Giant fronto-spheno-orbitary juvenile psammomatoid ossifying fibroma: Case report and literature review.

Juvenile psammomatoid ossifying fibroma (JPOF) is an osteofibrous neoplasm that originates in the craniofacial skeleton typically during the first three decades of life. JPOFs usually involve the orbit, paranasal sinuses or the jaws. Extensive involvement of the anterior cranial base with compromised visual function is a rare phenomenon. In such clinical context, a definite diagnosis can only be made on the basis of histopathological findings, given the absence of pathognomonic radiological features. Despite being considered a benign entity, JPOFs present a locally aggressive behavior. Therefore, these neoplasms must be included in the differential diagnosis in every patient harboring a skull base osteofibrous lesion, and, once diagnosed, gross total surgical removal should be attempted. In this study, we present our experience in the diagnosis and treatment of a patient diagnosed with a giant JPOF involving the cranial base.

Diagnosis and Therapeutic Management of Ventricular Gangliogliomas: An Illustrated Review.

BACKGROUND: Gangliogliomas (GGs) are extremely rare benign neoplasms frequently located within the temporal lobe that usually present with seizures. GGs growing predominantly within the ventricular system (VGGs) are even more infrequent, so definite conclusions concerning their diagnosis and therapeutic management are lacking. METHODS: A retrospective review of case reports of VGGs was performed from the introduction of modern imaging techniques, including 4 new illustrative cases treated in our department. RESULTS: Thirty-four cases were collected. Ages ranged from 10 to 71 years (mean, 26.62 years), and 55.9% were male. Most patients developed symptoms related to high intracranial pressure. The lateral ventricles were predominantly involved (58.8%). Obstructive hydrocephalus was observed in 54.5% of patients. Cystic degeneration and calcification were frequently observed. Surgical treatment was carried out in all cases. Morbidity and mortality were 17.6% and 2.9%, respectively. Gross total tumor resection was achieved in 64.5% of patients. Four patients experienced tumor dissemination along the neural axis. More than 90% of patients maintained a good functional status at last follow-up. CONCLUSIONS: Despite their low incidence, a diagnosis of VGGs should be considered in young male adults who progressively develop intracranial hypertension, caused by a ventricular mass showing signs of cystic degeneration and calcification. Maximal and safe surgical resection represents the gold standard for the treatment of symptomatic VGGs, although total removal is frequently precluded by difficulties in defining appropriate tumor boundaries. Adjuvant radiotherapy should be considered if an incomplete resection was carried out, especially in World Health Organization grade III neoplasms.

Ipsilateral hemiparesis: the forgotten history of this paradoxical neurological sign.

OBJECTIVE: Establishing the neurological localization doctrine for the contralateral hemispheric control of motor functions in the second half of the 19th century, researchers faced the challenge of recognizing false localizing signs, in particular paradoxical or ipsilateral hemiparesis (IH). Despite tremendous progress in current methods of neuroradiological and electrophysiological exploration, a complete understanding of this phenomenon has yet to be attained. METHODS: The authors researched the well-described cases of hemiparesis/hemiplegia ipsilateral to an intracranial lesion published in the scientific literature in the pre-MRI era (before 1980). A comprehensive review of the physiopathological mechanisms proposed for paradoxical hemiparesis throughout this period, as well as the pathological evidence substantiating them, is provided. RESULTS: A collection of 75 patients with hemiparesis/hemiplegia ipsilateral to the primary intracranial lesion reported between 1858 and 1979 were eligible for analysis. Most cases occurred in adults with supratentorial, slowly developing, extraparenchymatous mass lesions, such as neoplasms (38%) or chronic subdural hematomas (36%). Physiopathological theories proposed by the neurologists who investigated IH can be grouped into 4 major concepts: 1) lack of anatomical decussation of the corticospinal tract; 2) impaired functional activation of the contralateral hemisphere by the lesioned dominant hemisphere through the callosal connections; 3) Kernohan's notch phenomenon, or mechanical injury of the contralateral cerebral peduncle against the free edge of the tentorium; and 4) cerebrovascular dysfunction involving the contralateral hemisphere owing to kinking and mechanical flattening of the carotid artery contralateral to the primary intracranial lesion. CONCLUSIONS: IH represents a still underdiagnosed paradoxical neurological phenomenon. With the aid of modern neuroradiological and neurophysiological methods, Kernohan's peduncle notch mechanism has been confirmed to cause IH in many of the cases reported in recent decades. Nevertheless, alternative functional and/or vascular mechanisms must be investigated further for unexplained IH cases, in particular for transitory IH without evidence of peduncle injury. The historical theories reviewed in this paper represent a conceptual framework that may be helpful for this purpose.

Meningiomas quísticos: correlación radiológica y patológica con implicaciones quirúrgicas / Cystic meningiomas: Radiological and pathological correlation with surgical implications

Introducción: Los meningiomas que asocian cambios quísticos prominentes (MQ) han representado un reto diagnóstico y terapéutico desde los orígenes de la neurocirugía moderna. Presentamos la experiencia en el manejo clínico y quirúrgico de esta entidad en nuestro Servicio. Material y métodos: En este estudio descriptivo se incluyen los MQ intervenidos en nuestro Servicio en los últimos 15 años, evaluando las variables clínicas, diagnósticas y terapéuticas de este subgrupo de meningiomas, con especial énfasis en la correlación de los hallazgos radiológicos y patológicos. Los tumores se clasificaron siguiendo el esquema propuesto por Nauta y sus colaboradores. Resultados: Se identificaron 11 casos, lo que supone un 1,8% de los meningiomas intervenidos en ese periodo. Todos los pacientes eran adultos y 8 de ellos, mujeres. Entre los síntomas, destacó la alta frecuencia de crisis epilépticas, mientras que solo un caso desarrolló hipertensión intracraneal aguda. La morfología predominante fue el tipo 2, seguida de los tipos 1, 3 y 4. En 5 casos pudo establecerse un diagnóstico radiológico inequívoco de meningioma. Todos los pacientes fueron tratados mediante cirugía, sin evidenciarse casos de recurrencia durante un seguimiento medio de 52,8 meses. Aunque la mayoría de las neoplasias correspondieron al grado i de la OMS, incluyendo subtipos variados, 4 de ellas fueron diagnosticadas como meningiomas atípicos. Conclusiones: De forma excepcional, los meningiomas pueden desarrollar cambios quísticos, intra- o extratumorales, en número y tamaño variable. Estos constituyen hallazgos poco típicos que pueden dificultar seriamente su diagnóstico diferencial radiológico. Los meningiomas con quistes periféricos de cápsula fina (tipos 2 y 3) suponen el escenario quirúrgico más complejo, pues el realce capsular en las pruebas de imagen no traduce necesariamente infiltración tumoral, mientras que el quiste puede contener células neoplásicas. Por lo tanto, recomendamos realizar al menos biopsia de la cápsula o seudocápsula, y lavado minucioso de la cavidad quirúrgica especialmente en estos subtipos

Introduction: Meningiomas associating prominent cystic changes (CM) have challenged neurosurgeons since the beginning of this surgical discipline. We present the experience in the diagnostic and therapeutic management of this entity in our institution. Material and methods: A review of our patient database was carried out, searching for those CM that were operated on in the last 15 years. Relevant clinical data were recorded and analyzed for each case, with special emphasis in the correlation of radiological and pathological findings. Cystic changes were classified according to the scheme proposed by Nauta et al. Results: A total of 11 patients were gathered, which represents 1.8% of the meningiomas operated on in our department during the period studied. All were adults, predominantly female patients (8 cases). Among the clinical symptoms a high rate of epileptic seizures was observed while only one patient developed acute intracranial hypertension. Morphologically, most tumors corresponded to type 2 CMs, followed by types 1, 3 and 4. In only five patients an unequivocal radiological diagnosis of meningioma could be made. All neoplasms were surgically removed and there were no records of recurrence (mean follow-up: 52.8 months). Microscopic findings were consistent with the pathological diagnosis of atypical meningioma in 4 cases, while the remaining tumors corresponded to OMS grade I neoplasms with variable microscopic patterns. Conclusions: Meningiomas can exceptionally associate cystic changes, both intra and/or extratumoral, in variable number and size. When cystic changes become too prominent (a large number or big-sized cysts), a serious preoperative diagnostic dilemma may arise. The surgical management of those CMs displaying a peripheral, thin-walled cyst (types 2 and 3) is especially complex, as contrast enhancement of the tumor wall did not correlate strictly with neoplastic invasion; even in the absence of this feature free floating islands of meningothelial cells intermixed with cyst fluid can be found. Consequently both surgical biopsy of every suspicious tissue and copious irrigation of the surgical cavity are strongly recommended for these CM types

Cystic meningiomas: Radiological and pathological correlation with surgical implications. / Meningiomas quísticos: correlación radiológica y patológica con implicaciones quirúrgicas.

INTRODUCTION: Meningiomas associating prominent cystic changes (CM) have challenged neurosurgeons since the beginning of this surgical discipline. We present the experience in the diagnostic and therapeutic management of this entity in our institution. MATERIAL AND METHODS: A review of our patient database was carried out, searching for those CM that were operated on in the last 15 years. Relevant clinical data were recorded and analyzed for each case, with special emphasis in the correlation of radiological and pathological findings. Cystic changes were classified according to the scheme proposed by Nauta et al. RESULTS: A total of 11 patients were gathered, which represents 1.8% of the meningiomas operated on in our department during the period studied. All were adults, predominantly female patients (8 cases). Among the clinical symptoms a high rate of epileptic seizures was observed while only one patient developed acute intracranial hypertension. Morphologically, most tumors corresponded to type 2 CMs, followed by types 1, 3 and 4. In only five patients an unequivocal radiological diagnosis of meningioma could be made. All neoplasms were surgically removed and there were no records of recurrence (mean follow-up: 52.8 months). Microscopic findings were consistent with the pathological diagnosis of atypical meningioma in 4 cases, while the remaining tumors corresponded to OMS grade I neoplasms with variable microscopic patterns. CONCLUSIONS: Meningiomas can exceptionally associate cystic changes, both intra and/or extratumoral, in variable number and size. When cystic changes become too prominent (a large number or big-sized cysts), a serious preoperative diagnostic dilemma may arise. The surgical management of those CMs displaying a peripheral, thin-walled cyst (types 2 and 3) is especially complex, as contrast enhancement of the tumor wall did not correlate strictly with neoplastic invasion; even in the absence of this feature free floating islands of meningothelial cells intermixed with cyst fluid can be found. Consequently both surgical biopsy of every suspicious tissue and copious irrigation of the surgical cavity are strongly recommended for these CM types.

Rare association of secondary superficial siderosis caused by a fourth ventricle hemorrhagic ependymoma mimicking a cavernoma: Case report and literature review.

BACKGROUND: The association of a hemorrhagic tumor with secondary superficial siderosis (SS) is a relatively rare although well described phenomenon. CASE DESCRIPTION: We present the case report of a 35-year-old male with a history of drowsiness, hypoacusia, drop attacks, and multidirectional nystagmus during the last 2 months, who presented with acute obstructive hydrocephalus caused by a fourth ventricle mass displaying radiological signs of repeated intra and extratumoral hemorrhage with SS. He underwent gross surgical removal of the solid component of the tumor. Microscopic examination revealed an ependymoma with atypical features, including prominent angiomatous formations and internal chronic hemorrhages with hemosiderin deposits, resembling a cavernoma. The scarce tumoral component, which extended around these cavernous vessels, lacked the gross typical features of fibrillary stroma or perivascular pseudorosettes. CONCLUSION: To our knowledge, including the present case, there are 45 published reports of tumors associating secondary SS. Besides ependymoma, no other hemorrhagic lesion, tumoral or vascular, has been previously published associating a fourth ventricle location with secondary SS. The present case represents the fifth with this finding, and we strongly suggest ependymoma as a presumptive diagnosis when this rare association is encountered. In addition, this appears to be the first case reported in the scientific literature of a hemorrhagic fourth ventricle ependymoma mimicking both, radiologically and histologically, a cavernous malformation.