Abstract:
Uterine tumors resembling ovarian sex cord tumors (UTROSCTs), first characterized by Drs Clement and Scully in 1976, are rare neoplasms showing clinical, morphologic, and immunohistochemical overlap with a number of other uterine tumors, most being mesenchymal. Criteria for aggressive behavior are not clearly established. We report 75 tumors from patients ranging from 21 to 84 (mean=52.4) years. Seventy-one patients were treated by hysterectomy and 4 by conservative total excision. Thirty-eight tumors were intramyometrial, 34 submucosal, and 3 cervical; they ranged from 0.6 to 20 (mean=4.9) cm and were typically tan-yellow. Sixty-eight neoplasms were well-circumscribed and 7 had infiltrative borders (4 only minimally). In 56 tumors, a smooth muscle component was intimately admixed with the neoplastic cells (\"pseudoinfiltration\"; extensive in 29). Architectural patterns included cords (n=53), diffuse (n=51), hollow tubules (n=48), nests (n=38), trabeculae (n=37), retiform (n=23), solid tubules (n=21), pseudoangiomatoid (n=11), pseudopapillary (n=4), and whorled (n=2); typically, more than 1 pattern was seen. Tumor cells were epithelioid (n=62), epithelioid and spindled (n=12), or spindled (n=1) and/or rhabdoid (n=20; extensive in 2). Cytologic atypia was absent to mild in 57, moderate in 16, and moderate to severe in 2 tumors. Fifty-seven UTROSCTs had ≤2mitoses/10 high power fields (HPF), 12 had 3 to 5/10 HPF, and 6 >5/10 HPF. Necrosis was present in 3 and lymphovascular invasion in 1. Tumor cells showed a polyphenotypic immunohistochemical profile (with positivity for sex cord, smooth muscle, and epithelial markers), most commonly inhibin (17/33+) and calretinin (22/31+) positive. Five of 58 patients with follow-up (22 to 192; mean=73.2 mo) had recurrences/metastases from 30 to 144 months, and 2 died of disease. Malignant tumors showed >3 of the following 5 features compared with benign tumors: size >5 cm, at least moderate cytologic atypia, ≥3 mitoses/10 HPF, infiltrative borders, and necrosis. One of the 5 malignant tumors showed an extensive rhabdoid morphology. UTROSCTs are uncommon, show a wide morphologic spectrum, often pose problems in differential diagnosis, and typically have a benign outcome. Rare tumors are associated with late recurrences and a combination of more than 3 of the 5 features listed above predicted aggressive behavior in this series.
摘要:
类似于卵巢性索肿瘤(UTROSCTs)的子宫肿瘤,1976年由Clement博士和Scully博士首次表征,是临床上罕见的肿瘤,形态学,免疫组织化学与许多其他子宫肿瘤重叠,大多数是间充质。攻击性行为的标准尚未明确。我们报告了来自21至84(平均=52.4)年的患者的75个肿瘤。71例患者接受子宫切除术治疗,4例接受保守全切除术治疗。38个肿瘤是肌层内,34粘膜下,和3个宫颈;它们的范围从0.6到20(平均=4.9)cm,通常为棕褐色。68个肿瘤界限清楚,7个肿瘤具有浸润性边界(仅最低限度的4个)。在56个肿瘤中,平滑肌成分与肿瘤细胞紧密混合(“假性浸润”;29例广泛)。建筑图案包括帘线(n=53),漫反射(n=51),空心小管(n=48),巢(n=38),小梁(n=37),退休(n=23),实心小管(n=21),假血管瘤样(n=11),假乳头(n=4),轮生(n=2);通常,看到了一个以上的模式。肿瘤细胞为上皮样(n=62),上皮样和纺锤状(n=12),或纺锤状(n=1)和/或横纹肌(n=20;2中广泛)。细胞学异型性在57例中为轻度,在16例中为中度,在2例中为中度至重度。57个UTROSCT具有≤2个mitoses/10个高功率场(HPF),12有3到5/10的HPF,和6>5/10HPF。3例出现坏死,1例出现淋巴管浸润。肿瘤细胞表现出多表型免疫组织化学谱(性索阳性,平滑肌,和上皮标记物),最常见的是抑制素(17/33+)和钙视网膜素(22/31+)阳性。58例随访患者中有5例(22至192;平均=73.2个月)复发/转移30至144个月,2人死于疾病。与良性肿瘤相比,恶性肿瘤表现出以下5个特征中的>3个:大小>5cm,至少中度细胞学异型,≥3个有丝分裂/10个HPF,渗透边界,和坏死。5种恶性肿瘤中的一种表现出广泛的横纹肌样形态。UTROSCT并不常见,显示出广泛的形态谱,经常在鉴别诊断中提出问题,通常会有良性的结果。罕见的肿瘤与晚期复发以及以上列出的5个特征中的3个以上的组合相关,预测了本系列中的攻击行为。