Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore, MD, USA.
Am J Surg Pathol. 2010 Apr;34(4):502-9. doi: 10.1097/PAS.0b013e3181cf326d.
Smooth muscle neoplasms of the urinary bladder are relatively rare. We report the largest series to date examining the clinicopathologic features of leiomyomas and leiomyosarcomas of the bladder. This study sought to clarify several issues relating to smooth muscle neoplasms of the urinary bladder: (1) How to distinguish leiomyomas of the bladder from normal muscularis propria on transurethral resection (TUR) specimens; (2) Whether symplastic leiomyomas can be diagnosed in the bladder; (3) Can leiomyoma be definitively diagnosed on biopsy or TUR without the risk of there being unsampled leiomyosarcoma; and (4) Is the grade of leiomyosarcoma seen on biopsy or TUR heterogeneous and hence possibly not representative of the true grade. Thirty-one leiomyomas and 20 leiomyosarcoma cases of urinary bladder from 3 tertiary care medical centers were examined. Leiomyosarcoma cases were subdivided into low-grade and high-grade based on mitotic count (> or =5/10 HPF) and nuclear atypia. The mean age of the patients with leiomyoma and leiomyosarcoma was 52 and 58, respectively. The M:F ratio was significantly higher in patients with leiomyosarcoma (2:1) compared with leiomyoma (1:3), p<0.005. The specimen consisted of 20 TUR and 11 transurethral biopsies (TUBx) for leiomyomas, and 10 TUR, 3 TUBx, 5 cystectomies (Cyst), and 2 partial cystectomies (pCyst) for leiomyosarcomas. LEIOMYOMAS: Notable features in leiomyomas were hyalinization (7/31), degenerative atypia (7/31), necrosis (4/31), myxoid changes (2/31), and focal fatty metaplasia (1/31); although no surface ulceration was identified. Clinical follow-up was available for 24 patients (12 to 108 mo; mean 36 mo); 4 lost to follow-up and 3 recent cases. Two patients had repeat TUR within a year of the initial diagnosis with the same bland leiomyoma on histology, probably reflecting persistence of earlier unresected tumor as opposed to recurrent tumor. None of the patients were diagnosed with leiomyosarcoma on follow-up, including 7 cases with degenerative atypia. LEIOMYOSARCOMA: Of the 20 leiomyosarcomas, 8 were classified as low-grade and 12 as high-grade sarcomas. Histologic features included epithelioid morphology (5/20; 1 entirely epithelioid), tumor cell necrosis (11/20), and mucosal ulceration (7/20). Infiltration into the muscularis propria was seen predominantly as a nodular growth pattern with some cases exhibiting an irregular infiltrative pattern (6/10 with evaluable borders); an infiltrative pattern was not restricted to high-grade lesions. Lesional heterogeneity was present in only 1 case on the same specimen, which showed both low-grade and high-grade areas. Another case was low grade on TUR, yet high grade at cystectomy. None of the cases of leiomyosarcomas had areas histologically resembling leiomyoma. Clinical follow-up was available for 15 patients with leiomyosarcoma (11 to 144 mo; mean 47 mo); 3 lost to follow-up and 2 recent cases. Only 1 patient with low-grade sarcoma experienced 2 local recurrences treated only by TUR and is currently free of disease. Disease-related mortality was significantly higher in patients with high-grade compared with low-grade leiomyosarcomas (50% vs. none, respectively; P<0.001). Leiomyoma (including symplastic leiomyoma) may be diagnosed on TUR without risk of underdiagnosing unsampled leiomyosarcoma. High-grade leiomyosarcomas are highly aggressive neoplasms compared with low-grade leiomyosarcomas; in most cases grade can be accurately determined on TUR.
膀胱平滑肌肉瘤较为罕见。我们报告了迄今为止最大的系列研究,检查了膀胱平滑肌瘤和平滑肌肉瘤的临床病理特征。本研究旨在澄清与膀胱平滑肌肿瘤相关的几个问题:(1)如何在经尿道切除(TUR)标本上区分膀胱平滑肌瘤与正常的肌层;(2)膀胱是否可以诊断出合胞性平滑肌瘤;(3)在活检或 TUR 上是否可以明确诊断平滑肌瘤而不会有未取样平滑肌肉瘤的风险;以及(4)活检或 TUR 上的平滑肌肉瘤分级是否存在异质性,因此可能无法代表真实的分级。检查了来自 3 家三级医疗中心的 31 例平滑肌瘤和 20 例平滑肌肉瘤病例。根据有丝分裂计数(>或=5/10 HPF)和核异型性,将平滑肌肉瘤病例分为低级别和高级别。平滑肌瘤和平滑肌肉瘤患者的平均年龄分别为 52 岁和 58 岁。平滑肌肉瘤患者的男女比例(2:1)明显高于平滑肌瘤患者(1:3),p<0.005。平滑肌瘤的标本由 20 例 TUR 和 11 例经尿道活检(TUBx)组成,而平滑肌肉瘤的标本由 10 例 TUR、3 例 TUBx、5 例膀胱切除术(Cyst)和 2 例部分膀胱切除术(pCyst)组成。平滑肌瘤:平滑肌瘤的显著特征包括玻璃样变(31 例中的 7 例)、退行性异型性(31 例中的 7 例)、坏死(31 例中的 4 例)、黏液样变(31 例中的 2 例)和局灶性脂肪化生(31 例中的 1 例);尽管没有发现表面溃疡。24 例患者(12 至 108 个月;平均 36 个月)有临床随访资料;4 例失访,2 例为近期病例。2 例患者在初始诊断后的一年内再次接受 TUR,组织学上仍为相同的良性平滑肌瘤,可能反映出未切除的早期肿瘤的持续存在,而不是复发性肿瘤。在随访中没有患者被诊断为平滑肌肉瘤,包括 7 例具有退行性异型性的病例。平滑肌肉瘤:20 例平滑肌肉瘤中,8 例为低级别,12 例为高级别肉瘤。组织学特征包括上皮样形态(20 例中的 5 例;1 例完全为上皮样)、肿瘤细胞坏死(20 例中的 11 例)和黏膜溃疡(20 例中的 7 例)。主要表现为结节状生长模式的肌层浸润,部分病例表现为不规则浸润性模式(10 例中有 6 例边界可评估);浸润模式不限于高级别病变。仅在同一样本上存在 1 例病变异质性,显示出低级别和高级别区域。另 1 例 TUR 为低级别,而膀胱切除术为高级别。平滑肌肉瘤的任何病例均无组织学上类似于平滑肌瘤的区域。15 例平滑肌肉瘤患者有临床随访资料(11 至 144 个月;平均 47 个月);3 例失访,2 例为近期病例。仅有 1 例低级别肉瘤患者经历了 2 次局部复发,仅接受 TUR 治疗,目前无疾病。高级别与低级别平滑肌肉瘤患者的疾病相关死亡率差异有统计学意义(分别为 50%和 0%;P<0.001)。在 TUR 上可以诊断平滑肌瘤(包括合胞性平滑肌瘤),而不会有未取样平滑肌肉瘤的风险。与低级别平滑肌肉瘤相比,高级别平滑肌肉瘤是一种侵袭性更高的肿瘤;在大多数情况下,在 TUR 上可以准确确定分级。