• 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.

• Journal of the korean academy of Pediatric Dentistry
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• v.24 no.1
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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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• v.5 no.1
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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 %.

• Korean Journal of Head & Neck Oncology
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• v.28 no.2
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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.

• The Korean Journal of Cytopathology
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• v.9 no.2
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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.

• Korean Journal of Cleft Lip And Palate
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• v.8 no.1
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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.

• Korean Journal of Head & Neck Oncology
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• v.27 no.1
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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.

• Korean Journal of Cleft Lip And Palate
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• v.10 no.1
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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.

• Journal of the Society of Cosmetic Scientists of Korea
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• v.25 no.2
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• pp.45-57
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• 1999

Melanocytes, derived from neural crest, make melanin and protect skin from the hazardous ultraviolet light. Melanocytes with dendritic process has similar morphology with neurogenic cells and share growth factor receptors such as neurotrophin receptors. Melanogenesis can be regulated by ultraviolet light and inflammation of the skin. In addition, several factors such as hormone, cytokines, arachidonic acid can affect the proliferation and melanogenesis of melanocytes. For melanogenesis, melanocytes need expression of various genes including tyrosinase, TRP-1, TRP-2. In addition, melanin need to be transferred from melanocytes to surrounding keratinocytes. The biology of melanocytes is complex and mechanism of melanocytes proliferation and melanogenesis is still under the investigation.

• Korean Journal of Head & Neck Oncology
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• v.39 no.2
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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.