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。
