• Title/Summary/Keyword: Desmoid

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Desmoplastic Fibroma - 2 Cases Report - (결합조직형성 섬유종 - 2례 보고 -)

  • Park, Yong-Koo;Ryu, Kyung-Nam;Han, Chung-Soo
    • The Journal of the Korean bone and joint tumor society
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    • v.6 no.2
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    • pp.92-97
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    • 2000
  • Desmoplastic fibroma is a rare primary tumor of bone that histologically and biologically mimics the extra-abdominal desmoid tumor of soft tissue. This report is dealt with two cases of desmoplastic fibroma occurring in a 22-year-old male, scapular lesion and 34-year-old male, pubic lesion. Radiologically, the tumors were lucent and expansile lesions. Histologically, they contained slender spindle cells and various amounts of collagen fibers. Radical excision was done on both cases and no recurrence was reported. Because of its rarity, we report two cases of desmoplastic fibroma.

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Desmoplastic Small Round Cell Tumor of the Mediastinum -A case report - (종격동에 발생한 결합조직형성 소원형세포 종양 - 1예 보고-)

  • Kim, Jeong-Won;Cha, Hee-Jeong;Park, Sang-Kyu;Jung, Jong-Pil;Shin, Je-Kyoun;Park, Chang-Ryul
    • Journal of Chest Surgery
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    • v.40 no.2 s.271
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    • pp.147-150
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    • 2007
  • Desmoplastic small round cell tumor (DSRCT) is rare and a recently described, poorly differentiated malignant tumor and it usually presents with widespread intra-abdominal involvement. We report a case of DSRCT arising form the mediastinum which was treated with multimodality anticancer therapy in 15 year-old girl.

DESMOPLASTIC FIBROMA OF THE MANDIBLE IN A CHILDREN : A CASE REPORT (어린이의 하악골에 발생한 결합조직형성 섬유종(Desmoplastic fibroma) 1예)

  • Yoon, Jung-Hoon;Lee, Jae-Ho
    • Journal of the korean academy of Pediatric Dentistry
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    • v.28 no.1
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    • pp.171-174
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    • 2001
  • This case describes a case of desmoplastic fibroma of the mandible. A 9-year-old boy was seen with a history of progressive swelling and expansion of the left mandible for one month period. Desmoplastic fibroma was diagnosed on histopathologic examination This report reviews the diagnostic criteria, differential diagnosis and surgical treatment of choice in brief of this uncommon primary bone tumor of the oral and maxillofacial region.

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A CASE OF AGGRESSIVE FIBROMATOSIS INVADED MANDIBULAR BODY ON THE PAROTID REGION (이하부에 발생한 침습성 섬유종증이 하악체에 침범한 증례 보고)

  • Kim, Young-Jo;Lee, Dong-Keun;Um, In-Woong;Min, Seung-Ki;Chung, Chang-Joo;Kim, Eun-Cheol
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.16 no.2
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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.

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Review of Primary Chest wall Tumors (원발성 흉벽종양의 임상적 고찰)

  • Sohn, Sang-Tae;Chon, Soon-Ho;Shin, Sung-Ho;Kim, Hyuck;Chung, Won-Sang;Kim, Young-Hak;Kang, Chung-Ho;Park, Moon-Hang;Jee, Heng-Ok
    • Journal of Chest Surgery
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    • v.31 no.10
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    • pp.988-994
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    • 1998
  • Background: Chest wall tumors can classified into soft tissue tumors and bone tissue tumors and can be subclassified into benign and malignant tumors. Materials and methods: We report an analysis of 68 patients with primary chest wall tumors treated at the department of thoracic and cardiovascular surgery at Hanyang University Hospital from January, 1973 to September 1997. Results: Among a total of 68 patients 33(48.5%) were males and 35(51.5%) were females. The ages of the patients ranged from 10 to 79 years with a mean age of 39.3 years. According to the age distribution, 23 patients (33.8%) were from the 4th decade, 12 patients(17.6%) were from the 6th decade, and 10 patients(14.7%) were from the 5th decade. Among the primary chest wall tumors, 53 cases were benign and 15 cases were malignant. Among the benign tumors, 17 cases(32.1%) were in the 4th decade and among the malignant tumors, 6 cases(40%) were in the 4th decade. In both malignant and benign tumors the most common ages were in the 4th decade. The most common tumors were fibrous dysplasia and chondroma, each with a total of 14 cases(26.4%). Osteochondroma and lipoma each had 8 cases(15.1%). Among malignant tumors, osteosarcoma was most common with 8 cases (53.3%). According to location, 49 cases occured in both bone and cartilage tissue, 19 cases occurred in cartilage. Among the presenting symptoms, palpable mass was present in all cases. Fifty-one patients complained of tenderness and among cases with involvement of the lung, 3 patients had complained of respiratory distress. Among the malignant tumors 6 cases underwent a radical operation and 4 cases of benign tumors underwent a radical operation. Postoperativly, there was one case with recurrence from a desmoid tumor. There were no deaths postoperativly and no deaths due to complications(and their postoperative courses were uneventful). Conclusions: Most patients with primary chest wall tumors initially present with mass at admission. Resection is sufficient treatment for benign tumors but in malignant tumors wide resection of the chest wall is needed and mchest wall reconstruction.

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