• Journal of Korean Neurosurgical Society
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• v.60 no.3
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• pp.371-374
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• 2017

Ganglioneuroma (GN) is a rare benign tumor of neural crest origin usually found in the abdomen, but may occasionally present at uncommon sites including the cervical, lumbar, or sacral spine. However, GNs of thoracic spine are extremely rare. In this report, we describe a 12-year-old girl with giant GN in the thoracic spine, who underwent successful resection (T1-4 level) of the tumor. Histopathological examination confirmed the diagnosis. GN should be considered in the differential diagnosis of any paraspinal mass. A high index of suspicion and correlation of clinico-radiological findings is necessary in differentiating a large benign tumor from a malignant growth. Complete surgical excision is the treatment of choice; however tumor size and location need to be considered for the surgical approach (one-step or multiple surgeries). Close follow-up after surgery is mandatory.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.13 no.4
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• pp.436-443
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• 1991

A case of melanotic neuroectodermal tumor of infancy(MNTI) in 5 month old girl is presented with review of the literature. The review of literature indicated that there have been 189 reported cases of this lesion and there were 9 cases of malignant tumor for a rate of 4.8%. This rate of malignancy is very high in view of the fact that the tumor has been described as benign. And immunohistochemical study using avidin-biotin conjugate method demonstrated that this tumor had been originated from neural crest.

• Journal of Korean Neurosurgical Society
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• v.37 no.2
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• pp.137-140
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• 2005

Most vascular disorders tend to affect both the brain and heart, and among them, a clinical syndrome constituting cerebral aneurysm and aortic coarctation(AC) has been well recognized. Persistent hypertensive impact to the cerebral vasculature with developmental anomaly of the neural crest, precursor of ectomenchymal, would be closely associated with development of the cerebral aneurysm in AC. Gonadal steroid hormone, a guardian of the cardiovascular system, has been known for its protective effects on the vascular wall. Gonadal steroid hormone (androgen) insensitivity such as 46,XY female syndrome may increase the risk of hypertention and subsequent vascular anomalies. The authors report on a 46-year-old 46,XY female patient with AC who underwent surgical clipping of the ruptured cerebral aneurysm. Clinical implications and proposed pathogenetic mechanisms of aneurysm in this intersex syndrome are presented and discussed.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.10 no.1
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• pp.86-90
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• 2007

Carcinoid tumors are derived from the enterochromaffin cells of neural crest origin. Most are commonly found in the gastrointestinal (GI) tract. The rectum is the third most common site for GI carcinoids. Rectal carcinoid tumors make up 13.7% of all carcinoid tumors and the vast majority occurs in the sixth decade of life. Approximately 80% of rectal carcinoid tumors are less than 1 cm in size, limited to the submucosa without metastasis and can be safely treated by local excision. We report a case of rectal carcinoid tumor in a 13 year-old child that was successfully treated by endoscopic polypectomy.

• Proceedings of the KSAR Conference
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• 2001.10a
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• pp.53-54
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• 2001

Expression of HAND genes in sympathetic adrenal lineage suggests that HAND genes may regulate Mash-I independent neuronal genes. HAND genes are also expressed in other cell types, e.g. Cardiac cells, trophoblasts, and decidua, suggesting that HAND genes are not cell fate determination factors. It is unclear how HAND genes function specifically in different types of cells. Combinational actions of HANDs with other cell-lineage specific transcription factor may determine each cell fate and differentiation processes. Identifying the transcription target genes of HANDs and Mash-I will be important to elucidate the function of these bHLH factors in SNS factors in SNS development. (omitted)

• Advances in pediatric surgery
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• v.14 no.1
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• pp.75-82
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• 2008

Neuroblastoma arises from the primitive neural crest cells, and is a common malignancy in childhood. The clinical features are characterized by biological heterogeneity. Neuronal degeneration and differentiation occur in some patients. However treatment in the high risk group accounting for approximately half, has not been satisfactory despite a multimodal approach. Therefore, effective treatment is determined by the risk group of prognostic factors, such as age at diagnosis, stage of disease, pathological finding and N-myc amplification. Neuroblastoma can be diagnosed prenatally, which suggests its origin during the normal embryogenesis. Recent knowledge of molecular biology, such as Trk genes, and the concept of cancer stem cells have given us some improved understanding on this disease. Currently, targeted therapies based on the molecular biology of neuroblastoma are under investigation and increasing survival rate and decreasing late complications could be appreciated.

• Investigative Magnetic Resonance Imaging
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• v.25 no.1
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• pp.47-52
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• 2021

Paraganglioma is a rare tumor of paraganglia, derived from neural crest cells in sympathetic or parasympathetic ganglions. It can be widely distributed from the skull base to the bottom of the pelvis. The pancreas, however, is a rare location of this neoplasm, and only a limited number of cases have been reported in the English literature, especially with gadoxetic-acid-enhanced magnetic resonance imaging (MRI) and diffusion-weighted images (DWI). We herein report a case of pathologically proven paraganglioma in the pancreas head with a literature review on endoscopic ultrasonography (EUS), computed tomography (CT), gadoxetic-acid-enhanced MRI, and DWI sequence.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.31 no.2
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• pp.103-115
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• 2009

In vertebrates, the face is mainly formed with neural crest derived neural crest cells by the inherent programs and the interactive environmental factors. Extracellular signaling-regulated kinase (Erk) is one of such programs to regulate the various cellular functions. And retinoic acid (RA) also plays an important role as a regulator in differentiation process at various stages of vertebrate embryogenesis. We wanted to know that the segregation as well as the patterning of maxillary and mandibular structure is greatly influenced by the maxillomandibular cleft (MMC) and the failure of this development may result in the maxillomandibular fusion (syngnathia) or other patterning related disorder. It has been well documented that the epithelium at this cleft region has significant expression of Fibroblast growth factor (Fgf) 8, and it is essential for the patterning of the first arch derived structures. By the morphological, skeletal, cell proliferation and apoptotic, and hybridization analysis, we checked the effects of Erk inhibition and/or RA activation onto MMC and could observe that Erk and RA signaling is individually and synergically involved in the facial patterning in terms of FGF signaling pathway via Barx-l. So RA and Erk signaling work together for the MMC patterning and the segregation of maxilla-mandible by controlling the Fgf-related signaling pathways. And the abnormality in MMC brought by aberrant Fgf signaling may result in the disturbances of maxillary-mandibular segregation.

• Clinical and Experimental Pediatrics
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• v.48 no.4
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• pp.448-452
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• 2005

Neurofibromatosis(NF) is an autosomal dominant disorder, probably of neural crest origin that affects all three germinal layers. It is a group of heterogeneous multisystemic neurocutaneous disorders involving both neuroectodermal and mesenchymal derivatives. Type 1(von Recklinghausen disease) is the most common neurocutaneous disorder among the eight subtypes. Previous reports showed various involvements in the renal organ. Renovascular hypertension is the most common major manifestation of renal involvement in this disease. However, we experienced a case of ectopic kidney concurrent with neurofibromatosis type 1. The diagnosis of neurofibromatosis had been made by typical skin manifestation on physical examination, and ectopic kidney was discovered accidentally during routine abdominal sonography. The etiological basis of this association is not clear. We report a rare case of coexisting neurofibromatosis and ectopic kidney in a 7-year-old girl with a brief review.

• The Korean Journal of Cytopathology
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• v.1 no.1
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• pp.111-120
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• 1990

Small cell neuroendocrine carcinoma of the uterine cervix is a distinct subtype of cervical cancer that appears analogous to oat cell carcinoma and carcinoid tumors of the lung. It has been assumed to be derived from the neural crest via argyrophilic cells in the normal endocervix. We have recently encountered a case of small cell neuroendocrine carcinoma of the uterine cervix coexisting with adenocarcinoma which was argyrophil negative. A 66-year-old multiparous woman was admitted because of vaginal bleeding for 2 months. Cervicovaginal smear revealed several scattered clusters and sheets of monotonous small cells with some peripheral palisading in the background of hemorrhage and necrosis. Radical hysterectomy specimen revealed an ulcerofungating tumor on endocervical canal which was composed of two components. Major component of the tumor was made up of monomorphic population of small oval-shaped tumor cells arranged in sheets and partly in acinar structures or trabecular fashion. Other component was adenocarcinoma, endocervical well-differentiated type. Argyrophilia was present on the Grimelius stain and immunohistochemical studies revealed diffuse positivity to neuron-specific enolase and carcinoembryonic antigen. Electron microscopic examination showed clusters of small round to oval cells, which had a few well-formed desmosomes and several membrane-bound, dense-core neurosectetory granules.