Department of Pathology, Wilford Hall Medical Center, Lackland Air Force Base, San Antonio, Texas, USA.
Int J Gynecol Pathol. 2010 Mar;29(2):122-34. doi: 10.1097/PGP.0b013e3181bc98c0.
We report the clinicopathologic features of 4 cases of pure pleomorphic rhabdomyosarcoma of the uterine corpus with an emphasis on their frequent expression of CD10 and CD56, review the relevant literature, and discuss differential diagnostic considerations. The patients ranged from 51 to 79 years (mean 68 y). All were FIGO stage IIIC to IV at initial surgical staging, and 3 were dead from the disease at an average of 8.6 months follow-up. In addition to the expected findings, other notable morphologic features included tumor giant cells (4/4), osteoclast-like giant cells (1/4), patchy myxoid stroma (4/4), and only infrequent cytoplasmic cross striations (1/4). The tumors in all 4 cases were positive for myogenin, myo-D1, smooth muscle actin, desmin, muscle-specific actin (HHF-35), and CD10; 3 (75%) of 4 cases were positive for calponin and CD56; all cases were negative for cytokeratin 7, synaptophysin, epithelial membrane antigen, placental-like alkaline phosphatase, chromogranin, and a pan-keratin. Twenty-three cases have been reported earlier in the English-language literature between 1969 and 2009. In combination with the current 4, the 27 patients had an age range of 35 to 87 years (mean 66.33 y). Only 1 patient was deemed inoperable; most had staging operations. Following their initial evaluations, 16 (59%) were found to have extrauterine extension of disease. At follow-up, 73% (19/27) were dead from the disease and 19.2% had no evidence of recurrence. Ten (53%) of the 19 deaths occurred within 6.5 months of initial evaluation. Stage at presentation did not have any significant impact on outcome: 73% of the 11 patients with uterus-confined disease at presentation were dead from the disease at follow-up, a rate of disease-associated death that was nearly identical to the 75% in the 16 patients with extrauterine disease at presentation. A wide variety of neoadjuvant and adjuvant therapies were administered, which did not appear to significantly impact outcomes. These data indicate that pleomorphic rhabdomyosarcoma of the uterine corpus is a highly aggressive, rapidly progressive tumor with a high case-fatality rate.
我们报告了 4 例单纯性子宫体多形性横纹肌肉瘤的临床病理特征，重点强调其经常表达 CD10 和 CD56，复习相关文献，并讨论鉴别诊断的考虑因素。患者年龄 51-79 岁（平均 68 岁）。所有患者在初始手术分期时均为 FIGO 分期 III C 至 IV 期，3 例在平均 8.6 个月的随访中死于该疾病。除了预期的发现外，其他值得注意的形态学特征包括肿瘤巨细胞（4/4）、破骨细胞样巨细胞（1/4）、斑片状黏液样基质（4/4）和仅偶尔出现细胞质交叉条纹（1/4）。所有 4 例肿瘤均表达肌球蛋白，myo-D1、平滑肌肌动蛋白、结蛋白、肌肉特异性肌动蛋白（HHF-35）和 CD10；4 例中有 3 例（75%）表达钙调蛋白和 CD56；所有病例均为阴性细胞角蛋白 7、突触素、上皮膜抗原、胎盘碱性磷酸酶、嗜铬粒蛋白和广谱角蛋白。1969 年至 2009 年间，英文文献中已有 23 例报道。结合目前的 4 例，27 例患者的年龄范围为 35-87 岁（平均 66.33 岁）。仅 1 例患者被认为无法手术；大多数患者均进行了分期手术。在初始评估后，16 例（59%）发现有子宫外疾病的扩展。随访时，73%（19/27）死于该病，19.2%无复发迹象。19 例死亡中有 10 例（53%）发生在初始评估后 6.5 个月内。发病时的分期对结局没有任何显著影响：11 例发病时局限于子宫的患者中有 73%在随访时死于该病，其疾病相关死亡率与 16 例发病时伴有子宫外疾病的患者的 75%几乎相同。广泛应用了新辅助和辅助治疗，但似乎并没有显著影响结局。这些数据表明，子宫体多形性横纹肌肉瘤是一种高度侵袭性、快速进展的肿瘤，病死率高。
