Akrivi Stella, Varras Michail, Anastasiadi Zoi, Pappa Christina, Vlachioti Aikaterini, Varra Viktoria-Konstantina, Varra Fani-Niki, Balasi Eufemia, Akrivis Christos
Department of Obstetrics and Gynecology, Royal Jubille Maternity Hospital, Belfast Trust, Belfast BT12 6BA, UK.
Fourth Department of Obstetrics and Gynecology, 'Elena Venizelou' General Hospital, Athens 11521, Greece.
Mol Clin Oncol. 2021 Apr;14(4):69. doi: 10.3892/mco.2021.2231. Epub 2021 Feb 9.
Vulvar sarcomas located in the Bartholin's gland area are extremely uncommon mesenchymal vulvar tumors. These neoplasms can be mistaken as Bartholin' gland benign lesions such as cysts or abscesses, leading to a delay in the diagnosis of underlying malignancy. Currently, only a few cases of these aggressive cancers have been reported in the literature. A 42-year-old female patient without any previous complaint presented to Obstetrics and Gynecology Department of 'G. Chaztikosta' General Hospital due to a vulvar lump in the area of the left Bartholin's gland with a 6-month history of progressive swelling. Pelvic examination showed a solid mass of 6.5-cm in maximum diameter, localized in the left Bartholin's gland. The patient underwent wide local excision and histopathological examination of hematoxylin and eosin-stained sections indicated intersecting fascicles of spindle cells, with moderate to severe atypia. The number of mitoses was up to 8 per 10 high power fields. The neoplasm to its greatest extent was circumscribed and in places had an invasive growth pattern. Tumoral necrosis was not seen. Involved Bartholin' gland by the tumor was identified. The tumor extended focally to the surgical margin. The neoplastic cells showed positive staining for smooth muscle actin, desmin, HHF35, caldesmon, vimentin and estrogen and progesterone receptors. Immunohistochemistry was negative for S100, myoglobulin, keratin 116, CD117, CD34 and CD31. The patient denied further surgery or/and local radiotherapy, although the mass was >5-cm and a focally infiltrative surgical margin was found. During the close follow-up, no local recurrences or metastases were observed 53 months after surgery. In conclusion, wide local tumor excision with free surgical margins is a good option of surgery for vulvar leiomyosarcomas. In recurrences, a new extensive surgical resection of the lesion and radiotherapy are suggested. Ipsilateral lympadenectomy is indicated when there is a pathologic lymph node. Chemotherapy is provided in cases of distal metastases.
位于巴氏腺区域的外阴肉瘤是极其罕见的外阴间叶性肿瘤。这些肿瘤可能被误诊为巴氏腺良性病变，如囊肿或脓肿，从而导致潜在恶性肿瘤的诊断延迟。目前，文献中仅报道了少数几例这类侵袭性癌症。一名42岁既往无任何不适的女性患者因左侧巴氏腺区域出现外阴肿块，且有6个月进行性肿胀病史，就诊于“G. 查兹蒂科斯塔”综合医院妇产科。盆腔检查发现一个最大直径为6.5厘米的实性肿块，位于左侧巴氏腺。患者接受了广泛局部切除，苏木精和伊红染色切片的组织病理学检查显示梭形细胞束相互交叉，具有中度至重度异型性。每10个高倍视野中的有丝分裂数高达8个。肿瘤在最大范围内有包膜，部分区域呈浸润性生长模式。未见肿瘤坏死。肿瘤累及巴氏腺被识别。肿瘤局部延伸至手术切缘。肿瘤细胞平滑肌肌动蛋白、结蛋白、HHF35、钙调蛋白、波形蛋白以及雌激素和孕激素受体染色呈阳性。免疫组化S100、肌红蛋白、角蛋白116、CD117、CD34和CD31均为阴性。尽管肿块直径>5厘米且发现手术切缘有局灶性浸润，但患者拒绝进一步手术和/或局部放疗。在密切随访期间，术后53个月未观察到局部复发或转移。总之，具有游离手术切缘的广泛局部肿瘤切除是外阴平滑肌肉瘤的一个良好手术选择。对于复发患者，建议对病变进行新的广泛手术切除并放疗。当存在病理淋巴结时，建议行同侧淋巴结清扫术。对于远处转移患者，则给予化疗。
