• 대한소아치과학회지
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• 제24권1호
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• pp.19-26
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• 1997

Odontodysplasia is a rare developmental anomaly of tooth formation in which hard tissue are affected. Odontodyplasia affects the primary and permanent dentitions in the maxilla, mandible, or both jaws. The maxilla is involved twice as often as the mandible. The condition is more common in female than in male patients and in the anterior than in the posterior regions. The clinical manifestation of odontodysplasia are hypoplasia and hypocalcification of the enamel and dentin of affected teeth. Teeth tend to be small and discolored, with short roots and widely open pulp canal. Delayed eruption of affected teeth with abscess formation is common. Radiographically teeth assume a faint radiolucent image ("ghost teeth"). enamel and dentin appear thin and are similar in radiodensity. The pulp chambers are often larger than normal, calcifications(pulp stone and denticle) are found within them. The etiology of regional odontodysplasia is unknown. However, several causes have been discussed, including somatic mutation, local circulatory disorders, local trauma, failure of migration and differentiation of neural crest cells, local infection.

• Advances in pediatric surgery
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• 제5권1호
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• pp.58-63
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• 1999

Neuroblastoma is a solid tumor derived from neural crest cells of the sympathetic nervous system. It is the most common extracranial solid tumor in children. Although it has the highest rate of spontaneous regression, it has a bad prognosis. Recent reports indicate a much improved outcomes utilizing the multitreatment approaches and early diagnosis as a result of patient screening. We have studied 42 patients managed in the last decade at the Severance and Yongdong Severance Hospitals. The patients were followed until January 1998 and analyzed in terms of age, sex, admission period, stage, diagnostic studies, clinical symptoms, physical findings, operative time, treatment modalities, and survival rate. Twenty eight patients underwent operative procedures, 16 patients had postoperative chemotherapy, and 19 patients had preoperative chemotherapy. Sexual difference was 1.33:1 in favor of males, and 43 % of patients were under one year of age. The patients were initially diagnosed as a result of symptoms and signs. An abdominal mass was the most common clinical finding. Eighty six percent of the patients were in advanced stages (>Stage III of INSS). The 2 year survival rate was 59.2 % and the 5 year survival rate was 29.6 %.

• 대한두경부종양학회지
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• 제28권2호
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• pp.149-152
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• 2012

Paraganglioma of the thyroid is a neuroendocrine tumor originating from the neural crest. To our knowledge, only 32 cases of thyroid paraganglioma(TP) have been reported to date. TP is difficult to distinguish from medullary thyroid cancer(MTC), hence correct diagnosis is seldom obtained preoperatively. Although immunohistochemistry is helpful for confirmation of TP, these markers are not specific for differential diagnosis between TP and MTC. Therefore, an index of suspicion is important when encountered with MTC with unusual features, or given pathology with neuroendocrine features and unusual immunohistochemical findings. The authors report a case of TP misdiagnosed as MTC at preoperative work-up and intraoperative frozen section analysis, with a review of the literature. The case depicts various immunohistochemical characteristics of the tumor.

• 대한세포병리학회지
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• 제9권2호
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• pp.233-239
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• 1998

Clear ceil sarcoma(CCS) is an uncommon soft tissue sarcoma that occurs in tendons and aponeuroses, usually of the lower extremities and is believed to be of neural crest origin that have a capability to produce melanin. These tumors commonly metastasize and have a very poor prognosis. The fine needle aspiration cytologic finding of CCS is not well documented. We recently experienced a case of CCS. The patient was a 54-year-old male with painful swelling of the right inguinal area. Fine needle aspiration cytology revealed polygonal or fusiform tumor cells with clear or granular cytoplasm and vesicular nuclei containing one or two nucleoli. Immunohistochemical staining for S-100 protein and HMB-45 revealed strong positivity and variable developing stages of premelanosomes were observed by electron microscopy in the excised specimen.

• 대한구순구개열학회지
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• 제8권1호
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• pp.23-29
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• 2005

The facial cleft and duplicated maxilla are lire congenital anomaly. After Rushton and Walker had reported a unilateral facial cleft with excess tooth and bone formation in 1937, few authors described similar cases. The etiology of this anomaly is not well understood, but considered embryologically as a neurocristopathy. A neurocristopathy is defined as a condition arising from aberrations in early migration, growth and differentiation of neural crest cells. This aberrations result in facial malformation such as facial clefts and loss or duplication of facial structures. We experienced a male newborn baby with bilateral facial cleft and duplicated maxilla. The cleft was surgically corrected when he was 5 months old. The function and appearance of lip are improved. Duplicated maxilla will be surgically removed. We report this case with review of literatures.

• 대한두경부종양학회지
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• 제27권1호
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• pp.80-83
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• 2011

Paraganglioma is a relatively rare, generally benign and slow growing tumor arising from widely distributed paraganglionic tissue thought to originate from the neural crest. In the head and neck region, paraganglionic tissue is distributed in the superior and inferior paraganglia such as the carotid body, the vagal body, and the jugulotympanic region. Approximately 10% of patients with paraganglioma have a family history of such tumors. Multiple lesions can be found in 26% of patients with family history. It is important to distinguish between paraganglioma and atypical carcinoid tumor and medullary thyroid cancer. This can be done with histopathologic evaluation and immunohistochemical markers. Complete excision is recommended for the treatment of this tumor. Paraganglioma is particularly aggressive and rapid recurrence of paraganglioma is common after local resection. We present a case of malignant paraganglioma in the neck which recurred 2 months after local resection. We suppose that radiation therpay could be the last option for the treatment in this clinical setting.

• 대한구순구개열학회지
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• 제10권1호
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• pp.17-22
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• 2007

The vertebrate upper lip forms from initially freely projecting maxillary, medial nasal, and lateral nasal prominences at the rostral and lateral boundaries of the primitive oral cavity. These facial prominences arise during early embryogenesis from ventrally migrating neural crest cells in combination with the head ectoderm and mesoderm and undergo directed growth and expansion around the nasal pits to actively fuse with each other. Initial fusion is between lateral and medial nasal processes and is followed by fusion between maxillary and medial nasal processes. Fusion between these prominences involves active epithelial filopodial and adhering interactions as well as programmed cell death. Slight defects in growth and patterning of the facial mesenchyme or epithelial fusion result in cleft lip with or without cleft palate, the most common and disfiguring craniofacial birth defect. This review will summarize the current understanding of the basic morphogenetic processes and molecular mechanisms underlying upper lip development.

• 대한화장품학회지
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• 제25권2호
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• pp.45-57
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• 1999

멜라닌세포는 신경능에서 기원한 세포로서 멜라닌을 생성하여 피부를 자외선으로부터 보호하는 중요한 기능을 수행하고 있다. 멜라닌세포는 수지상 돌기를 갖는 등 신경세포와 형태학적으로 유사하며 많은 신호 전달 물질, 성장 인자 등에 대한 수용체를 공통적으로 갖고 있어 발생학적 기원이 신경 세포와 같음을 보여주는 많은 특징들이 있다. 멜라닌세포는 자외선, 염증 등의 외부조건, alpha-MSH 등의 호르몬, IL-1, TNF-alpha, GM-CSF 등 의 싸이토카인, leukotriene 등 여러 가지 인자의 영향을 받고 있다. 또한 멜라닌세포로부터 멜라닌이 생성되기 위해서는 tyrosinase, TRP-1, TRP-2 등 여러 가지 유전자와 그에 해당하는 효소들의 작용, 또한 멜라닌이 각질형성세포로 이동하는 과정에 역시 여러 종류 의 유전자가 관여하고 있어 피부의 색소형성 과정을 이해하기 위해서는 이러한 과정을 복합적으로 이해하여야 한다(그림 1). 그러나 아직까지 멜라닌세포의 멜라닌 형성 과정과 증식, 사망 과정이 정확히 어떻게 진행되는지에 대한 연구가 부족한 실정이다.

• 대한두경부종양학회지
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• 제39권2호
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• pp.45-48
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• 2023

Malignant peripheral nerve sheath tumor (MPNST) is one of the soft tissue sarcoma believed to originate from neural crest cells. The patients with neurofibromatosis type I (NF1) have about 8-13% of the lifetime risk of the malignant transformation. Neurofibroma on patients with NF1 can be surgically resected and has good prognosis if approach to the tumor is possible. We experienced a case of a 50-year-old woman with NF who had incompletely resected neurofibroma, which presented a rapid malignant transformation to MPNST, 3 months after the first surgery. We reported this case with a brief review of literature.

• Journal of Chest Surgery
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• 제29권9호
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• pp.1023-1027
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• 1996

경동맥체 종양(carotid body tumor)은 총경동맥 분지부에 위치한 경동맥체 즉 화학 수용기에서 발생하는 종양으로서 그 발생빈도가 드물다. 이 경동맥 체의 기능은 혈액내 화학조성에 의해 호흡,혈압, 맥박수의 반사조절이 이루어지는 것으로 알려져 있다. 조직학적으로 대부분 주세포(chiefcell)로 구성되어 있고 대부분 피막에 쌓여 있으며 분엽성, 압축성으로 서서히 자라나중에 경부혈관을 둘러싸 압박하게되며 그 이외 다른 혈관이나 신경을 누르게 된다. 경동맥체의 치료방법은 수술적 절제, 방사선 조사, 색 전술등이 알려져 있으며 이중 외과적 절제가 대표적인 치료 방법으로 알려져 있다. 하지만 외과적 절제가 쉽지 않고수술중비교적 높은 이병율과뇌혈류 부전으로 인한 반측마비등의 후유증을 유발할 수 있기 때문에 고도의 주의를 요한다. 저자들은 경동맥 체종양으로 진단받고 절제수술을 받은 2례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.