described in 1973. This lesion is most commonly found in the lungs, but other organs' involvement has also been reported. Intracranial location of Inflammatory Myofibroblastic Tumor is rare, and the first case was reported in 1980. An intriguing fact about the intracranial IMT is its resemblance with meningioma on clinical presentation and neuroimaging. We came across a case of intracranial Inflammatory Myofibroblastic Tumor (IIMT) in a 27-year-old male who presented with recurrent episodes of seizures and was diagnosed as meningioma on neuroimaging. The lesion did not subside with medical management and kept on progressing in size. The patient had to undergo surgery, and diagnosis of Inflammatory Myofibroblastic Tumor was ascertained on histopathology. This 'surprise' diagnosis prompted us to review the literature on all cases of IIMTs reported to date to better understand the entity and its implications. In this review article, we present our observations regarding various studied parameters, including patient profile, clinical presentation, site of involvement, focality of the lesion, special associations, and lines of management of the 49 published cases of IIMTs.

Intracranial Inflammatory Myofibroblastic Tumor: A Review of 49 cases

Inflammatory Myofibroblastic Tumor (IMT) is a rare pathologic entity that was first described in 1973. This lesion is most commonly found in the lungs, but other organs' involvement has also been reported. Intracranial location of Inflammatory Myofibroblastic Tumor is rare, and the first case was reported in 1980. An intriguing fact about the intracranial IMT is its resemblance with meningioma on clinical presentation and neuroimaging. We came across a case of intracranial Inflammatory Myofibroblastic Tumor (IIMT) in a 27-year-old male who presented with recurrent episodes of seizures and was diagnosed as meningioma on neuroimaging. The lesion did not subside with medical management and kept on progressing in size. The patient had to undergo surgery, and diagnosis of Inflammatory Myofibroblastic Tumor was ascertained on histopathology. This 'surprise' diagnosis prompted us to review the literature on all cases of IIMTs reported to date to better understand the entity and its implications. In this review article, we present our observations regarding various studied parameters, including patient profile, clinical presentation, site of involvement, focality of the lesion, special associations, and lines of management of the 49 published cases of IIMTs.

Laminotomia dorsal do áxis para excisão de meningioma em cão / Axis dorsal laminotomy for excision of a meningioma in a dog

Background: Surgical access to the spinal cord at the level of C2 vertebra presents technical limitations, due to anatomical particularities in this region. The ventral slot technique does not allow space for the removal of dorsolateral masses. The axis hemilaminectomy technique provides limited access to the dorsal aspect of spinal cord and there is a risk of damaging the vertebral arteries. The axis dorsal laminotomy technique allows good access to spinal cord and preserves the atlantoaxial and nuchal ligaments, however few papers describe this technique. The objective of this paper is to report the axis dorsal laminotomy performed in a dog with a meningioma at this level. Case: An 11-year-old male, Maltese dog, was presented with a 15-day history of progressive weakness, intense cervical pain and difficulty in locomotion of the four limbs. He was being treated with painkillers without improvement. On clinical examination, the physiological parameters were normal. Neurological examination showed tetraparesis with the syndrome localized in the cranial cervical region. The main differential diagnoses were intervertebral disc disease, neoplasia, inflammatory or infectious disease. Computed tomography was performed and the evaluation of images allowed the visualization of an intradural-extramedullary neoplastic process, promoting important spinal compression, with approximate dimensions of the lesion 0.70 cm high x 0.67 cm wide x 0.78 cm long, with apparent involvement of the meninges, with a more lateralized location on the left, with meningioma being the main differential diagnosis. Thus, exploratory surgery, through the technique of dorsal laminotomy of the axis was performed. After the removal of the mass, the spinous process was repositioned in its anatomical location and fixed with 0.6 mm steel cerclage wire that was passed through the holes made in the dorsal lamina of the...(AU)

Meningioma primario intraóseo esfenotemporal: presentación de caso / Primary spheno-temporal intra-osseous meningioma: a case report

RESUMEN Fundamento: los meningiomas ectópicos, definidos como aquellos que no tienen ninguna conexión con la duramadre, poco frecuentes. Son una variante rara y representan cerca del uno por ciento de todos los meningiomas intracraneales. Objetivo: presentar una variante poco frecuente de un meningioma ectópico como causa de proptosis y oftalmoparesia encontrado en una paciente joven. Presentación del caso: paciente de 40 años de edad, que hace seis meses comenzó con dolor en el ojo derecho de moderada intensidad, aumento de volumen de la región frontorbitaria, disminución de la agudeza visual y visión doble. Los estudios de tomografía axial computarizada y resonancia magnética de cráneo y órbita mostraron lesión extraaxial a nivel de la pared lateral de la órbita con extensión extra e intraorbitaria con compresión de estructuras adyacentes que provocó desplazamiento anterior del globo ocular. Se realizó tratamiento quirúrgico con excéresis y el estudio histológico concluyó un meningioma meningotelial ectópico del hueso grado I. Conclusiones: los meningiomas ectópicos resultan poco frecuentes, el tratamiento quirúrgico con la resección total de la lesión es la elección para evitar recurrencias y pueden tener indicación de tratamiento oncológico complementario.

ABSTRACT Background: ectopic meningioma, defined as those that have no connection with the dura mater, are rare. They are a rare variant and represent approximately 1 % of all intracranial meningioma. Objective: to present a rare variant of an ectopic meningioma as a cause of proptosis and ophthalmoparesis found in a young patient. Case report: patient of 40 years of age, who 6 months ago began with pain in the right eye of moderate intensity, increased volume of the front-orbital region, decreased visual acuity and double vision. Computed tomography and MRI of the skull and orbit showed extra-axial lesion at the level of the lateral wall of the orbit with extra and intra-orbital extension with compression of adjacent structures that caused anterior displacement of the eyeball. Surgical treatment was performed with resection and the histological study concluded an ectopic meningotial meningioma of bone grade I. Conclusions: ectopic meningioma are infrequent, surgical treatment with total resection of the lesion is the choice to avoid recurrences and may have an indication of complementary oncological treatment.

Laminotomia dorsal do áxis para excisão de meningioma em cão / Axis dorsal laminotomy for excision of a meningioma in a dog

Background: Surgical access to the spinal cord at the level of C2 vertebra presents technical limitations, due to anatomical particularities in this region. The ventral slot technique does not allow space for the removal of dorsolateral masses. The axis hemilaminectomy technique provides limited access to the dorsal aspect of spinal cord and there is a risk of damaging the vertebral arteries. The axis dorsal laminotomy technique allows good access to spinal cord and preserves the atlantoaxial and nuchal ligaments, however few papers describe this technique. The objective of this paper is to report the axis dorsal laminotomy performed in a dog with a meningioma at this level. Case: An 11-year-old male, Maltese dog, was presented with a 15-day history of progressive weakness, intense cervical pain and difficulty in locomotion of the four limbs. He was being treated with painkillers without improvement. On clinical examination, the physiological parameters were normal. Neurological examination showed tetraparesis with the syndrome localized in the cranial cervical region. The main differential diagnoses were intervertebral disc disease, neoplasia, inflammatory or infectious disease. Computed tomography was performed and the evaluation of images allowed the visualization of an intradural-extramedullary neoplastic process, promoting important spinal compression, with approximate dimensions of the lesion 0.70 cm high x 0.67 cm wide x 0.78 cm long, with apparent involvement of the meninges, with a more lateralized location on the left, with meningioma being the main differential diagnosis. Thus, exploratory surgery, through the technique of dorsal laminotomy of the axis was performed. After the removal of the mass, the spinous process was repositioned in its anatomical location and fixed with 0.6 mm steel cerclage wire that was passed through the holes made in the dorsal lamina of the...

Síndrome de degeneración combinada de la médula espinal y radiculopatía en dos pacientes por quimioterapia intratecal con metrotrexato y citarabina: casos clínicos / Spinal subacute combined degeneration after intrathecal chemotherapy: report of two cases

Intrathecal chemotherapy may be complicated with the development of myelopathies or toxic radiculopathies. This myeloradicular involvement, of toxic character, is unpredictable, since these patients have repeatedly received Intrathecal chemotherapy with the same drugs without apparent injury. The toxic effect should be mainly attributed to Cytarabine and not to methotrexate, since the central nervous system lacks Cytidine deaminase, the enzyme that degrades Cytarabine. We report two patients, an 18-year-old woman and a 16 years old male, who received systemic and intrathecal chemotherapy (methotrexate, cytarabine) for the treatment of an acute lymphoblastic leukemia and developed, in relation to this procedure, a spinal subacute combined degeneration. They had a proprioceptive and motor alteration of the lower extremities and neuroimaging showed selective rear and side spinal cord hyper intensity produced by central axonopathy. Two weeks later the woman developed a quadriplegia and the young man a flaccid paraplegia due to added root involvement.

Radiocirurgia e radioterapia estereotática no tratamento de meningeomas sintomáticos do seio cavernoso / Radiosurgery and stereotactic radiotherapy in the treatment of symptomatic cavernous sinus meningiomas

Introdução: Radiocirurgia estereotática (RCE) e radioterapia estereotática fracionada (RCEF) são inovações modernas de procedimentos radioterápicos, de alta precisão que modelam o feixe de radiação para coincidir com o contorno da lesão, por meio de um sistema de imobilização exata do paciente ao aparelho, com definição do alvo através da fusão de imagens de RM, TC, Angiografia e PET/CT; em que pelas coordenadas de referência estereotática, determina-se que a dose de radiação de alta energia prescrita pelo médico seja depositada somente no volume-alvo, com preservação dos tecidos sadios, órgãos ou estruturas localizadas em suas adjacências. Meningeomas do seio cavernoso (MSCs) representam um problema especial porque podem evoluir comprimindo ou infiltrando estruturas neurovasculares presentes no seio cavernoso. Há evidências de que a RCE e a RCEF proporcionam controle satisfatório do crescimento dos meningeomas do seio cavernoso (MSCs) com efeitos adversos reduzidos. Objetivo: Avaliar resultados da avaliação clínica e da neuroimagem de doentes sintomáticos com MSCs tratados com RCEF ou RCE exclusivamente ou de modo adjuvante à neurocirurgia. Casuística e métodos: Estudo tipo coorte e retrospectivo sobre a avaliação de 89 doentes com MSC sintomático tratados com RCE (36%) ou RCEF (64%) entre janeiro de 1994 e março de 2009 e acompanhados até o final de 2012. Haviam sido submetidos à ressecação neurocirúrgica parcial (Simpson IV) ou à biopsia (Simpson V) previamente à radioterapia 29,2% dos doentes. A dose média de RCE foi de 14Gy, e a dose total de RCEF variou entre 50,4 e 54Gy, sendo fracionada em 1,8-2Gy/dose/dia. Resultados: O período de acompanhamento variou entre 36 e 180 meses (mediana de 73 meses). A percentagem de melhora dos sintomas neuroclínicos individuais e de melhora clínica e radiológica (p > 0,05) apresentou valores semelhantes nos doentes tratados com RCE ou RCEF, sendo respectivamente de 41,6% e 48,3%. Em 37% dos doentes, houve...

Introduction: Stereotactic radiosurgery (SRS) and fractionated stereotactic radiotherapy (FSRS) are modern innovations in radiotherapy procedures, precision shaping the radiation beam to match the contour of the lesion, through a system of accurate patient immobilization to the device, defining target through the fusion of MRI, CT, angiography and PET / CT, which is determined by reference to stereotactic coordinates. The radiation dose of high energy prescribed by the doctor to be delivery only in the target interest, with preservation of healthy tissues, organs or structures located in their vicinity. Cavernous sinus meningiomas (CSMs) pose a special problem because they can evolve compressing or infiltrating the neurovascular structures present of the cavernous sinus. There are evidences that SRS and FRS are efficient in the treatment of CSMs. Objectives: The evaluation of the long-term clinical results and neuroimaging findings in patients with symptomatic CSM treated with FSRT or SRS as single therapy or after a previous neurosurgical treatment. Patients and methods: Retrospective cohort study involving 89 patients with symptomatic CSMs treated with SRS (36%) or FSRS (64%) from January 1994 to March 2009, and followed until the end of 2012. Previous neurosurgical partial resection (Simpson IV) or biopsies (Simpson V) had been performed in 29.2% of the patients. The median dose of SRS was 14Gy and the total dose of FSRT ranged from 50.4 to 54Gy, fractionated in 1.8 to 2Gy/dose/day. Results: The follow-up period ranged from 36 to 180 months (median= 73months). There was improvement in the individual symptoms and in the clinical and radiological findings regardless the radiotherapeutic method in 41.6% and 48.3% of the patients treated with SRS or FSRT, respectively (p > 0,05). In 37% of the patients, at least one neurological complaint present before the treatment did not change and in 43.8% patients, the image of the tumor remained stable....

Meningiomas recurrentes: factores asociados / Recurrent meningiomas: associated factors

Objetivo: Identificar los factores relacionados con la recurrencia de los meningiomas intracraneales operados en el INEN. Material y método: Estudio retrospectivo, comparativo, analítico, de caso control. Pacientes con diagnóstico anatomopatológico de meningioma operados con control postoperatorio a cinco años. Resultados: Se realizó la remoción quirúrgica total en 36 pacientes y subtotal en 19 pacientes. Tuvieron recurrencia el 57,8% de los pacientes con resección quirúrgica subtotal y el 33,3% de pacientes con remoción total; esto nos da un OR de 2,7 con una p < 0,05. Se observó mayor recurrencia en pacientes de sexo masculino, con un OR de 1,7. La edad de recurrencia fue de 43,9 +/- 16,3 años versus 40,85 +/- 17,7 del grupo control. El tiempo de recurrencia fue en promedio de 30,7 +/- 16,3 meses. La localización del meningioma que más recurre fue la zona temporal. No existe preponderancia de ningún tipo de meningioma con respecto a la recurrencia. Conclusiones: El factor de la recurrencia fue el grado de resección quirúrgica, encontrándose que un paciente sometido a una remoción quirúrgica subtotal tiene 2,7 veces más posibilidades de presentar recurrencia del meningioma en comparación con la resección quirúrgica total. El sexo masculino presentó mayor recurrencia. La edad promedio fue de 43,9 años. El tiempo de recurrencia en promediofue de 30,7 meses. La localización más frecuente fue la zona temporal. El abordaje que presentó mayor recurrencia fue el frontoparietal. No existió preponderancia de ningún tipo histológico en el meningioma recurrente.

Objective: To identify factors related with recurrence of intracranial meningiomas operated at the Instituto Nacional de Enfermedades Neoplásicas (Peruvian National Cancer Institute). Material and method: Retrospective, comparative, analytical, case control study. Patients operated who had an anatomopathological diagnosis of meningioma and who underwent a post-operative control after five years were included. Results: Total surgical resections were performed in 36 patients, and subtotal resections were performed in 19 patients. Nearly sixty per cent (57.8%) of patients undergoing subtotal resections developed recurrences, as well as 33.3% of those who underwent total resections, giving a 2.7 odds ratio (OR) and p < 0.05. Recurrences were most frequently observed in male patients, with a 1.7 OR. Age of recurrence was 43.9 +/- 16.3 years compared to 40.85 +/- 17.7 in the control group. Time for recurrence was on average 30.7 +/- 16.3 months. The location of most frequently recurring meningiomas was the temporal area. There is no predominance of any type of meningioma with respect to recurrence. Conclusions: The factor for recurrence was surgical resection, and it was found that a patient undergoing a subtotal surgical resection had 2.7 times more likelihood for developing meningioma recurrence compared to total surgical resection. Male patients recurred more frequently. Average age of patients was 43.9 years. Average time for recurrence was 30.7 months.