Sawada Morio, Tochigi Naobumi, Sasajima Yuko, Hasegawa Tadashi, Kasamatsu Takahiro, Kitawaki Jo
Department of Obstetrics and Gynecology, Kyoto Prefectural University of Medicine, Japan.
J Obstet Gynaecol Res. 2011 Nov;37(11):1706-10. doi: 10.1111/j.1447-0756.2011.01559.x. Epub 2011 Jun 9.
Primary extraskeletal myxoid chondrosarcoma (EMC) of the vulva is extremely rare. There is little available information about the biological behavior and treatment strategy for primary EMC of the vulva. We report a rare case of primary EMC of the vulva treated surgically. A 24-year-old Japanese woman had demonstrated a small and elastic mass of the vulva and underwent enucleation of the mass at a previous hospital, but a definitive histopathological diagnosis was not obtained. Therefore, the patient was referred to our hospital for further evaluation and treatment. We histopathologically diagnosed the tumor as primary EMC of the vulva and performed vulvectomy with vulvoperineal reconstruction. Microscopic examination of the resected specimens demonstrated residual tumor nodules of EMC. However, there were no viable tumor cells at the surgical margin. Approximately two years after wide local excision was performed, the patient is doing well and there is no apparent recurrence of EMC.
外阴原发性骨外黏液样软骨肉瘤（EMC）极为罕见。关于外阴原发性EMC的生物学行为和治疗策略，目前可获取的信息很少。我们报告一例经手术治疗的罕见外阴原发性EMC病例。一名24岁的日本女性外阴出现一个小的、质地有弹性的肿物，曾在之前的医院接受肿物摘除术，但未获得明确的组织病理学诊断。因此，该患者被转诊至我院进行进一步评估和治疗。我们通过组织病理学诊断该肿瘤为外阴原发性EMC，并进行了外阴切除术及外阴会阴重建术。对切除标本的显微镜检查显示有EMC残留肿瘤结节。然而，手术切缘未见存活的肿瘤细胞。在进行广泛局部切除术后约两年，患者情况良好，EMC无明显复发。
