• Tuberculosis and Respiratory Diseases
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• 제48권3호
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• pp.388-393
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• 2000

저자들은 우측 흉통과 기침을 주소로 내원한 57세 남자환자에서 우측 폐하부에 $12{\times}8cm$ 가량의 종괴를 단순 흉부 X-선 촬영과 흉부 CT 촬영등으로 진단후 좌측 개흉술로 치료한 국소성 섬유성 종양을 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제36권6호
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• pp.303-307
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• 2014

Undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma, occurs commonly in the soft tissues in adult, but is rare in the maxillofacial region. It consists of undifferentiated mesenchymal tumor cells resembling histiocytes and fibroblasts. The purpose of this article is to report a case of UPS in the mandible. A 44-year-old patient presented with a painful growing mass in the mandible of two months' duration. Computed tomography and positron emission tomography-computed tomography revealed an ill-defined heterogenous, hypermetabolic mass about 4 cm in size in the left mandible invading adjacent soft tissues. A left mandiblulectomy and reconstruction with a fibular free flap were performed. Immunohistochemical study gave a diagnosis of UPS. The patient was referred for adjuvant chemotherapy after surgical removal of the tumor.

• Tuberculosis and Respiratory Diseases
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• 제38권1호
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• pp.59-64
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• 1991

The malignant fibrous histiocytoma was the most common soft tissue sarcoma in late life adult. It was first described in 1964 by 0' Brien and Stout. It's histiogenesis had been considered to be of histiocytic origin. It Involves the extremities, retroperitoneum and trunk. It usually metastasizes to the lung. but primary lung lesion is extremly rare and it's prognosis was poor. We have experienced a case of MFR, which was confirmed by open lung biopsy. So we report a case of MFR of the lung with review of literature.

• Journal of Chest Surgery
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• 제37권5호
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• pp.432-437
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• 2004

배경 : 고립성 섬유성 흉막 종양(SFTP)은 중피하 중간엽에서 기원한 매우 드문 종양으로, 임상 경과나 적절한 수술적 절제의 범위 및 추적 관찰 결과 등에 대해 알려진 바가 매우 제한적이다. 대상 및 방법: 9년간(1994∼2003) 양성 및 악성 고립성 섬유성 흉막 종양으로 수술적 절제를 받은 환자를 대상으로 후향적 연구를 시행하였다. 결과: 총 22명의 환자(남 14, 여 8)가 포함되었고 평균 연령은 50.2세 (25∼83세)였다. 진단 당시 증상을 나타낸 환자는 13명(59%)이었고 가장 흔한 증상은 호흡 곤란이었다. 개흉술이 14예, 비디오 흉강경을 이용한 수술이 8예에서 행해졌으며, 종괴만을 절제한 경우는 12예, 주변 구조물을 포함한 광범위 절제를 행한 경우는 10예였다 모든 경우에 완전 절제술이 가능하였으며 수술 사망은 없었다. 수술 후 병리학적 진단 결과 양성 종양이 11예, 악성 종양이 11예였다. 추적 관찰 중 국소 재발 2예, 전신 전이가 6예에서 있었으며 재발과 전이는 모두 악성에서만 발생하였다. 결론: 진단 당시 증상이 있는 환자, 광범위 절제술, 재발 등은 모두 악성형에서 유의하게 많았다. 아직까지는 수술적 완전 절제가 가장 적절한 치료로 알려져 있으나, 재발과 전이를 억제하기 위한 수술 전후 전신 치료의 방법에 대해서 더 많은 연구가 필요할 것으로 생각된다.

• 대한영상의학회지
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• 제85권3호
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• pp.654-660
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• 2024

원발성 악성 섬유성 조직구종은 요로, 특히 방광에서 흔하게 발생하지 않는 중간엽 유래의 악성 종양이다. 방광암의 대부분을 차지하는 요로 상피암과는 달리, 악성 섬유성 조직구종은 점막 고유층, 고유근층, 장막층으로 구성된 방광 벽의 요로 상피 하부에서 발생한다. 조직학적 기원은 섬유아세포와 조직구 세포가 부분적으로 분화된 저분화 중간엽 줄기세포에서 발생하는 것으로 추정된다. 영상의학적으로는 요로 상피암에서 흔히 볼 수 있는 유두상 성장 패턴을 보이지 않아 '비유두상 종양'으로 알려져 있으며, 흔히 고유근층 이상의 침범을 보이고 괴사를 동반하지 않는 거대 종괴의 형태로 진단된다. 이 드문 악성 종양의 예후는 다양한 병리학적 인자에 의해 결정되지만, 일반적으로 불량한 예후와 높은 국소 재발률을 보인다. 또한 무통성 혈뇨를 주로 호소하는 요로 상피암 환자와 달리 주변 장기를 압박하여 비특이적인 하복부 통증이 나타날 수 있어 진단에 어려움이 있을 수 있다. 저자들은 혈뇨 없이 하복부 통증을 주 증상으로 내원하여 방광의 원발성 악성 섬유성 조직구종으로 진단된 후 빠른 치료를 시작하였음에도 매우 공격적인 임상 경과를 보였던 증례를 보고하고자 한다.

• Imaging Science in Dentistry
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• 제33권4호
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• pp.239-244
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• 2003

Malignant fibrous histiocytoma (MFH) is a pleomorphic soft tissue sarcoma. Three cases of MFH were reported in our study. The first case involved in the right infratemporal fossa of a 32-year-old female was presented. MR imaging revealed a 5.0 × 3.3 cm soft tissue mass of inhomogeneous high signal intensity. The second case was found in the right hard palate of a 66-year-old male. CT demonstrated bone destruction and MR imaging showed a 4 × 4 cm sized soft tissue mass of heterogeneous high signal intensity. The final case was found in the left masticator space of a 37-year-old male. The CT image showed a large mass with massive bone destruction of the left mandibular ramus, while the MRI displayed a soft tissue mass, 8 cm diameter. Our cases exhibited the general features of MFH. MRI is essential in the imaging of MFH, namely to depict tumor borders and demonstrate relationships with adjacent structures.

• Tuberculosis and Respiratory Diseases
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• 제54권6호
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• pp.645-650
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• 2003

저자들은 우측 흉부 불편감을 주소로 내원한 25세 여자 환자를 개흉 조직 검사를 시행하여 원발성 횡격막 기원 악성 섬유성 조직구종을 진단하였고 ifosfamide, doxorubicin으로 항암 화학 요법 시행 후 부분 관해가 있었으나 진단 후 12개월째 외래 추적 검사 중 뇌전이로 사망한 젊은 여성 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• 대한골관절종양학회지
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• 제5권4호
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• pp.201-207
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• 1999

Malignant tumors of the hand are very rare. Between 1989 and 1998, 17 patients with malignant tumors of the hand were evaluated for clinical features, prevalence, treatment and results. The mean age was 45 years and median duration of follow-up was 38 months. A painless mass was the most common symptom and the most common lesion was the digit. Fifteen cases of soft tissue and 2 cases of bone tumor were diagnosed. On pathology, soft tissue tumors were comprised of 8 cases of malignant melanoma, 2 cases of angiosarcoma and 1 case each of malignant fibrous histiocytoma, malignant peripheral nerve sheath tumor, extraskeletal chondrosarcoma, and squamous cell carcinoma. Bone tumors were metastatic tumors and each originated from the rectal cancer or lung cancer. Lymph node involvements were noted in 4 cases of malignant melanoma and 1 case of squamous cell carcinoma. Six cases of metacarpo-phalangeal joint disarticulation and 4 cases of phalanx amputation were performed. Wide excision, ray amputation and below-elbow amputation were also performed. Three cases expired due to metastasis and progression of the original lesion. Among the surviving 14 cases, a malignant melanoma had metastasis on the axillary lymph node and 13 cases showed no local recurrence or metastasis during the follow-up.

• Archives of Reconstructive Microsurgery
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• 제17권1호
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• pp.14-18
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• 2008

Purpose: Evaluation of results of free flap as a method of reconstruction in soft tissue defect after wide excision of soft tissue tumor of extremity. Materials and Methods: From 2000 through 2007, 11 patients received free flap surgery for soft tissue defect after wide excision operation for soft tissue tumor of limbs. Four cases were upper extremities and seven were lower extremities. Four subjects were diagnosed as squamous cell carcinoma, three as malignant melanoma, two as synovial sarcoma and one as malignant fibrous histiocytoma and alveolar soft part sarcoma. Donor sites of free flap varied with anterolateral thigh flaps in six cases, latissimus dorsi flaps in four, reverse forearm flap in one. By the method of doppler ultrasound, venous circulation was evaluated for the survival of each flap on the third, fifth and seventh day respectively after the operation. Results: 10 of 11 free flaps were successfully survived. Necrosis of free flaps in 1 cases occurred in case of anterolateral thigh flap. Conclusion: Free flap can be a useful method for reconstruction of soft tissue defect after wide excision of soft tissue sarcoma of extremity.

• Brain Tumor Research and Treatment
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• 제6권2호
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• pp.86-91
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• 2018

Aneurysmal bone cyst (ABC) is a rare non-neoplastic bone lesion that involves mostly the long bones and vertebrae and may occur very rarely in the craniofacial bones. ABCs may occur as secondary bony pathologies in association with various benign and malignant bone tumors and with fibrous dysplasia (FD). FD is a common non-neoplastic bony pathology mostly affecting craniofacial bones. Secondary ABC occurring in craniofacial FD is extremely rare, with only approximately 20 cases reported in the literature to date. Here, we report on a case of secondary ABC in a 25-year-old woman who has had a craniofacial deformity for over 10 years and who presented to us with a rapidly growing painful pulsatile mass in the right frontal region that began over 2 months prior to admission. On thorough examination of computed tomography and magnetic resonance imaging brain scans taken at two-month interval, an aggressive, rapidly enlarging ABC, arising from the right frontal FD, was diagnosed. The patient underwent preoperative embolization followed by gross total resection of the ABC and cranioplasty. The 6-month follow up showed no recurrence of the ABC, nor was any progression of the FD noticed.