Silva Elvio G, Deavers Michael T, Bodurka Diane C, Malpica Anais
Department of Pathology, Box 85, University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
Am J Surg Pathol. 2004 Feb;28(2):244-9. doi: 10.1097/00000478-200402000-00013.
In this study, we investigated HMB-45 expression in epithelioid uterine leiomyosarcomas with clear cell areas. From 12 epithelioid leiomyosarcomas, we selected 5 that had: 1) clear cell areas and 2) spindle cell areas that were at least focally positive for desmin and caldesmon. The patients' ages ranged from 47 to 82 years (mean 64 years). Presenting symptoms were uterine bleeding (three), abdominal pain (one), and a pelvic mass (one). There was no history of tuberous sclerosis or lymphangioleiomyomatosis. One patient had stage II disease, one stage III, and three stage IV. All were treated with total abdominal hysterectomy and bilateral salpingo-oophorectomy. Two received radiotherapy, and three were also treated with chemotherapy. The tumors ranged in size from 4 x 3 x 3 cm to 10 x 7 x 6 cm; all had significant cellular atypia, areas of coagulative necrosis, and between 10 and 90 mitoses per 10 high power fields. Vascular invasion was seen in three cases. The epithelioid component varied from 50% to 90% in each case; and the percentage of clear cells was < 1% in one case, 5% in one case, and 10% to 80% in three cases. Smooth muscle actin and desmin were positive in all cases. Four cases were positive for HMB-45 only in the clear cell areas. The tumor with < 1% of clear cells was negative for HMB-45. All were negative for S-100 and c-kit. Three patients died of disease at 9, 30, and 32 months; one patient is alive with progressive disease at 6 months, and one patient (stage II disease) is alive with no evidence of disease at 8 months. Unequivocal uterine epithelioid leiomyosarcomas may have clear cells positive for HMB-45. These tumors might belong to the group of lesion designated as PEComas; however, it is advisable to designate them as uterine leiomyosarcomas. In uterine smooth muscle tumors, some epithelioid cells most likely undergo clear cell changes and become positive for HMB-45. It would be advisable to perform this stain in all epithelioid smooth muscle tumors of the uterus.
在本研究中，我们调查了具有透明细胞区域的上皮样子宫平滑肌肉瘤中HMB-45的表达情况。从12例上皮样平滑肌肉瘤中，我们选取了5例具有以下特征的肿瘤：1）透明细胞区域；2）梭形细胞区域，其至少局灶性地对结蛋白和钙调蛋白呈阳性。患者年龄在47至82岁之间（平均64岁）。主要症状为子宫出血（3例）、腹痛（1例）和盆腔肿块（1例）。无结节性硬化症或淋巴管平滑肌瘤病病史。1例患者为II期疾病，1例为III期，3例为IV期。所有患者均接受了全腹子宫切除术和双侧输卵管卵巢切除术。2例接受了放疗，3例还接受了化疗。肿瘤大小从4×3×3cm至10×7×6cm不等；所有肿瘤均有明显的细胞异型性、凝固性坏死区域，每10个高倍视野有10至90个核分裂象。3例可见血管侵犯。每例中上皮样成分占50%至90%；透明细胞百分比在1例中<1%，1例中为5%，3例中为10%至80%。所有病例中平滑肌肌动蛋白和结蛋白均呈阳性。4例仅在透明细胞区域HMB-45呈阳性。透明细胞<1%的肿瘤HMB-45呈阴性。所有病例S-100和c-kit均为阴性。3例患者分别在9个月、30个月和32个月死于疾病；1例患者在6个月时带进展性疾病存活，1例（II期疾病）患者在8个月时无疾病证据存活。明确的子宫上皮样平滑肌肉瘤可能有HMB-45阳性的透明细胞。这些肿瘤可能属于指定为PEComa的病变组；然而，将它们指定为子宫平滑肌肉瘤是可取的。在子宫平滑肌肿瘤中，一些上皮样细胞很可能发生透明细胞改变并对HMB-45呈阳性。对所有子宫上皮样平滑肌肉瘤进行该染色是可取的。
