低悪性線維粘液性肉腫
WordNet
- unrefined in character; "low comedy"
- an air mass of lower pressure; often brings precipitation; "a low moved in over night bringing sleet and snow" (同)depression
- a low level or position or degree; "the stock market fell to a new low"
- used of sounds and voices; low in pitch or frequency (同)low-pitched
- very low in volume; "a low murmur"; "the low-toned murmur of the surf" (同)low-toned
- in a low position; near the ground; "the branches hung low"
- less than normal in degree or intensity or amount; "low prices"; "the reservoir is low"
- literal meanings; being at or having a relatively small elevation or upward extension; "low ceilings"; "low clouds"; "low hills"; "the sun is low"; "low furniture"; "a low bow"
- level to the right gradient
- a relative position or degree of value in a graded group; "lumber of the highest grade" (同)level, tier
- the height of the ground on which something stands; "the base of the tower was below grade" (同)ground level
- a variety of cattle produced by crossbreeding with a superior breed
- the gradient of a slope or road or other surface; "the road had a steep grade"
- a degree of ablaut (同)gradation
- assign a grade or rank to, according to ones evaluation; "grade tests"; "score the SAT essays"; "mark homework" (同)score, mark
- determine the grade of or assign a grade to
- a usually malignant tumor arising from connective tissue (bone or muscle etc.); one of the four major types of cancer
- arranged in a sequence of grades or ranks; "stratified areas of the distribution" (同)ranked, stratified
- British political cartoonist (born in New Zealand) who created the character Colonel Blimp (1891-1963) (同)David Low, Sir David Low, Sir David Alexander Cecil Low
PrepTutorEJDIC
- (高さ・位置が)『低い』,低い所にある / (価格・数量・程度などが)『低い』,少ない / (身分・地位などが)『低い』,卑しい / (人格・行為などが)『卑しい』,下劣な,下品な / 《補語にのみ用いて》(体が)『弱った』;元気のない,憂うつな / (供給が)不十分な;(…が)乏しい《+『in』(『on』)+『名』》 / (音・声が)低い,小さい / (生物が)下等な / 『低く』,低い所に / (価格・程度などが)『安く』,低く / 低い声(調子)で;静かに / 最低点,最低水準
- (牛の)モーという鳴き声 / (牛が)モーと鳴く
- 《米》(小・中・高校の)『学年』(小学校から12年まで通算する) / 《おもに米》(学業の)『成績』,評点 / (位階・品質・価値などの)『階級』,『等級』 / 《米》(道路・線路などの)傾斜,勾配(こうばい)(《英》gradient) / …‘を'『等級分けする』,‘の'格づけをする / 《米》…‘の'採点をする / 〈道路など〉‘の'勾配(こうばい)をゆるくする / 等級別になる,徐々に変わる,〈色彩などが〉次第に他の色に移る
- 肉腫(にくしゅ)
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2015/10/21 23:32:06」(JST)
[Wiki en表示]
Low-grade fibromyxoid sarcoma |
Micrograph of a low-grade fibromyxoid sarcoma. H&E stain.
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Classification and external resources |
Low-grade fibromyxoid sarcoma (LGFMS) is a rare type of low grade sarcoma first described by Harry Evans in 1987. It is characterized by a long and indolent clinical course and the possibility of local recurrence or distant metastases in a subset of patients. Unlike many other types of cancer, low grade fibromyxoid sarcoma can metastasize after many years, sometimes decades after the initial presentation of the tumor.[1]
References
- ^ Evans, Harry L (2011). "Low-Grade Fibromyxoid Sarcoma: A Clinicopathologic Study of 33 Cases With Long-Term Follow-Up". The American Journal of Surgical Pathology 35 (10): 1450–1462. doi:10.1097/PAS.0b013e31822b3687. PMID 21921785.
- Evans HL: Low-grade fibromyxoid sarcoma: a report of two metastasizing neoplasms having a deceptively benign appearance. Am J Clinical Pathology 1987, 88:615-619.
- Goodlad JR, Mentzel T, Fletcher CD: Low grade fibromyxoid sarcoma: clinicopathological analysis of eleven new cases in support of a distinct entity. Histopathology 1995, 26(3):229-37.
- Billings SD, Giblen G, Fanburg-Smith JC: Superficial low-grade fibromyxoid sarcoma (Evans tumor): a clinicopathologic analysis of 19 cases with a unique observation in the pediatric population. Am J Surg Pathol 2005, 29(2):204-10.
- Zámecník M, Michal M: Low-grade fibromyxoid sarcoma: a report of eight cases with histologic, immunohistochemical, and ultrastructural study. Ann Diagn Pathol 2000, 4(4):207-17.
- Evans HL: Low-grade fibromyxoid sarcoma. A report of 12 cases. Am J Surg Pathol 1993, 17(6):595-600.
- Saito R, Kumabe T, Watanabe M, Jokura H, Shibuya M, Nakazato Y, Tominaga T: Low-grade fibromyxoid sarcoma of intracranial origin. J Neurosurg 2008, 108(4):798-802.
- Tun K, Ozen O, Kaptanoglu E, et al.: Primary intracranial low-grade fibromyxoid sarcoma (Evans tumor). J Clin Neurosci 2008, 15(11):1298-301.
- Jakowski JD, Wakely PE Jr: Primary intrathoracic low-grade fibromyxoid sarcoma. Hum Pathol 2008, 39(4):623-8.
- Park IJ, Kim HC, Yu CS, Kim JS, Jang SJ, Kim JC: Low-grade fibromyxoid sarcoma of the colon. Dig Liver Dis 2007, 39(3):274-7.
- Reid R, de Silva MV, Paterson L, Ryan E, Fisher C: Low-grade fibromyxoid sarcoma and hyalinizing spindle cell tumor with giant rosettes share a common t(7;16)(q34;p11) translocation. Am J Surg Pathol 2003, 27(9):1229-36.
- Matsuyama A, Hisaoka M, Shimajiri S, Hayashi T, Imamura T, Ishida T, Fukunaga M, Fukuhara T, Minato H, Nakajima T, Yonezawa S, Kuroda M, Yamasaki F, Toyoshima S, Hashimoto H: Molecular detection of FUS- CREB3L2 fusion transcripts in low-grade fibromyxoid sarcoma using formalin-fixed, paraffin-embedded tissue specimens. Am J Surg Pathol 2006, 30(9):1077-84.
- Folpe AL, Lane KL, Paull G, Weiss SW: Low-grade fibromyxoid sarcoma and hyalinizing spindle cell tumor with giant rosettes: a clinicopathologic study of 73 cases supporting their identity and assessing the impact of high-grade areas. Am J Surg Pathol 2000, 24:1353-1360.
- Guillou L, Benhattar J, Gengler C, Gallagher G, Ranche?re-Vince D, Collin F, Terrier P, Terrier-Lacombe MJ, Leroux A, Marque?s B, Aubain Somerhausen Nde S, Keslair F, Pedeutour F, Coindre JM: Translocation-positive low- grade fibromyxoid sarcoma: clinicopathologic and molecular analysis of a series expanding the morphologic spectrum and suggesting potential relationship to sclerosing epithelioid fibrosarcoma: a study from the French Sarcoma Group. Am J Surg Pathol 2007, 31(9):1387-402.
- Mertens F, Fletcher CD, Antonescu CR, Coindre JM, Colecchia M, Domanski HA, Downs-Kelly E, Fisher C, Goldblum JR, Guillou L, Reid R, Rosai J, Sciot R, Mandahl N, Panagopoulos I: Clinicopathologic and molecular genetic characterization of low-grade fibromyxoid sarcoma, and cloning of a novel FUS/CREB3L1 fusion gene. Lab Invest 2005, 85(3):408-15.
- Panagopoulos I, Storlazzi CT, Fletcher CD, Fletcher JA, Nascimento A, Domanski HA, Wejde J, Brosjö, Rydholm A, Isaksson M, Mandahl N, Mertens F: The chimeric FUS/CREB3l2 gene is specific for low-grade fibromyxoid sarcoma. Genes Chromosomes Cancer 2004, 40(3):218-28.
- Storlazzi CT, Mertens F, Nascimento A, Isaksson M, Wejde J, Brosjo O, Mandahl N, Panagopoulos I: Fusion of the FUS and BBF2H7 genes in low grade fibromyxoid sarcoma. Hum Mol Genet 2003, 12(18):2349-58.
- Vernon SE, Bejarano PA: Low-grade fibromyxoid sarcoma: a brief review. Arch Pathol Lab Med 2006, 130(9):1358-60.
- Matsuyama A, Hisaoka M, Shimajiri S, Hashimoto H: DNA-based polymerase chain reaction for detecting FUS-CREB3L2 in low-grade fibromyxoid sarcoma using formalin-fixed, paraffin-embedded tissue specimens. Diagn Mol Pathol 2008, 17(4):237-40.
- Folpe A, van den Berg E, Molenaar WM: Low grade fibromyxoid sarcoma. In World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Edited by: Fletcher CDM, Unni KK, Mertens F. IARC Press: Lyon; 2002:104-5.
- Panagopoulos I, Möller E, Dahlén A, Dahlén A, Isaksson M, Mandahl N, Vlamis-Gardikas A, Mertens F: Characterization of the native CREB3L2 transcription factor and the FUS/CREB3L2 chimera. Genes Chromosomes Cancer 2007, 46(2):181-91.
UpToDate Contents
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English Journal
- Myxoid Variant of So-called Angiomatoid "Malignant Fibrous Histiocytoma": Clinicopathologic Characterization in a Series of 21 Cases.
- Schaefer IM, Fletcher CD.Author information Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA.AbstractAngiomatoid "malignant fibrous histiocytoma" (AMFH) is a tumor of intermediate malignancy and undefined lineage, mostly arising in the extremities of young patients. Examples with a prominent myxoid matrix are very uncommon. Twenty-one cases of myxoid AMFH (among a total of 414) identified in consult files are described, including clinicopathologic features, fluorescence in situ hybridization analysis in a subset of cases, and follow-up. Thirteen patients were female and 8 male, ranging in age from 2 to 51 y (median 17 y). These circumscribed tumors arose in subcutaneous or deep somatic soft tissue, with a median size of 2.5 cm (range, 1 to 8 cm), being located in the extremities (14/21), trunk (4/21), and upper limb girdle (3/21). Characteristic features included a fibrous pseudocapsule (20/21), peritumoral lymphoplasmacytic infiltrates (20/21), blood-filled cystic spaces (17/21), and prominent myxoid morphology comprising 60% to 100% of the tumor surface area examined. Histiocytoid or spindled tumor cells exhibited vesicular nuclei, inconspicuous nucleoli, palely eosinophilic cytoplasm, and multinodular growth without necrosis. Mucin pools and scattered multinucleate giant cells were observed in a subset of cases. Mild to moderate atypia was observed in 4 cases; 1 tumor showed a pseudochondroid matrix. Immunohistochemically, 14/21 cases expressed desmin, 12/21 expressed EMA, and 4/7 exhibited EWSR1 rearrangement. Follow-up, available for 11 patients (median, 43 mo), revealed that 3 developed local recurrence after 2, 7, and 48 months, respectively. All patients were alive without metastases. AMFH may present with prominent myxoid features making diagnosis difficult and causing possible confusion with other myxoid tumors including low-grade fibromyxoid sarcoma, extraskeletal myxoid chondrosarcoma, and myxoid liposarcoma.
- The American journal of surgical pathology.Am J Surg Pathol.2014 Feb 5. [Epub ahead of print]
- Angiomatoid "malignant fibrous histiocytoma" (AMFH) is a tumor of intermediate malignancy and undefined lineage, mostly arising in the extremities of young patients. Examples with a prominent myxoid matrix are very uncommon. Twenty-one cases of myxoid AMFH (among a total of 414) identified in consul
- PMID 24503754
- Low-grade fibromyxoid sarcoma with nuclear pleomorphism arising in the subcutis of a child.
- Sedrak MP, Parker DC, Gardner JM.Author information Department of Pathology, University of Texas Medical Branch, Galveston, TX, USA.AbstractLow-grade fibromyxoid sarcoma (LGFMS) represents a rare soft tissue tumor that was first characterized in 1987. LGFMS usually presents as a large, deeply situated mass in adults and is characterized by deceptively bland histopathologic features. LFGMS is less common in superficial soft tissue and in children. It is distinctly uncommon for LGFMS to exhibit nuclear pleomorphism. Herein, we present a case of a 10-year-old male who presented with a subcutaneous back mass that displayed features typical for LGFMS as well as scattered large, hyperchromatic and pleomorphic nuclei. The constellation of clinicopathologic features, including the young age of the patient, the small size and superficial location of the tumor and the presence of scattered nuclear pleomorphism are all unusual features for LGFMS. Fluorescent in situ hybridization (FISH) with a break-apart probe for FUS revealed the presence of a FUS gene rearrangement confirming the diagnosis of LGFMS. This case highlights the importance of maintaining a high index of suspicion for LGFMS even in the context of small, superficially-located tumors, pediatric patients or tumors with scattered nuclear pleomorphism.
- Journal of cutaneous pathology.J Cutan Pathol.2014 Feb;41(2):134-8. doi: 10.1111/cup.12245. Epub 2013 Nov 5.
- Low-grade fibromyxoid sarcoma (LGFMS) represents a rare soft tissue tumor that was first characterized in 1987. LGFMS usually presents as a large, deeply situated mass in adults and is characterized by deceptively bland histopathologic features. LFGMS is less common in superficial soft tissue and in
- PMID 24188507
- Fisher C, Thway K.Author information Royal Marsden Hospital, London, United Kingdom.AbstractSUMMARY: Aggressive (deep or desmoid-type) fibromatoses are locally infiltrative collagen-forming tumours with potential for recurrence but not metastasis. They exert their clinical effects primarily in relation to location and have variable biological behaviour. In sporadic cases there are somatic mutations in the β-catenin (CTNNB1) gene on 3p21, resulting in immunohistochemically demonstrable overexpression in nuclei. Fibromatosis in patients with familial adenomatous polyposis (FAP) harbours inactivating germline mutations in the desmoid region of the adenomatous polyposis coli (APC) gene on 5q21-q22. The differential diagnosis includes other myofibroblastic lesions, perineurioma, low grade fibromyxoid sarcoma and, in the abdomen, gastrointestinal stromal tumour and liposarcoma with 'low-grade' dedifferentiation. The primary management is surgical, though some desmoids cease to grow and can be watched. Other therapies have a role in stabilising growth or shrinking tumours. Although no single therapy is effective in all cases, available modalities including irradiation, hormonal therapy, chemotherapy, and receptor tyrosine kinase inhibition can be of value in appropriate clinicopathological subgroups.
- Pathology.Pathology.2014 Feb;46(2):135-40. doi: 10.1097/PAT.0000000000000045.
- SUMMARY: Aggressive (deep or desmoid-type) fibromatoses are locally infiltrative collagen-forming tumours with potential for recurrence but not metastasis. They exert their clinical effects primarily in relation to location and have variable biological behaviour. In sporadic cases there are somatic
- PMID 24378386
Japanese Journal
- 症例 背部に生じたLow-grade Fibromyxoid Sarcoma の1例
- 症例 Low-grade fibromyxoid sarcomaの1例
- An Intrathoracic Low-Grade Fibromyxoid Sarcoma Arising from the Chest Wall with Massive Pleural Effusion
- Annals of Thoracic and Cardiovascular Surgery 20(Supplement), 509-512, 2014
- NAID 130004889710
Related Links
- low-grade fibromyxoid sarcoma 低悪性線維粘液肉腫 (LGFMS), Evans tumor † 1987年Evans H.L.が組織学的には異型に乏しく一見良性にみえる線維性腫瘍の像を示しながら再発, 転移をきたした2症例を報告した。1993年にはさらに12症例を ...
- PATHOGENESIS CHARACTERIZATION CHROMOSOMAL ALTERATIONS-FUS-CREB3L2 or FUS-CREB3L1 Am J Dermatopathol. 2011 Apr;33(2):140-3. FUS (16p11) Gene Rearrangement as Detected by Fluorescence In-Situ ...
★リンクテーブル★
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- 英
- low-grade fibromyxoid sarcoma
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- 関
- deficient、inadequate、insufficiency、insufficient、insufficiently、poorly、scanty
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- degree、extent、grading、measure、phase、stage、step
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- quantitative、stepwise、stepwisely