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[侵袭性纤维瘤病(韧带样瘤)的临床表现与治疗]

[The clinical picture and therapy of aggressive fibromatosis (desmoids)].

作者信息

Hoffmann W, Weidmann B, Schmidberger H, Niederle N, Seeber S, Bamberg M

机构信息

Abteilung für Strahlentherapie, Eberhard-Karls-Universität Tübingen.

出版信息

Strahlenther Onkol. 1993 Apr;169(4):235-41.

PMID:8488459
Abstract

Desmoid tumors are accounted among histologically benign soft tissue tumors. Though metastases do not occur the clinical course of the disease with their tendency to grow locally aggressive and to relapse after curative intended surgery is similar to sarcomas. After surgical excision adjuvant therapy of desmoid tumors is necessary in such cases, where a radical excision with a wide margin is not possible, or must be doubted or after the surgical excision of a locally recurrent tumor. Radiation doses of 50 to 60 Gy reduce the risk of local recurrence of desmoid tumors to 18%. In such cases, where surgery and radiotherapy have been leading to incomplete responses, systemic therapy with hormones (tamoxifen or progesterone), with alkylating agents (e. g. ifosfamide) or with prostaglandin antagonists (indomethacin) is possible. The published data on therapeutic responses to the treatment of desmoid tumors are scarce. We are reporting on seven of our own cases, treated with surgery and radiotherapy (four) or with a combination of surgery, radiotherapy, hormones and chemotherapy (three).

摘要

韧带样瘤属于组织学上良性的软组织肿瘤。虽然不会发生转移,但该疾病的临床病程具有局部侵袭性生长的倾向,且在进行根治性手术切除后容易复发,这与肉瘤相似。在手术切除后,对于那些无法进行广泛切缘的根治性切除、或对此存在疑问的情况,以及在手术切除局部复发性肿瘤后,韧带样瘤都需要进行辅助治疗。50至60Gy的放射剂量可将韧带样瘤的局部复发风险降低至18%。在手术和放疗导致反应不完全的情况下,可以采用激素(他莫昔芬或孕酮)、烷化剂(如异环磷酰胺)或前列腺素拮抗剂(吲哚美辛)进行全身治疗。关于韧带样瘤治疗反应的已发表数据很少。我们报告了我们自己治疗的7例病例,其中4例采用手术和放疗,3例采用手术、放疗、激素和化疗联合治疗。

相似文献

1
[The clinical picture and therapy of aggressive fibromatosis (desmoids)].[侵袭性纤维瘤病(韧带样瘤)的临床表现与治疗]
Strahlenther Onkol. 1993 Apr;169(4):235-41.
2
Desmoid tumors: need for an individualized approach.硬纤维瘤:需要个体化治疗方法。
Expert Rev Anticancer Ther. 2009 Apr;9(4):525-35. doi: 10.1586/era.09.9.
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[Therapeutic options for aggressive fibromatosis].[侵袭性纤维瘤病的治疗选择]
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Cancer Radiother. 2004 Oct;8(5):279-87. doi: 10.1016/j.canrad.2004.07.004.
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[Extra-abdominal desmoid tumors. Therapeutic indications. Apropos of 28 cases].[腹外硬纤维瘤。治疗指征。附28例报告]
Acta Orthop Belg. 1991;57(3):227-33.
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[Desmoid tumors or intra-abdominal fibromatoses].
Ann Chir. 1993;47(4):352-9.
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Local recurrence of soft tissue sarcoma. A Scandinavian Sarcoma Group Project.软组织肉瘤的局部复发。一项斯堪的纳维亚肉瘤研究小组项目。
Acta Orthop Scand Suppl. 2001 Feb;72(300):1-31.
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Desmoid tumors and current status of management.硬纤维瘤及治疗现状
Orthop Clin North Am. 2006 Jan;37(1):53-63. doi: 10.1016/j.ocl.2005.08.004.
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Post-treatment complications of soft tissue tumours.软组织肿瘤的治疗后并发症
Eur J Radiol. 2009 Feb;69(2):209-21. doi: 10.1016/j.ejrad.2008.10.008. Epub 2008 Dec 19.

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Desmoid tumor of the facet joint: a case report.小关节韧带样瘤:一例报告
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Multifocal extra-abdominal desmoid tumor--diagnostic and therapeutic problems.
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