• Journal of Yeungnam Medical Science
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• v.30 no.2
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• pp.105-108
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• 2013

Type 1 neurofibromatosis (von Recklinghausen's disease, NF-1) is an autosomal-dominant neurocutaneous-disorder characterized by systemic cafe'-au-lait spots, multiple cutaneous neurofibromas, axillary or inguinal freckling, and Lisch nodules (pigmented iris hamartomas). Approximately 10-25% of NF1 patients have gastrointestinal neoplasms. Gastrointestinal stromal tumor (GIST) in patients with neurofibromatosis is most commonly found in the small bowel and the stomach, and approximately 60% of such patients have multiple tumors or multiple tumor sites. Although, the increased incidence of GIST in patients with neurofibromatosis is well documented in pathology literature in English, but has rarely been documented in Korea. Here, we report a case of multiple GISTs in a 48-year-old woman accompanied by NF1. She was admitted to Yeung-nam University Hospital with complaints of melena and dyspnea. A contrast-enhanced computed tomography (CT) scan revealed that multiple soft tissue masses were occupying the entire peritoneal cavity. An ultrasonogram- guided biopsy was performed and the tumors were found to have been composed of tumor cells that were positive for c-kit protein. The patient was put on Imatinib mesylate treatment, and further follow-up will be carried out.

• Journal of Genetic Medicine
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• v.6 no.1
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• pp.91-94
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• 2009

Werner syndrome (WRN), or adult progeria, is a very rare, autosomal recessive disorder characterized by the appearance of accelerated aging, including cataracts, gray hair, skin atrophy, and atherosclerosis. This syndrome is caused by mutations in the WRN gene and had a high risk of a spectrum of rare neoplasms including: i) non-epithelial malignant or pre-malignant tumors/conditions, osteosarcomas and soft tissue sarcomas, malignant melanomas, myeloid leukemia and myelodysplastic syndrome; ii) an epithelial neoplasm, thyroid carcinoma, and iii) meningiomas. Recently, authors experienced a case of Werner syndrome complicated by bone metastasis of rhabdomyosarcoma in a 20-year old Korean man. The patient revealed a painful mass on his right knee and progeroid features, short stature, scalp alopecia, abnormal dentition, craniofacial disproportion, hypothyroidsm, cataracts and osteoporosis. The onset of symptoms of Werner syndrome generally precedes any later symptoms of associated conditions, such as malignant tumor. Therefore, early recognition of Werner syndrome is important to assist identification of malignant tumors at an early stage in this patient group.

• Journal of Chest Surgery
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• v.25 no.6
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• pp.598-604
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• 1992

Rhabdomyosarcoma is the most common soft tissue sarcoma in childhood and acounts for 6% to 15% of all cases of childhood cancer, Rhabdomyosarcoma in seventh most common form of childhood neoplasms, following acute leukemia, tumors of the central nervous system, lymphoma neuroblastoma, Wilm`s tumor, bone tumor. Rhabdomyosarcoma can arise anywhere in the body, but primary site in the thorax is relatively rare. We experienced a case of aveolar rhabdomyosarcoma arising from intercostal muscle, A 12 year-old woman was suffered from the intermittent left chest pain radiating to the scapular area and dyspnea, On physical examination, pulmonary friction rub was heard on the left upper lobe area. Qn adimission, the chest simple radiography revealed a 7 x 6, 5cm sized radio-opaque mass with pleural effusion in the superior mediastinum and the CT showed a well difined radio-opaque mass including the destructed 2nd rib and pleural effusion. The percutaneous tra-nsthoracic needle aspiration biopsy was likely to show blastoma. After the chemotherapy[vincristine, actinomycin-D, cyclophosphamde] was done to treat blastoma, the pleural effussion was subsided and the mass was slightly decreased by 4.5x 4. 5cm. For treatment and diagnosis, we performed en-bloc resection and the defected chest was reconstucted with Gortex patch. Grossly, the specimen was colored graysh-white and arised in between two ribs The microscopic findings showed that the tumor cells were small round with scant pinkish cytoplasm on the H-E stain and the tumor cell nests were grouped by reticulum fibers and showed alveolar pattern on the silver stain The electromicroscopic finding presented that the cytoplasm contained tangled fibrillar and flocculent materials. The histopathologic findings were compatable with laveolar rhabdomyosarcoma. She was discharged without any complication. After discharge, she has been treated with radiation theraphy and chemotheraphy, and not recurred untill last follow-up We report a case of alveolar rhabdomyosarcoma arising to intercostal muscle, developed in 12 year-old waman, with brief review of literatures.

• Archives of Craniofacial Surgery
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• v.16 no.2
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• pp.67-72
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• 2015

Background: A schwannoma is a benign, slow-growing peripheral nerve sheath tumor that originates from Schwann cells. Orbital schwannomas are rare, accounting for only 1% of all orbital neoplasms. In this study, we retrospectively review orbital schwannomas and characterize clinical, radiologic, and histologic features of this rare entity. Methods: A retrospective review was performed to identify patients with histologically confirmed orbital schwannoma, among a list of 437 patients who had visited our hospital with soft tissue masses within the orbit as the primary presentation between 2010 and 2014. Patient charts and medical records were reviewed for demographic information, relevant medical and family history, physical examination findings relating to ocular and extraocular sensorimotor function, operative details, postoperative complications, pathologic report, and recurrence. Results: Five patients (5/437, 1.1%) were identified as having histologically confirmed orbital schwannoma and underwent complete excision. Both computed tomography (CT) and magnetic resonance imaging (MRI) studies were not consistent in predicting histologic diagnosis. There were no complications, and none of the patients experienced significant scar formation. In two cases, patients exhibited a mild postoperative numbness of the forehead, but the patients demonstrated full recovery of sensation within 3 months after the operation. None of the five patients have experienced recurrence. Conclusion: Orbital schwannomas are relatively rare tumors. Preoperative diagnosis is difficult because of its variable presentation and location. Appropriate early assessment of orbital tumors by CT or MRI and prompt management is warranted to prevent the development of severe complications. Therefore, orbital schwannomas should be considered in the differential diagnosis of slow-growing orbital masses.

• Journal of Yeungnam Medical Science
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• v.28 no.1
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• pp.70-76
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• 2011

Rhabdomyosarcomas are soft tissue sarcomas; while extremely rare in adults, they are one of the most common neoplasms in children and adolescents. Histologically, they can be classified into embryonal (ERMS), alveolar (ARMS), pleomorphic, and undifferentiated types. The ARMS type is very rare, and is associated with a poor prognosis. Common primary sites of ARMS are the trunk and extremities. We report on a case of paraaortic, supraclavicular, and axillary lymph node metastasis from paratesticular ARMS treated with VAC (vincristine, dactinomycin, cyclophosphamide)/ IE(ifosfamide, etoposide) chemotherapy in a young adult. Administration of six cycles of chemotherapy with VAC/IE resulted in complete remission. The patient has maintained complete remission over the past 27 months.

• Archives of Plastic Surgery
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• v.39 no.4
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• pp.376-383
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• 2012

Background No consensus has been reached regarding the outcome of management of local recurrence after transverse rectus abdominis myocutaneous (TRAM) flap breast reconstruction. This study demonstrated the presentation, management, and outcomes of local recurrence after immediate TRAM breast reconstruction. Methods A comparison was conducted among 1,000 consecutive patients who underwent immediate breast reconstruction with a pedicled TRAM flap (TRAM group) and 3,183 consecutive patients who underwent only modified radical mastectomy without reconstruction (MRM group) from January 2001 to December 2009. The presentation, treatment, and outcome including aesthetics and overall survival rate were analyzed. Results Local recurrences occurred in 18 (1.8%) patients (TRAM-LR group) who underwent TRAM breast reconstruction and 38 (1.2%) patients (MRM-LR group) who underwent MRM only (P=0.1712). Wide excision was indicated in almost all the local recurrence cases. Skin graft was required in 4 patients in the MRM-LR group, whereas only one patient required a skin graft to preserve the mound shape in the TRAM-LR group. The breast mound was maintained in all 17 patients that survived in the TRAM-LR group even after wide excision. The overall survival rate was 94.4% in the TRAM-LR group and 65.8% in the MRM-LR group (P=0.276). Conclusions Local recurrence after immediate TRAM flap breast reconstruction could be detected without delay and managed effectively by multiple modalities without reducing overall survival rates. Breast mound reconstruction with soft autologous tissue allowed for primary closure in most of the cases. In all of the patients who survived, the contour of their reconstructed breast remained.

• Journal of Chest Surgery
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• v.41 no.1
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• pp.141-144
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• 2008

Low grade fibromyxoid sarcoma (LGFM) is a rare, deep soft-tissue malignant tumor. Although its histologic features are benign, the clinical course is malignant. The usual tumor locations are the lower extremity and chest wall. LGFM originating from the visceral pleura is extremely rare. We report here on a 37 year old man with a LGFM of the visceral pleura. Thirty three months after surgery, the patient is alive without any sign of local recurrence or distant metastasis.

• Journal of Chest Surgery
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• v.41 no.2
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• pp.289-291
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• 2008

Pleomorphic hyalinizing angiectatic tumor (PHAT) is a rare, low grade soft tissue neoplasm of an unknown histogenesis. It is characterized by sheets of mitotically inactive oval and pleomorphic cells, mono- and multi-nucleated giant cells, intranuclear cytoplasmic inclusions and prominent clusters of thin-walled ectatic vessels with perivascular hyalinization. We have experienced a 50 years old male patient who had a palpable mass in his right anterior lower chest wall. The mass was excised and it was confirmed as PHAT. He has been well 2 years postoperatively without recurrence.

• Archives of Reconstructive Microsurgery
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• v.18 no.1
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• pp.31-34
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• 2009

Purpose: Merkel cell carcinoma, also called neuroendocrine carcinoma, is a very rare type of skin cancer that develops as Merkel cells grow out of control. Merkel cell carcinoma is reported below 1% of whole skin neoplasms in the United States and is known that the 2-year survival rate is about 50~70%. The principles of treatment are wide excision of primary lesion with radiotherapy and/or chemotherapy that decrease the local recurrent rate. There has been no report of reconstruction with free flap after resection of Merkel cell carcinoma in Korea. Methods: We reconstructed the skin and soft tissue defect after wide excision of Merkel cell carcinoma with anterolateral thigh perforator free flap in two cases. No distant metastasis was found at the preoperative imaging work-up. In one case, preoperative chemotherapy was performed and the size of lesion was decreased. Results: There were no recurrence and significant complications. Functionally and aesthetically satisfactory results were obtained with reconstruction. Conclusion: Wide excision and reconstruction with anterolateral thigh perforator free flap for Merkel cell carcinoma patient is the first report in Korea. We regard this method as the treatment of choice in Merkel cell carcinoma.

• Journal of the korean academy of Pediatric Dentistry
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• v.25 no.2
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• pp.458-466
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• 1998

Non-odontogenic tumors can be classified as malignant or benign. Most oral tumors in children are benign. In the Belfast series only 7.5 percent of soft tissue tumors were malignant and Bhaskar(l963) found only 9 percent of 293 oral tumors of all kinds to be malignant. Benign tumors may be classified as epithelial and mesenchymal. The most common tumor of surface epithelium is the squamous papilloma. These are easily recognized clinically as cauliflower-like lesions. Fibrous lesions are very common in children's mouths. Many of these are not true neoplasms but are related to fibrous hyperplasia. Another common oral tumor in children is angiomatous tumors. Hemangioma occurred more frequently than lymphangioma. Cystic hygroma, a cystic subtype of lymphangioma, is a developmental tumor of lymphatic origin. It is a considered to be a relatively rare lesion. About 50 percent of cystic hygroma are present at birth, and most of the remaining 50 percent appear in the early years of life during the period of active lymphatic growth. The preferred treatment for these lesions, except for hemangioma, is complete surgical excision. With proper surgical techniques, recurrence is not expected.