No abstract available.
Neural Plate* ; Neuroectodermal Tumors*

Neurofibromatosis(von Reckling hausen's disease) is a congenital heredofamilial disorderresulting from dysplasia of neuroectodermal and mesodermal tissues. It is characterized by developmental anormaly, pigmentary skin lesions and multiple tumors derived from the Schwannian cells of the peripheral and sensory nerve. The authors have experienced two cases of Neurofibromatosis. which is clinically diagnosed. and confirmed by histopathological examination. It was reviewed clinically with the literature.
Mesoderm ; Neural Plate ; Neurofibromatoses* ; Skin

The authers report a case of intracerebral extraventricular cyst of possible neuroectodermal origin, presenting as a right frontotemporoparietal mass lesion which has no communication with the subaracnhnoid space or ventricular system. A 4 months old male patinet recovered rapidly after partial removal of cystic wall, cystoventriculostomy and cystoperitoneal shunt. The pathogenesis and principles of diagnosis and surgical management of this rare lesion is discussed.
Diagnosis ; Humans ; Infant ; Male ; Neural Plate

Primitive neuroectodermal tumors(PNETs) are composed of undifferentiated cells resembling germinal matrix cells of the embryonic neural tube. The concept of the primitive neuroectodermal tumors is controversial due to indistinct clinicopathologic entities. While some neuropathologists believe that the PNET concept should be applied to all these tumors with the addition of qualifying terms, the opponents of this approach believe this concept to be too simplistic and that well-established diagnostic entities should not be grouped together as a single entity. Four patients with PNET were reviewed. Although the PNETs bear some differences to posterior fossa medulloblastomas, we should be grouped together as a single pathologic entity because of their primitive nature. The purpose of this study is to review the similarties and differences between two tumors from their histologic and embryologic features.
Humans ; Medulloblastoma ; Neural Plate ; Neural Tube ; Neuroectodermal Tumors, Primitive*

Neural crest cells have embriologically important role for the development and growth in oral and maxillofacial region. If the early hereditary defect occurs or environmental factors affect these cells diminutive mesenchymal cells are disabled to make neural plate and decreased proliferation of mesenchymal cells result in hypoplastic development of neural crest. As a result, this brings about severe facial malformations such as various located facial clefts and/or loss or duplication of facial structure. These are two cases of accessory maxilla and zygomatic deformity with and without facial cleft.
Congenital Abnormalities ; Growth and Development ; Maxilla* ; Neural Crest ; Neural Plate

Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) family mainly occurs from skeletal system. ES/PNET from the female reproductive organs such as ovary and uterus has been reported very rarely. Recently, we experienced one case of malignant ovarian neoplasm, which was diagnosed as extraosseous ES/PNET of ovary and received operation and chemotherapy. So, we report this case and include a brief literature review.
Female ; Humans ; Neural Plate ; Neuroectodermal Tumors ; Ovarian Neoplasms ; Ovary ; Uterus

Microphthalmos with cyst which results from a defect of ventral fissure is a rare malformation, with no previously reported case in Korea. The defect permits neuroectodermal tissue to proliferate into the orbit, forming a cystic structure that remains connected to the inferior aspect of the diminutive globe. We have observed such a malformation in a 8-year-old girl and surgically excised the diminutive eye and communicating cyst en-bloc.
Child ; Female ; Humans ; Korea ; Microphthalmos* ; Neural Plate ; Orbit

Neural crest and placodes share a number of important features, pointing to a possible common evolutionary origin. They both arise from the neural plate border, which is the boundary between the non-neural ectoderm and neural plate. The transcription factor Sox9 has been implicated in neural crest and otic placode induction in several species. To investigate the differential regulation of neural crest and otic placode induction by Sox9, a gain of function assay was performed using a hormone-inducible version of the Sox9 construct at different doses and time periods. Sox9 was expressed in both neural crest and otic placode cell populations in the same stage embryos by in situ hybridization. Using a gain of function approach, increased expression of neural crest marker (Snail2) and otic placode marker (Pax8) in Sox9-overexpressed embryos was observed. Higher dose of Sox9 reduced or eliminated both neural crest and placode cells in the embryos. Interestingly, otic placodes cells were more strongly affected as compared to neural crest cells. So, optimal dosage and timing of Sox9 expression are important for the development of the neural crest and otic placode. The development of the neural crest and otic placode are affected by Sox9 in a time- and dose-dependent manner.
Ectoderm ; Embryonic Structures ; In Situ Hybridization ; Neural Crest* ; Neural Plate ; Transcription Factors ; Xenopus

Variable manifestations of neurofibromatosis are properly defined as a hereditary, harmatomatous disorder, probably of neural crest origin, involving not only neuroectoderm and mesoderm but also endoderm, with the potential of appearing in any organ system of the body. Recently, we have experienced 3 cases of neurofibromatosis, one case associated with glioblastoma multiforme in left frontal lobe, another case associated with multiple neurofibromas in thoracic region and cauda equina, the third case associated with retroperitoneal neurofibroma and dural ectasia.
Cauda Equina ; Dilatation, Pathologic ; Endoderm ; Frontal Lobe ; Glioblastoma ; Mesoderm ; Neural Crest ; Neural Plate ; Neurofibroma ; Neurofibromatoses*

Spinal neurenteric cyst results from the persistence of an abnormal communication between endodermal and neuroectodermal layer. It is a rare malformation that lead to spinal cord compression. The patient is a 22-month-old boy with sudden, progressive left side motor weakness. Preoperative magnetic resonance image revealed intradural extramedullary cyst in C4-C5 level that compressed the spinal cord on the left side. After the resection of the cyst, motor weakness of the left side was partially improved, and his motor power was completely recovered after rehabilitation. Histologic examination revealed a ciliated columnar epithelial neurenteric cyst. The pre- and postoperative clinical features of the case of neurenteric cyst is described with the review of literature.
Endoderm ; Hemiplegia* ; Humans ; Infant ; Male ; Neural Plate ; Neural Tube Defects* ; Rehabilitation ; Spinal Cord ; Spinal Cord Compression