• 대한골관절종양학회지
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• 제9권1호
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• pp.77-83
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• 2003

공격성 섬유종증은 비교적 드문 연부 조직 종양이지만, 국소 증식 및 침윤성이 강한 종양으로 알려져 있다. 그렇지만 종양 증식에 관한 정확한 기전은 아직 보고되어 지지 않고 있다. 한편, 종양 억제 유전자 PTEN은 국소 침윤성과 전이를 보이는 여러 암 조직에서 발현의 감소 또는 돌연 변이가 보고되고 있다. 본 연구에서는 공격성 섬유종증에서 면역조직화학적 염색과 면역탁본법을 통해 종양 억제 유전자 PTEN의 발현의 양상을 알아보고자 하였다. 면역 탁본법과 면역조직화학적 염색상 PTEN의 발현은 정상 근막 조직에서는 균일하게 발현되었으나, 공격성 섬유종증 종양조직에서는 발현이 감소하였다. 공격성 섬유종증에서 PTEN발현의 감소는 급속한 증식과 재발 및 주위 조직의 침윤이 특징인 공격성 섬유종증의 병인에 관련이 있을 것으로 사료된다.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제16권2호
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• pp.186-195
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• 1994

Fibromatosis is benign fibroblastic proliferative lesion with abundant collagenous neo-formation located principally in the abdominal wall and in the upper and lower extremities (Masson & Soule, 1966). Wilkins and Waldron, in 1975, suggested that the title aggressive fibromatosis was a more appropriate term, reflecting the invasive characteristics of the disease. Synonyms listed were extra-abdominal desmoid, juvenile fibromatosis, aggressive infantile fibromatosis and congenital fibrosarcoma. A total of 12% of all fibromatosis arise in head and neck. Fibromatosis of the oral cavity is uncommon and is even more rare when in involve the mandibule. It is a locally aggressive fibrous tissue tumor, generally does not metastasize, but may cause considerable morbility and even death due to local infiltration. The degree of microscopic cellularity is variable, not only from tumor to tumor but also from area to area in the same tumor. Some tumors present with proliferation of mature fibroblasts and a dominating collagenous component : others may show a lack of the tumor in both types. The common histologic denominator appears to be cellular interlacing bundles of elongated fibroblasts, showing little or no mitotic activity and no pleomorphism. Mitosis are not a consistent index of malignancy when found in younger age groups. Fibromatosis still posses difficult problems of diagnosis and treatment. It is frequently recurrent and infliltrates neighbouring tissues. These lesion infliltrate widely and replace muscle, fat, and even bone with fibrous tissue of varying cellularity. Lesion representing fibromatosis in the oral cavity must be carefully evaulated by both surgeon and pathologists to ensure proper diagnosis and treatment planning. When these lesions involve bone, surgeon must be aware of the lesion's potential to perforate the cortex and expand while remaining hidden from the surgeon's view. Careful and precise clinical correlation with histologic appearance is essential to preclude misdiagnosis of fibrosarcoma yet provide surgical treatment plan that provides adequate local excision and long-term follow up. As regards cause, little is known. It is attributed to trauma or alteration in the sex hormone(Carlos, et al, 1986). Clinially, the lesion is reported to be not painful in most cases, but capable of rapid growth. The treatment is essentially surgical excision with wide margin of adjacent uninvolved tissue. Radiotherapy, hormone treatment or chemotherapy are of no use (WIkins et al, 1975 ; Majumudar and Winiarkl, 1978). We report a case of aggressive fibromatosis of 15-year-old with a lesion in the soft tissue of the parotid area that invaded the underlying bone of the mandibular body.

• 대한두경부종양학회지
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• 제25권2호
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• pp.168-170
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• 2009

Fibromatosis is a broad group of benign fibroblastic proliferation that shows locally aggressive growth but never metastasize. Common anatomic sites include abdominal wall, extremity, and mesentery. Little is reported about clinical features and outcome of fibromatosis of the head and neck. The treatment of choice is wide excision, which is often difficult. Postoperative recurrence rates are high. We recently confirmed a unique case of fibromatosis occurred on the hypopharynx of 44-year-old male patient and report this interesting case with review of literature.

• Journal of Yeungnam Medical Science
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• 제8권2호
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• pp.217-221
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• 1991

The fibromatosis is a rare tumorous with local invasion, but is not metastasized distantly. This term should not be applied to nonspecific reactive fibrous proliferations that are part of an inflammatory process of are secondary to injury of hemorrhage and have no tendency toward growth or recurrence. It arises principally from the connnective tissue of muscle and overlying fascia or aponeurosis(musculaponeurotic fibromatosis), and chiefly affects the muscle of shoulder, pelvic girdle, and extremity. The term 'aggressive fibromatosis' is also employed to describe this disease, but it is impossible to predict the clinical course in the individual case. The fibromatosis arising in the mediastinum is very rare, and the report about it is nearly absent. The plain radiography shows merely mass with soft tissue density. The CT demonstrates a poorly defined homogenous or heterogenous mass, isodense with skeletal muscle on precontrast-images, and slightly hyperdense to muscle on postcontrast-scan. Accurate delineation between the tumor & surrounding tissue is vague or frequently impossible. The authors experienced one case of the mediastinal fibromatosis recently and report the case with review of concerned literature.

• Korean Journal of Radiology
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• 제22권6호
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• pp.944-950
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• 2021

Objective: To demonstrate the feasibility of percutaneous microwave ablation in desmoid fibromatosis with respect to tumor volume control and improvement in the quality of life. Materials and Methods: Twelve microwave ablations were performed in 9 patients with a histological diagnosis of desmoid fibromatosis between January 2010 and January 2019. The study population included 6 female and 3 male, with an age range of 21-76 years (mean = 46.6 years; standard deviation [SD] = 19.3 years). The mean major axis of the tumors was 10.9 cm (SD = 5.2 cm) and mean lesion volume was 212.7 cm³ (SD = 213 cm³). Their anatomical distribution was as follows: 3 lesions in the thigh, 2 in the gluteus, 2 in the leg and 2 in the periscapular region. We evaluated the reduction in tumor volume and improvement in the quality of life based on the Eastern Cooperative Oncology Group (ECOG) scale. Results: An average tumor volume reduction of 70.4% (SD = 24.9) was achieved, while the quality of life (ECOG scale) improved in 88.9% of patients. Conclusion: Percutaneous microwave ablation may potentially be a safe, effective, and promising technique for controlling tumor volume and improving the quality of life in patients with desmoid fibromatosis.

• Imaging Science in Dentistry
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• 제41권2호
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• pp.85-88
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• 2011

Central odontogenic fibroma is a rare odontogenic neoplasm that originates from odontogenic ectomesenchyme. Here, a case of central odontogenic fibroma in a 17-year-old male is reported. Since the present case showed a multilocular radiolucency with partially ill-defined border between the right mandibular condyle and the distal root of the right mandibular third molar, differential diagnosis involved a wide range of pathosis from benign lesions like ameoloblastic fibroma and odontogenic myxoma to more aggressive lesions such as desmoplastic fibroma, juvenile aggressive fibromatosis, or fibrosarcoma.

• 대한골관절종양학회지
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• 제1권2호
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• pp.205-209
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• 1995

A desmoid tumor is a locally aggressive growth of connective tissue origin which infiltrates the surrounding tissue and has a marked tendency for recurrence. And so it was also called as an aggressive fibromatosis, musculofascial fibromatosis or fibrosarcoma etc. Thirteen cases of desmoid tumor was treated since 1980, and their retrospective study was done with 79 months of follow-up after initial surgical excision. The female was involved in 12 cases(one male) with the age ranged from 7-50 years, average 28 years, and seven patients in third decade. A slowly growing mass was excised on average 4 months after first notice of the mass, but their margins are not demarcated clearly in most cases. Wide excision in 12 cases was done, but wide excision and saphenous vein graft was performed in one case because of invasion of posterior tibial artery by tumor mass. The tumor was found on extraabdominal region in 8 cases(61.5%) but 5 cases in abdominal wall(38.5%). The recurrence rate was high(6/13, 46.2%), and 11 times in 6 patients were recurred(average 1.8 times), within 27 months of initial excision. Six cases of recurrence were treated with wide excision again in 3 cases, wide excision combined with radiotherapy(4,000-6,000cGy) in 4 cases and wide excision with chemotherapy in one case. During the follow-up for average 21 months after treatment, no recurrences are found. Tumor remission periods without recurrence are average 67 months in all, and 11 years in longest case. Histologically it was very mimic with fibrosarcoma but could be differentiated with Trichrome stain, and their findings are not changed after recurrence.

• Radiation Oncology Journal
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• 제37권1호
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• pp.37-42
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• 2019

Purpose: To identify prognostic factors influencing progression-free survival (PFS) of aggressive fibromatosis (AF) after postoperative radiotherapy (PORT) and assess correlations between immunohistochemistry (IHC) features of β-catenin/smooth muscle actin (SMA) and PFS. Materials and Methods: Records of 37 patients with AF treated by PORT from 1984 to 2015 were retrospectively reviewed. Fifteen patients underwent wide excision for AF and 22 patients received debulking operation. The median total dose of PORT was 59.4 Gy. IHC staining results of β-catenin and SMA were available for 11 and 12 patients, respectively. Results: The median follow-up duration was 105.9 months. Five-year PFS rate was 70.9%. Tumor size or margin status was not related to PFS in univariate analysis (p = 0.197 and p = 0.716, respectively). Multivariate analysis showed that increased interval from surgery to PORT (>5.7 weeks) was a marginal risk factor for PFS (p = 0.054). Administration of PORT at the initial diagnosis resulted in significantly improved PFS compared to deferring PORT after recurrence (p = 0.045). Patient with both risk factors of deferring PORT after recurrence and interval from surgery to PORT >5.7 weeks had significantly lower 5-year PFS than patients without risk factor (34.1% vs. 100.0%; p = 0.012). Nuclear β-catenin intensity tended to inversely correlate with 5-year PFS, although it did not reach statistical significance (62.5% at low vs. 100.0% at high; p = 0.260). SMA intensity was not related to PFS (p = 0.700). Conclusion: PORT should be performed immediately after surgery irrespective of margin status or tumor size especially in recurrent case. Nuclear β-catenin staining intensity of IHC might correlate with local recurrence.

• Archives of Plastic Surgery
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• 제36권3호
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• pp.344-347
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• 2009

Purpose: Malignant soft tissue tumors, known as sarcomas, are well known to be locally aggressive, frequently metastatic, and highly recurrent. In other hands, intermediate soft tissue tumors often recur locally with adjacent tissue infiltration so the clinical management is difficult as sarcoma. In the present study, we evaluate the clinical course of the intermediate soft tissue tumors and consider the management plan for those. Methods: From March, 1998 to April 2008, total 3 patients of intermediate soft tissue tumors underwent operations. A patient with fibrohistiocytic neoplasm, a free flap reconstruction was performed, and he underwent several more operations because of recurrences. Other patients with desmoids - type fibromatosis showed local invasion and adhesion, and one of them underwent reoperation due to local recurrence. Results: 2 of 3 patients underwent recurrences of tumors and reoperations were performed. In another patient with no recurrence, follow-up period was just 5 months, so there may be recurrence of tumor in long term follow-up. Conclusion: The clinical course of intermediate soft tissue tumors shows high recurrence rate. So clinically, intermediate soft tissue tumors should be considered as sarcomas. The successful management requires wide resection, carefully planned reconstruction, and close follow up with radiologic evaluation.

• 치과방사선
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• 제29권1호
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• pp.357-365
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• 1999

Desmoplastic fibroma is a rare. benign intraosseous fibroblastic tumor. which is locally aggressive. It is osseous counterpart of soft tissue fibromatosis. The authors experienced the patient who complained persistent mouth opening limitation with mild swelling on the left mandibular angle area. After careful analysis of clinical. radiological and histopathological findings. we diagnosed as desmoplastic fibroma of the mandible. The results were as follows: 1. Main clinical symptoms were mouth opening limitation which had been persistent for 9 months and mild swelling on the left mandibular angle area. 2. Radiographs showed the radiolucent lesion and expansion of lingual cortex. CT finding is homogeneous soft tissue mass with expansion of left mandibular ramus. Destruction of medial wall of ramus and invasion to adjacent soft tissue is also seen. 3. Histopathologically, plump spindle shaped fibroblasts arranged in bundles or fascicles are observed. The cells of tumor are infiltrating into muscle fiber with destruction of bony trabeculae and merged with surrounding salivary gland.