1/23. Heterotopic nasopharyngeal brain tissue associated with cleft palate.OBJECTIVE: The occurrence of extracranial brain tissue is rare. Most of the literature describes cases in which it is located around the nose and throat and has been classified as nasal glioma. Even more unusual is heterotopic brain tissue in the nasopharynx. We were able to find only 17 previously reported cases. Of these 17 cases, 6 had heterotopic brain tissue located in a cleft palate. This report comments on the identification and treatment of heterotopic brain tissue associated with cleft palate without connection to the central nervous system. Our case subject is a 10-month-old girl diagnosed with heterotopic nasophranygeal brain tissue associated with cleft palate. RESULTS: Excision and palatoplasty were performed conjunctively with excellent results. CONCLUSIONS: Simultaneous excision of heterotopic nasopharyngeal brain tissue and palatoplasty of the cleft palate is an excellent option for treatment of these cases.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
2/23. Intracranial extracerebral neuroglial heterotopia: A case report and review of the literature.Heterotopic masses of neuroglial tissue are uncommon and most frequently involve extracranial midline structures. We report an unusual case of an intracranial, extracerebral neuroglial heterotopia involving the middle and anterior cranial fossae of a 5-year-old girl who presented with facial asymmetry. The lesion was composed of mature but disorganized gray and white matter admixed with surrounding soft tissues and exhibited histologic features reminiscent of cortical dysplasia. These rare lesions have been postulated to arise from a protrusion of tissue from the neuraxis through a pial defect, from abnormalities in the migration of embryonic neuroepithelial tissue, or from an accessory evagination of the neural tube inferior to the telencephalic vesicles. Regardless of the underlying pathogenic mechanism, these lesions must be histologically distinguished from both teratomas and primary central nervous system neoplasms.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/23. Glial heterotopia in the subcutaneous tissue overlying T-12.Heterotopic glial nodules occur most commonly in the head and neck area, and are theorized to arise following abnormalities in the development of the facial and skull bone plates. However, in spite of the fact that some of these lesions are associated with communication with the central nervous system (CNS), the lack of a meningeal component, argues against simple herniation and separation of brain tissue through a defect in the skull. We present an infant with a nodule directly over the spine present in the T-12 region with no skin abnormalities. magnetic resonance imaging (MRI) and computerized axial tomography (CT) showed no spinal abnormalities with an overlying fibrotic soft tissue mass. The patient had no other associated clinical findings. Histologic findings showed a cellular component arising within the reticular dermis with a deep circumscribed margin. The nodule contained irregularly shaped cells containing abundant cytoplasm and indistinct cellular margins with bland nuclei. These cells were clustered around and between a fibro-mucinous stroma. Immunohistochemical stains showed positive staining for S-100 protein, vimentin, GFAP, NSE, and CD57, and negative staining for Ki-67, CD34, Neurofilament protein, cytokeratin, and EMA. The spindle cells showed positive staining for CD34 and vimentin. The clinical and histologic features and immunohistochemical profiles are used to separate this lesion from the closely related, ependymal rests, ependymomas, and primary cutaneous chorodomas.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
4/23. Bilateral subpleural ectopic brain tissue in a 23-week-old fetus.Bilateral lesions were seen in the subpleural region in a 23-week-old aborted male fetus. This fetus was not macerated and showed no central nervous system abnormality on physical examination and vertebral magnetic resonance imaging (MRI). Postmortem examination revealed bilateral, paravertebral, subpleural, circumscribed, yellowish-white, fluent lesions 2.5 x 1 x 1 cm in size. These lesions were localized on the upper part of both lungs and there was no other internal malformation. Histological examination of lesions showed adult neurones and well-differentiated neural tissue with white and gray matter, choroid plexus, ependymal structures and, rarely, some peripheral neural cells in addition to immature neuroectodermal cells. These cells were more mature than those in the brain tissue.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/23. Nasal glioma or nasal glial heterotopia? Morphological, immunohistochemical and ultrastructural study of two cases.The term nasal glioma has been used to describe a congenital benign tumor of the nasal region containing neural tissue. The nature of these lesions remains open to controversy, because of the different locations of the heterotopic neural tissue involved, the deficient development of the bony structures and the persistence or not of the structural relations with the central nervous system. More recent terms define these lesions as ectopic nervous tissue. A clinical, morphological, ultrastructural and immunohistochemical study is made of two cases of nasal glioma, one associated with agenesis of the corpus callosum. In this case, the mother had been treated with clomiphene. In such cases, morphological and immunohistochemical findings support that "nasal glioma" remain valid as a descriptive term defining a congenital benign tumor composed of heterotopic neural tissue within the nasal region and covered by skin, that may recur following incomplete surgical resection.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
6/23. Intrapulmonary neuroglial heterotopia.Nonteratomatous intrapulmonary neuroglial heterotopia not associated with birth trauma or frank vascular embolization has been described rarely. This article briefly reviews the literature, and presents two additional cases of intrapulmonary neuroglial heterotopia. We found 14 cases in the literature. Twelve of these cases had central nervous system disruption, where neuroglial elements were in direct contact with amniotic fluid. Several hypotheses have been proposed, including fetal aspiration of detached neural fragments within amniotic fluid, neural crest migration defects, and vascular embolization with implantation. Of our two cases, one represents the first occurrence where central nervous system abnormalities were secondary to mechanical disruption, rather than to a primary neural tube defect. Our second case represents the youngest documented occurrence (17 weeks gestation) of intrapulmonary neuroglial heterotopia. Additionally, immunohistochemical studies were performed on these lesions, the results of which favor their neural origin. We present these findings and suggest they support the aspiration mechanism for neuroglial heterotopia in lung tissue.- - - - - - - - - - ranking = 2keywords = central nervous system, nervous system (Clic here for more details about this article) |
7/23. Gastric heterotopia in the nasopharynx causing airway obstruction in the newborn.We describe a newborn boy with a gastric heterotopia located in the nasopharynx, which caused airway obstruction soon after delivery. Gastric heterotopia is an uncommon lesion generally found throughout the alimentary tract, but nasopharynx is an exceptional location. To our knowledge, this is the second reported case of gastric heterotopia located in the nasopharynx, and the first one presenting in a newborn with symptoms at birth and with an associated central nervous system mass.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/23. Periventricular neuronal heterotopia, oro-facio-digital anomalies, and microphthalmia. A new syndrome?Congenital malformations involving the face, oral cavity, and digits are the main findings in the oro-facio-digital (OFD) syndromes. Various eye anomalies and central nervous system involvement have also been reported in this condition. Here we report on a Brazilian boy with features belonging to the clinical spectrum of the OFD syndromes. He also had additional findings of periventricular nodular heterotopia (PVNH), asymmetric limb involvement, and microphthalmia. This unusual pattern of anomalies has not been reported previously and appears to be unique.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
9/23. Temporal soft tissue glioneuronal heterotopia in a child with a seizure disorder: case report and review of the literature.Tissue and cellular elements generally attributed to the central nervous system (CNS) are infrequently found in areas outside the CNS proper. Most of these lesions contain predominantly glial tissue. In rare instances, heterotopic CNS tissue is found in the scalp, many associated with an intracranial connection and overlying skin and hair anomalies. In follow-up of these patients, development is normal. We present a case of a temporal scalp mass in a 19-month-old girl with a seizure disorder beginning at about age 1 year. At the time of excision, the mass was not associated with intracranial connection or overlying cutaneous abnormalities and demonstrated abundant neuronal and glial elements with features of dysplastic CNS tissue. We speculate that, in certain circumstances, aberrantly localized CNS tissue may be related to abnormal CNS development.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
10/23. Complex choristoma of the gyrus rectus: a distinct clinicopathologic entity?We report 2 cases of an unusual central nervous system lesion involving the gyrus rectus and characterized by well-differentiated epithelial and mesenchymal components. One patient was a 30-year-old woman, and the other was a 42-year-old man. Both presented with seizures. Radiologically, the lesions were solid, with ill-defined margins and increased signal on T2-weighted MRI. Both were contrast enhancing. There was no significant mass effect or edema. Histopathologically, the epithelial component was characterized by a tubule-forming, stratified epithelium. The lumina of the tubules were lined, in some areas, by a distinct layer of mature keratinocytes. Smooth muscle actin-immunoreactive myoepithelial cells were present in the basal layer, indicating glandular differentiation. Some lumina were cystically dilated and contained anucleate squames and/or PAS-positive material. In some areas, the epithelial structures were separated by reactive glial tissue resembling pilocytic astrocytoma, with prominent, piloid processes, Rosenthal fibers, and gemistocytic astrocytes. In other areas, glial nuclei formed small clusters reminiscent of subependymoma, but no glioma was identified in either case. Tissue resembling mature adipose was present focally. No mitotic figures were identified. Follow-up interval of up to 2 years showed no evidence of recurrent tumor. By location and histologic features, this lesion appears to represent a rare, distinct clinicopathologic entity, perhaps derived from misplaced cranial neural crest cells.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
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