• Title/Summary/Keyword: Desmoid Tumor

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Clinical Investigation of Desmoid Tumors (유건종(類腱腫)의 임상적 고찰)

  • Rhee, S.K.;Kim, J.M.;Kim, H.M.;Kang, Y.K.;Kim, Y.S.;Kwon, S.Y.;Lee, K.T.;Kim, I.
    • The Journal of the Korean bone and joint tumor society
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    • v.1 no.2
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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.

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The Imaging Features of Desmoid Tumors: the Usefulness of Diffusion Weighted Imaging to Differentiate between Desmoid and Malignant Soft Tissue Tumors

  • Lee, Seung Baek;Oh, Soon Nam;Choi, Moon Hyung;Rha, Sung Eun;Jung, Seung Eun;Byun, Jae Young
    • Investigative Magnetic Resonance Imaging
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    • v.21 no.3
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    • pp.162-170
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    • 2017
  • Purpose: To evaluate the imaging findings of desmoid tumors using various imaging modalities and to evaluate whether diffusion-weighted imaging (DWI) can help differentiate between desmoid and malignant tumors. Materials and Methods: The study included 27 patients with pathologically confirmed desmoid tumors. Two radiologists reviewed 23 computed tomography (CT), 12 magnetic resonance imaging (MRI) and 8 positron emission tomography-computed tomography (PET-CT) scans of desmoid tumors and recorded data regarding the shape, multiplicity, size, location, degree of enhancement, and presence or absence of calcification or hemorrhage. The signal intensity of masses on T1- and T2-weighted imaging and the presence or absence of whirling or band-like low signal intensity on T2-weighted imaging were recorded. The apparent diffusion coefficient (ADC) values of the desmoid tumors in nine patients with DWIs were compared with the ADC values of 32 malignant tumors. The maximum standardized uptake value ($SUV_{max}$) on PET-CT images was measured in 8 patients who underwent a PET-CT. Results: The mean size of the 27 tumors was 6.77 cm (range, 2.5-26 cm) and four tumors exhibited multiplicity. The desmoid tumors were classified by shape as either mass forming (n = 18), infiltrative (n = 4), or combined (n = 5). The location of the tumors was either intra-abdominal (n = 15), within the abdominal wall (n = 8) or extra-abdominal (n = 4). Among the 27 tumors, 21 showed moderate to marked enhancement and 22 showed homogeneous enhancement. Two tumors showed calcifications and one displayed hemorrhage. Eleven of the 12 MR T2-weighted images showed whirling or band-like low signal intensity areas in the mass. The mean ADC value of the desmoid tumors ($1493{\times}10^{-6}mm^2/s$) was significantly higher than the mean of the malignant soft tissue tumors ($873{\times}10^{-6}mm^2/s$, P < 0.001). On the PET-CT images, all tumors exhibited an intermediate $SUV_{max}$ (mean, 3.7; range, 2.3-4.5). Conclusion: Desmoids tumors showed homogenous, moderate to marked enhancement on CT and MRI scans and a characteristic whirling or band-like pattern on T2-weighted images. DWI can be useful for the differentiation of desmoid tumors from malignant soft tissue tumors.

Osteosarcoma Arising from Desmoplastic Fibroma of Proximal Tibia - A Case Report - (근위 경골의 결합조직형성 섬유종 치료 후 발생한 골육종 - 1예 보고 -)

  • Kim, Tai-Seung;Park, Yong-Wook;Park, Moon-Hyang;Jang, Si-Hyong
    • The Journal of the Korean bone and joint tumor society
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    • v.13 no.2
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    • pp.162-166
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    • 2007
  • Desmoplastic fibroma of bone is rare, involves usually long bones, and histologically resemblance to the desmoid tumor of soft tissue. It is benign, but aggressive locally. Recurrence rate is high when treated with simple curettage and bone graft. Moreover, malignant transformation of desmoplastic fibroma with recurrence has been reported very rarely, and then it make us review again whether previous tumor probably was low grade malignant tumor. We report a surgically proven case of osteosarcoma arising from desmoplastic fibroma. A 17 years old male had been treated for a desmoplastic fibroma of the proximal tibia with curettage and bone graft. But after 11 years, the patient showed recurrence accompanying malignant transformation with osteosarcoma. This is very rare case of an osteosarcoma arising in a desmoplatic fibroma of the proximal tibia.

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The Necessity of Early Adjuvant Radiotherapy for Better Outcomes in the Treatment of a Desmoid Tumor (데스모이드 종양에 대한 조기 방사선치료의 필요성)

  • Lee, Me-Yeon;Chang, Ah-Ram;Kim, Hak-Jae;Kim, Kyu-Bo;Kim, Jin-Ho;Park, Charn-Il;Ha, Sung-Whan;Wu, Hong-Gyun;Kim, Il-Han
    • Radiation Oncology Journal
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    • v.25 no.4
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    • pp.201-205
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    • 2007
  • Purpose: This retrospective study was conducted to assess outcome and to determine the prognostic factors in patients with a desmoid tumor treated with postoperative radiotherapy. Materials and Methods: Twenty-seven patients with a desmoid tumor who were treated with postoperative radiotherapy between June 1984 and October 2005 were analyzed. There were 13 male and 14 female patients. The age of the patients ranged from 3 to 79 years (median age, 28 years). Tumors were located in an extra-abdominal area (21 cases), and in the abdominal walls (6 cases). The tumor size ranged from 2.5 to 25 cm (median size, 7.5 cm) in the largest linear dimension. Thirteen cases received radiotherapy after initial surgery, and 14 recurrent cases received radiotherapy after additional surgery. The total radiation dose given was $45{\sim}66$ Gy (median dose, 59.4 Gy), and the fraction size was $1.8{\sim}2.0$ Gy. Results: The median follow-up period was 61 months (range, $12{\sim}203$ months). Two patients developed local progression and six patients experienced local recurrence. The 5-year disease-free survival rate and the 5-year progression-free survival rate were 61 % and 70%, respectively. Wide local excision was associated with better disease free survival with statistical significance (p=0.028). Radiotherapy after initial surgery (p=0.046) and a higher radiation dose of more than 60 Gy (p=0.049) were associated with better progression free survival with statistical significance. At the time of the last follow-up, the number of additional surgeries was higher in patients that received radiotherapy after reoperation (p<0.001). Conclusion: Radiotherapy after the initial operation improved local control and decreased the number of subsequent operations. Thus, postoperative radiotherapy after an initial operation is recommended in patients with a high risk of recurrence for a desmoid tumor.

Assessment of malignity in medical claims review (보험의학적 악성도 판단)

  • Lee, Sin-Hyung
    • The Journal of the Korean life insurance medical association
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    • v.24
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    • pp.27-42
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    • 2005
  • Among medical claims review, decision of malignancy is very important. According to the pathologic report may be ordinary pathway. Some tumors are not completely studied especially malignancy. Wheather malignancy or benign is the important thing in medical claims review. We here disscuss on the debatable tumors such as carcinoid tumor, gastrointestinal stromal tumor (GIST), desmoid tumor, MALToma, and pseudomyxoma peritonei. Another controversial subject in the medical claims review is selection of pathologic report. If the result of the pathologic report is not same in one patient, We prefer the selsection of the report from more professional hospital. We have called this professional hospital l as "third hospital" or 'refferal hospital".

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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 Distal Femur: A Case Report (결합조직형성 섬유종: 증례 보고)

  • Song, Joon-Ho;Shim, Jae-Chan;Lee, Ghi-Jae;Kim, Jin-Goo;Kang, Yun-Kyung
    • Investigative Magnetic Resonance Imaging
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    • v.13 no.2
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    • pp.199-202
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    • 2009
  • Desmoplastic fibroma of bone is an extremely rare tumor that was first described by Jaffe in 1958. It histologically resembles the desmoid tumor of soft tissue. It is known as locally aggressive tumor but we experienced definitely benign and resembling simple bone cyst radiographically. We report a case of desmoplastic fibroma of bone and it should be included in the differential diagnosis list of any lytic bone lesion. The radiograph, MR imaging features, radiological and pathological differential diagnosis of the case are described, and literatures are reviewed.

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