Extraskeletal Myxoid Chondrosarcoma of the Vulva: A Case Report.
Extraskeletal Myxoid Chondrosarcoma of the Vulva: A Case Report.
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DOI：
10.7759/cureus.33601
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发表时间：
2023-01
期刊：
影响因子：
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通讯作者：
Shibahara H
中科院分区：
文献类型：
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作者：
Omote M;Tsubamoto H;Ide Y;Kawai K;Futani H;Shibahara H
外阴骨外粘液样软骨肉瘤（EMC）极其罕见。我们报告我们的经验与情况下的疾病控制放射治疗的局部病变的EMC。一位41岁的妇女来到我们的诊所与外阴肿块。磁共振成像显示会阴和大腿内侧肌肉之间有一个15厘米的肿块。是“大收肌。“在针吸活检后，进行了EMC的组织病理学诊断。组织基因组分析检测到EWSR 1-NR 4A 3融合基因。骨科、妇科和整形外科进行了联合手术，包括会阴广泛切除、大腿内侧肌部分切除和腹直肌瓣膜成形术。术中检测到耻骨浸润。术后给予盆腔放疗作为辅助治疗。观察到照射野外复发性髂总淋巴结转移和多发性肺转移。帕唑帕尼作为辅助治疗给药。肺部转移得到控制，但盆腔肿瘤已经扩散，因此患者接受了放射治疗。阿霉素二线化疗后，出现左侧胸腔积液和纵隔淋巴结转移，给予甲磺酸艾日布林三线化疗。胸腔积液改善，但患者再次出现咳嗽，给予曲贝替丁作为第四次化疗。本病例放疗后三年未见局部复发，提示放疗局部控制有效。
Extraskeletal myxoid chondrosarcoma (EMC) of the vulva is extremely rare. We report our experience with a case of disease control by radiation therapy to a localized lesion of EMC. A 41-year-old woman presented to our clinic with a vulvar mass. Magnetic resonance imaging showed a 15 cm mass between the perineum and the medial thigh muscle. It was the "adductor magnus muscle." After the needle biopsy, a histopathological diagnosis of EMC was made. Tissue genomic analysis detected the EWSR1-NR4A3 fusion gene. A joint operation by the Department of Orthopedics, Gynecology, and Plastic Surgery was performed, which included a wide excision of the perineum, partial excision of the medial thigh muscle, and rectus abdominis valvuloplasty. Intraoperatively, pubic infiltration was detected. Postoperative pelvic radiotherapy was administered as adjuvant therapy. Recurrent common iliac lymph node metastases outside the irradiation field and multiple lung metastases were observed. Pazopanib was administered as adjuvant therapy. Pulmonary metastases were controlled, but the pelvic tumor had spread, so the patient underwent radiation therapy. After second-line chemotherapy with doxorubicin, left pleural effusion and mediastinal lymph node metastasis appeared, and third-line chemotherapy with eribulin mesylate was administered. The pleural effusion improved, but the patient developed cough again, and trabectedin was administered as the fourth chemotherapy. In this case, there was no local recurrence for three years after radiotherapy, suggesting the effectiveness of radiotherapy in local control.
影响因子：
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作者：
Liou SS;Memarzadeh S;Dry SM;Graham RP;Moatamed NA
通讯作者：
Moatamed NA