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A Giant Aggressive Angiomyxoma of the Pelvis Misdiagnosed as Incarcerated Femoral Hernia: A Case Report and Review of the Literature

机译:误诊为股骨嵌顿疝的骨盆巨大侵袭性血管粘液瘤:一例报道并文献复习

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摘要

Aggressive angiomyxoma (AA) is an uncommon mesenchymal tumor that is mostly derived from the female pelvic and perineal regions. AA is a locally infiltrative slow growing tumor with a marked tendency to local recurrence. Painless swelling located around the genitofemoral region is the common symptom; thus, it is often misdiagnosed as a gynecological malignancy or a groin hernia. A 35-year-old female patient who previously underwent surgery for left femoral hernia operation resulting in surgical failure was reoperated for a giant AA located in the pelvis. The tumor was completely excised with free margins. Histopathologic examination revealed an AA. The tumor size was measured as 24 × 12 × 6 cm with a weight of 4.2 kg. Immunohistochemically, the cells show positive staining with vimentin, desmin, estrogen, and progesterone receptor. S100, MUC4, CD34, and SMA were negative in the tumor cells. AA should be considered in the differential diagnosis of any painless swelling located in the genitofemoral region, particularly in women of reproductive age. The principle treatment should be complete surgical excision with tumor-free margins. Long-term follow-up and careful monitoring are essential due to its high tendency of local recurrence in spite of wide excision of the tumor. Adjuvant antihormonal therapy yields promising results for preventing recurrence.
机译:侵袭性血管粘液瘤(AA)是一种罕见的间充质肿瘤,主要起源于女性骨盆和会阴区。 AA是一种局部浸润性生长缓慢的肿瘤,具有局部复发的明显趋势。生殖器股骨周围无痛肿是常见症状。因此,它经常被误诊为妇科恶性肿瘤或腹股沟疝。一名先前因左股疝气手术导致手术失败而接受手术的35岁女性患者因位于骨盆的巨大AA而再次手术。肿瘤被完全切除,具有自由边缘。组织病理学检查显示AA。肿瘤尺寸为24×12×6 cm,重量为4.2 weightkg。在免疫组织化学上,细胞显示波形蛋白,结蛋白,雌激素和孕激素受体呈阳性染色。肿瘤细胞中S100,MUC4,CD34和SMA阴性。在生殖器股骨区域,特别是育龄妇女中,无痛性肿胀的鉴别诊断中应考虑使用AA。主要治疗方法应是完全手术切除,无肿瘤边缘。尽管广泛切除肿瘤,但由于其高度局部复发的趋势,因此长期随访和仔细监测至关重要。辅助抗激素治疗可预防复发。

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