Nielsen G P, Rosenberg A E, Koerner F C, Young R H, Scully R E
Department of Pathology, Harvard Medical School, Boston, Massachusetts, USA.
Am J Surg Pathol. 1996 Jul;20(7):779-93. doi: 10.1097/00000478-199607000-00001.
The clinical and pathological features of 25 smooth-muscle tumors of the vulva were analyzed. The patients ranged in age from 17 to 67 (mean, 37.6) years; two were pregnant. Twenty-three tumors were 1.5 to 16 (mean, 5.2) cm in greatest dimension; the size of two tumors was unknown. Microscopic examination showed that 16 tumors were circumscribed, six had focally infiltrative margins, and the margins could not be evaluated in three tumors. Fourteen tumors were composed mainly of spindle cells; two of these tumors had prominent myxoid stroma. Seven tumors were predominantly epithelioid and had a prominent hyalinized or myxoid stroma; often the cells had a plexiform pattern. Four tumors contained an approximately equal number of epithelioid and spindle cells. Ten tumors had mild, nine moderate, and six severe cytologic atypia. Mitotic figures ranged from 0 to 10 (average, 1.8) per 10 high-power fields (hpf). Immunohistochemically, all the tumors stained for one or more muscle markers. Thirteen of 17 tumors were positive for estrogen receptors, and 16 of 18 were positive for progesterone receptors. Follow-up information ranging from 1 month to 19 years (average, 5 years) was available in 19 cases. Four tumors recurred locally, and one patient with recurrent tumor died of metastases 7 months after the initial operation. We propose an expanded criteria to distinguish between leiomyomas and leiomyosarcomas of the vulva. Tumors that manifest three or all of the four following features should be considered sarcomas: > or = 5 cm in greatest dimension, infiltrative margins, > or = 5 mitotic figures per 10 hpf, and moderate to severe cytologic atypia. Those that have only one of these characteristics should be diagnosed as leiomyoma, and those that exhibit only two of these features should be considered benign but atypical leiomyomas. The sarcomas should be excised with widely negative margins; the leiomyomas and the atypical leiomyomas should be excised conservatively, with long-term, careful follow-up.
对25例外阴平滑肌肿瘤的临床和病理特征进行了分析。患者年龄在17至67岁之间（平均37.6岁）；2例患者处于妊娠期。23个肿瘤的最大直径为1.5至16厘米（平均5.2厘米）；2个肿瘤的大小未知。显微镜检查显示，16个肿瘤边界清晰，6个肿瘤边缘有局灶性浸润，3个肿瘤的边缘情况无法评估。14个肿瘤主要由梭形细胞组成；其中2个肿瘤有明显的黏液样间质。7个肿瘤以上皮样细胞为主，有明显的玻璃样变或黏液样间质；细胞常呈丛状排列。4个肿瘤中上皮样细胞和梭形细胞数量大致相等。10个肿瘤有轻度细胞学异型性，9个有中度异型性，6个有重度异型性。每10个高倍视野（hpf）的核分裂象数为0至10个（平均1.8个）。免疫组织化学检查显示，所有肿瘤均对一种或多种肌肉标志物呈阳性染色。17个肿瘤中有13个雌激素受体阳性，18个中有16个孕激素受体阳性。19例患者有随访信息，随访时间从1个月至19年不等（平均5年）。4个肿瘤局部复发，1例复发患者在初次手术后7个月死于转移。我们提出了扩大的标准来区分外阴平滑肌瘤和平滑肌肉瘤。具有以下四个特征中的三个或全部的肿瘤应被视为肉瘤：最大直径≥5厘米、边缘浸润、每10个hpf≥5个核分裂象以及中度至重度细胞学异型性。仅具有这些特征之一的肿瘤应诊断为平滑肌瘤，仅表现出其中两个特征的肿瘤应被视为良性但非典型平滑肌瘤。肉瘤应行广泛阴性切缘切除；平滑肌瘤和非典型平滑肌瘤应行保守切除，并进行长期、仔细的随访。
