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Desmoid Tumors of the Pelvis and Abdominal Wall

机译:骨盆和腹壁的胶质瘤

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Background: Desmoid tumors are rare, benign, locally invasive neoplasms characterized by proliferation of fibroblasts without cytologic features of malignancy. Wide excision is the recommended primary therapy. Adjunctive radiation therapy may be helpful in patients with positive surgical margins or recurrence. Progestins and tamoxifen are noted to be beneficial in recurrent or inoperable desmoid tumors.Case Reports: A 20-year-old woman with desmoid tumors of the rectus muscles associated with pregnancy is treated by surgical excision alone. A 32-year-old woman with a paravaginal desmoid involving the levator ani muscle was successfully managed with a combination of surgical excision and adjunctive radiation therapy.Discussion: The optimal primary therapy for desmoid tumors is wide, radical, local excision. Other treatments have included radiation therapy, tamoxifen, medroxyprogesterone acetate, corticosteroids, antibiotics, phenylbutazone, indomethacin, ascor-bate, and cytotoxic chemotherapy agents. Desmoid tumors have been associated with Familial Adenoma-tous Polyposis and Gardner's Syndrome, suggesting a genetic predisposition to the disease. The occurrence of these tumors has also been associated with pregnancy and soft tissue trauma. Conclusion: Although historically the primary management of aggressive fibromatosis has been by surgical resection, the diffuse nature of this tumor with its overall benign-appearing histologic features often compromises the surgeon's ability to determine and achieve microscopically free, adequately wide surgical margins of resection. We review the role of adjuvant treatments.
机译:背景:类胶质瘤是罕见的,良性的,局部浸润性肿瘤,其特征是成纤维细胞增殖而无恶性细胞学特征。推荐广泛切除。辅助放射治疗对手术切缘或复发阳性的患者可能会有帮助。孕激素和他莫昔芬在复发性或不能手术的类胶质瘤中被认为是有益的。病例报告:一名20岁的患有与妊娠相关的直肌肌肉类胶质瘤的妇女仅通过手术切除即可治疗。结合手术切除和辅助放疗成功治疗了一名32岁的患有肛提肌,累及肛提肌的妇女,讨论:消融法对鼻窦肿瘤的最佳初步治疗是广泛,根治性,局部切除。其他治疗方法包括放射疗法,他莫昔芬,醋酸甲羟孕酮,皮质类固醇,抗生素,苯丁a,消炎痛,抗坏血酸和细胞毒性化学治疗剂。类胶质瘤与家族性腺瘤样息肉病和加德纳氏综合症有关,提示该病具有遗传易感性。这些肿瘤的发生也与怀孕和软组织创伤有关。结论:尽管从历史上看,侵袭性纤维瘤病的主要治疗方法是通过外科手术切除,但这种肿瘤的弥散性及其整体良性的组织学特征通常会损害外科医生确定并实现无镜下,足够宽的手术切除范围的能力。我们回顾了辅助治疗的作用。

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