Metástasis meníngea de leiomiosarcoma uterino. Reporte de caso y revisión de literatura

Brain metastases are the most commonly seen intracranial lesions in adults. What is more, meningiomas are the most common primary intracranial tumours after gliomas and their imaging characteristics are well known in both CT and MRI scans. However, there are lesions that can mimic meningiomas in imaging studies, including metastases of extracranial tumours, confronting us with a diagnostic and therapeutic challenge. We present the case of a patient with meningeal metastasis of a uterine leiomyosarcoma that was not known at the time of the surgical intervention.     

Ganglioglioma anaplásico: diseminación leptomeníngea cervico-dorso-lumbar. A propósito de un caso

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.     

Metástasis medulares de gliosarcoma. Presentación de un caso y revisión de la literatura

IntroductionGliosarcoma is a rare neoplasm of the central nervous system, similar to glioblastoma multiforme. In contrast to glioblastoma, it is characterised by its propensity for extracranial metastasis (11% of the cases) due to its sarcomatous component. Intramedullary metastasis from primary gliosarcoma is extremely rare.Case reportA patient who had surgery for primary cerebral gliosarcoma developed paraparesis during the course of the disease. A magnetic resonance image showed an intramedullary spinal cord metastasis requiring surgical treatment. This article reviews the literature on intramedullary spinal cord metastasis from gliosarcoma, and highlights the characteristics, treatment and overall survival.ConclusionsOnly 4 cases of intramedullary gliosarcoma metastasis are described in the literature. This extremely rare entity should be suspected with the onset of spinal cord symptoms during the course of primary cerebral gliosarcoma.     

Glioblastomas de larga supervivencia: un análisis sistemático de la literatura a propósito de un caso

IntroductionIn spite of the changes for the treatment of glioblastoma since 2005, we haven’t seen differences between long-survival patients of more than 10 years showing a value minor than 1%.Material and methodWe realize a systematic analysis and identify important factors for long survivor patients. We also show an own case with more of 20 years of survival. We make a new pathological study of the old paraffin block of this patient.ResultsThe most important variable associated with long-survival between all multivariant studies is the age. When we try to find genetic and molecular alterations in glioblastoma associated with prolongated survival, the MGMT promoter methylation play the most important role. We find a correct diagnosis in the current analysis of our patient's sample with very long survival.ConclusionsMultiple variables are found that affect long survival of glioblastoma series but analyzed studies are very heterogeneous and it is very difficult comparation between them. Most articles we review are obtained from databases of different countries with hundreds of patients. It would be very interesting to promote the use of a single database in Spain that allows us to study these long-term glioblastoma survivors.     

Meningioma intramedular tipo rabdoide. Reporte de un caso y revisión de la literatura

Meningiomas are the most frequent tumors located at the spinal level together with neurinomas and metastases. These tumors tend to be intradural and extramedullar. There are few cases described in the literature with a purely intramedullary location (less than 10 cases) and they are frequently observed in the union craneal-cervical. In the presence of an intramedullary tumor we perform differential diagnoses with ependymomas, astrocytomas... In this article we present the first case described in the literature of a patient with a rabdoid-type meningioma exclusively intramedullary at the level of the medullary cone.     

Aneurisma de la arteria cerebral anterior que se presenta como masa del tercer ventrículo e hidrocefalia. A propósito de un caso

Aneurysms which appear as third ventricular masses are uncommon; most are giant aneurysms arising from the basilar apex.We present the case of a 67-year-old male who was admitted to hospital with a 4-week history of gait instability, urinary incontinence and progressive visual loss.A cranial computed tomography scan revealed a hyperdense mass in the third ventricle with triventricular dilatation. Cerebral magnetic resonance imaging, magnetic resonance-angiography and conventional angiography identified this lesion as a partially thrombosed aneurysm of the anterior cerebral artery.To our knowledge, this is the first report of an anterior cerebral artery aneurysm with these clinical and radiological features.     

Ciática extrarraquídea: caso clínico y revisión de la literatura

The persistent sciatic artery is an uncommon disease, considered an axial congenital vascular malformation due to the lack of involution of the sciatic artery during embryonic development. It may be associated with abnormalities in the development of the iliac, common femoral and superficial femoral arteries. Patients may be asymptomatic, or they could present chronic pain, such as sciatic neuralgia, caused by nerve damage, since it is close to the abnormal persistent vessel, or due to ischemic pain, as a result of a thrombosis or embolism of an aneurysm, which could compromise the viability of the limb.     

Displasia fibrosa craneofacial y quiste óseo aneurismático en una paciente con síndrome de McCune-Albright. Presentación de un caso y revisión de la literatura

McCune-Albright syndrome (MAS) is a rare heterogeneous genetic disorder that is characterized by a triad of polyostotic fibrous dysplasia (FD), café au lait spots (CAL), and multiple hyperfunctional endocrinopathies. In general, it is diagnosed clinically. From the triads, 2 of the findings are enough to make the diagnosis.Craniofacial fibrous dysplasia is a term that is used to describe the fibrous dysplasia, which was localized at the craniofacial skeleton and is common in MAS patients.Aneurysmal bone cyst (ABC) is a rare non-neoplastic bone lesion that involves mostly the long bones and vertebrae and may occur very rarely in the craniofacial bones. ABCs may occur as secondary bony pathologies in association with various benign and malignant bone tumors and with fibrous dysplasia. Secondary ABC occurring in craniofacial FD is extremely rare. We present the case of a 21-year-old patient treated at our center for a right orbital aneurysmal bone cyst associated with MAS and provide a review of the relevant 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.     

Ganglioneuroma lipomatoso paravertebral con extensión intraespinal. Descripción de un caso y revisión de la literatura

Lipomatous ganglioneuromas are a rare variant of ganglioneuromas characterized by a mature adipocytic component admixed with a conventional ganglioneuroma component. We present the case of a 34 year old patient with a paravertebral right lesion L1-L4 with intraspinal extension and secondary neurological deficit, who underwent surgery in our hospital. The pathological anatomy showed a yellowish encapsulated neoplasm, which under microscopic evaluation showed areas of ganglioneuroma admixed with areas of mature fat.In the follow up, the patient was asymptomatic, had recovered paresis in the right lower extremity and in the last image control did not present data of lesion recurrence.Fewer than 10 cases of lipomatous ganglioneuromas have been reported in the literature, being this the first paravertebral case wih intraspinal extension and with neurological deficit, hence the interest of this work.