• Archives of Plastic Surgery
• /
• 제34권3호
• /
• pp.403-405
• /
• 2007

Purpose: Malignant degeneration of fibrous dysplasia is an uncommon recognized complication of this disease. Especially, degeneration of fibrous dysplasia to malignant fibrous histiocytoma(MFH) in facial bone is rare and the publications had been limited. The purpose of this report is to share our experience. Methods: A 46-year-old patient with facial fibrous dysplasia visited our clinic for recent facial tingling and swelling. Malignant degeneration of fibrous dysplasia was suspected. Results: Total excision of the mass and adjacent facial bone was performed. Defect was immediately reconstructed with bone graft and bone cement. At a month follow up, metastasis was detected at ipsilateral parotid gland. Superficial parotidectomy and neck dissection was performed. The patient is currently taking chemotherapy. Conclusion: Because of the uncommon presentation of this entity, clinical course of treatment was dependent on other histological types of malignant degeneration. We report this case to share our experience.

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

• Radiation Oncology Journal
• /
• 제13권3호
• /
• pp.225-231
• /
• 1995

악성 섬유성 조직구종은 상악골부위에서는 매우 드물게 발생하는 악성 종양으로서, 두경부에 발생되는 예가 전체 악성 섬유성 조직구종의 $7{\%}$ 이며 이들 $7{\%}$ 중 $12{\%}$가 상악골에 발생한다고 한다. 상악골의 악성 섬유성 조직구종은 국소재발이나 원격전이가 $55{\%}$의 환자들에서 발생하며 평균 생존기간은 상악골, 하악골 및 구강연부조직의 악성 섬유성 조직구종에 관한 연구에서 30개월로 보고되었다. 상악골의 악성 섬유성 조직구종은 일차적으로 근치적 수술요법이 주 치료방법이다. 방사선치료에 관한 보고들은 대상환자들이 적어서 지금까지 체계적으로 보고되지 않았고, 증례보고에 의한 종양의 퇴행 또는 조직학적 변화가 발표된 바 있으며 일부 저자들은 수술후 방사선치료가 도움이 되지 않으므로 재발성 또는 수술 불가능한 경우에 시행하기를 권하기도 한다. 재발성 또는 전이성 악성 섬유성 조직구종 환자들에서 항암화학요법으로 $33{\%}$의 관해율을 보였다는 보고가 있다. 저자들은 상악골의 악성 섬유성 조직구종 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Journal of Korean Neurosurgical Society
• /
• 제30권11호
• /
• pp.1340-1344
• /
• 2001

뇌로 전이한 악성 섬유성 조직구종 1례를 문헌 고찰과 함께 보고하고자 한다. 환자는 62세 남자로 다발성 전이성 뇌종양 의증하에 개두술 및 종양 제거술을 시행받았다. 술전 검사상 두개강외 원발 부위를 의심할만한 병변은 발견하지 못하였다. 수술과 전뇌 방사선 치료후 6개월째에 성장하는 우측 견부 종양이 발견되어 적출술을 시행하였다. 뇌와 견부 조직 표본에서 악성 섬유성 조직 세포종에 부합되는 일치된 병리 소견을 보였으나 GFAP-양성 세포들은 뇌병변의 조직 표본에서만 발견되었다. 이후에 발생한 척추 등의 골격계 전이성 병변에 대하여서는 고식적 방사선 치료를 행하였다. 최초 진단 후 12개월째 우측 견부 종양이 재발하여 항암 화학 치료 중이다. 이 증례에서 특이한 점은 원발 부위 발견전 전이성 뇌종양이 진단된 것인데, 악성 섬유성 조직구종으로부터 뇌로 전이한 경우가 드물거니와 특히 원발부위가 확인되기 전에 뇌로 전이한 병변이 먼저 발견된 경우는 보고된 바가 없다. 또 하나의 중요한 소견은 GFAP 발현이 뇌병변에서만 나타나며 원발부위인 견부 종양에서는 관찰되지 않은 점이다. 원발성 두개강내 악성 섬유성 조직구종에서 GFAP-양성 세포의 존재는 중요한 조직학적 특성의 하나로 알려져 있으나, 본 증례의 소견은 원발성 두개강내 악성 섬유성 조직구종에 존재하는 GFAP-양성 세포들도 악성 섬유성 조직구종에 의해 이차적으로 생성된 비종양성 성상 세포들이라는 가설을 뒷받침하고 있다.

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

• 대한골관절종양학회지
• /
• 제1권2호
• /
• pp.181-188
• /
• 1995

Malignant tumor of the pelvic bone has nonspecific symptom and it is not easily detected by physical examination or by radiologic study in early stage, because of its anatomical characteristics. Associated with their variety of disease entity, the treatment of malignant pelvic bone tumor is a problematic point. We have analysed 36 cases of malignant pelvic bone tumors diagnosed at the Severance hospital from 1968 to 1993 to provide a reference for diagnosis and treatment of the malignant pelvic bone tumors. We found that the chondrosarcoma(27.8%) and the osteogenic sarcoma(27.8%) were the most common type of pelvic bone malignancy, and then, in the order of incidence, there were Ewing's sarcoma(16.7%), malignant fibrous histiocytoma(11.1%). There were differences of the age distribution among each diseases and the average age was Ewing's sarcoma 20.5, osteogenic sarcoma 27.2, chondrosarcoma 40.0, malignant fibrous histiocytoma 64.8, respectively. Three of the 5 patients with low grade tumors survived(60%), whereas 3 of the 17 patients with high grade tumors survived(18%). The survival rate of the low grade malignant group was 60%, the high grade was 18%.

• Archives of Plastic Surgery
• /
• 제37권1호
• /
• pp.79-82
• /
• 2010

Purpose: Carcinosarcomas are rare neoplasms in which both malignant epithelial and mesenchymal elements are identified. We have found only twenty one cases of primary cutaneous carcinosarcoma in the English language literature. Therefore it has been difficult to diagnosis because its unclear etiology and low frequency. Methods: A 31-year-old young man with a protruding mass on his ankle dorsum was examined. The tumor was $7{\times}6{\times}3\;cm$ sized and arose from a burn scar. The mass was wide excised and applied split thickness skin graft. Characteristic pathologic finding was a mixture of squamous cell carcinoma and malignant fibrous histiocytoma. Results: Three months after the operation, the patient died of multiple metastasis to the liver, lung and finally of sepsis despite adjuvant chemotherapy and adjuvant radiotherapy. Conclusion: If a metastatic cutaneous carcinosarcoma is diagnosed, wide excision is required. There should be more aggressive management to minimize the risk of recurrence. Further research into the etiology and pathophysiology of the disease and a more careful differential diagnosis may allow improvement in treatment.

• Journal of Chest Surgery
• /
• 제39권10호
• /
• pp.802-804
• /
• 2006

악성 섬유 조직구종은 사지나 후복막의 연부조직에 주로 발생하는 종양으로 종격동에 발생하는 경우는 극히 드물다. 환자는 71세 남자 환자로 전종격동에 발생한 원발성 종양에 대해 종양 제거술을 시행하였고 조직학적으로 악성 섬유 조직구종으로 진단되었다.

• Tuberculosis and Respiratory Diseases
• /
• 제38권1호
• /
• pp.59-64
• /
• 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
• /
• 제18권4호
• /
• pp.792-799
• /
• 1985

Primary neoplasms of the trachea are rare, but are a very serious critical life-threatening disease. Nearly all the lesions of the trachea are presented as obstructive lesions. Bronchoscopic examination including chest C-T, tomogram and air tracheogram are essential for the further definition of these lesions. The need for removal of tracheal tumors whether complete or incomplete, is clear enough regardless of the histology of the tumor. We have experienced 9 cases from Jan. 1965 to June, 1985. One patient with tracheal hamartoma was cured with complete resection through rigid bronchoscopy and another patient with fibrous histiocytoma was treated with re-excision and laser evaporation through superior mediastinotomy due to recurrence, 1 year later. The remaining patients were treated with mass excision or segmental resection and end-to-end anastomosis through collar incision and superior mediastinal sternotomy. The remaining two patients were operated with and segmental resection and end-to-end anastomosis of trachea using partial cardiopulmonary bypass. The histologic diagnosis were adenoid cystic Ca[5], fibrous histiocytoma[1], mucoepidermoid Ca[1]. hamartoma[1], anaplastic Ca.[1]. Three patients were treated post-operatively with radiation; with adenoid cystic Ca.[2] and anaplastic Ca.[1]. Their post-operative courses were uneventful during the follow-up from 2 months to 7 years.