Li Rong-Gang, Huang Jun
Department of Pathology, Jiangmen Central Hospital, Affiliated Jiangmen Hospital of Sun Yat-Sen University, Jiangmen 529030, Guangdong Province, China.
Department of Urology, Jiangmen Central Hospital, Affiliated Jiangmen Hospital of Sun Yat-Sen University, Jiangmen 529030, Guangdong Province, China.
World J Clin Cases. 2020 Feb 6;8(3):606-613. doi: 10.12998/wjcc.v8.i3.606.
Prostatic stromal sarcoma presenting with rhabdoid features is extremely rare, and only four cases have been reported in the English-language literature to date. Accordingly, there is no absolute definition of this group of tumors as yet, and our overall understanding of its morphological features, therapeutic regimen and prognosis is limited.
A 34-year-old male patient was referred to our hospital to address a 2-mo history of hematuria and progressive dysuria. Pelvic computed tomography scan revealed a 6.0 cm × 5.2 cm × 7.2 cm mass in the prostate, with bladder invasion. The patient underwent transurethral prostatectomy as upfront therapy. He refused further treatment and died of uncontrollable tumor growth 3 mo after surgery. Pathology analysis revealed the stroma to be pleomorphic, with a huge number of atypical spindle cells. Rhabdomyoblastic cells, with abundant eosinophilic cytoplasm, were detected. The spindle cells were positive for vimentin, INI1 and β-catenin, and the rhabdomyoblastic cells were positive for MyoD1, myogenin and INI1. The spindle cells and epithelial cells were sporadically positive for P53.
The prostatic stromal sarcoma tumor was immunoreactive for β-catenin, suggesting a role for the Wnt/β-catenin pathway in this tumor type.
具有横纹肌样特征的前列腺间质肉瘤极为罕见，迄今为止英文文献中仅报道了4例。因此，目前尚无对这类肿瘤的确切定义，我们对其形态学特征、治疗方案及预后的整体认识有限。
一名34岁男性患者因2个月的血尿及进行性排尿困难被转诊至我院。盆腔计算机断层扫描显示前列腺有一个6.0 cm×5.2 cm×7.2 cm的肿块，侵犯膀胱。患者接受了经尿道前列腺切除术作为初始治疗。他拒绝进一步治疗，术后3个月死于肿瘤生长无法控制。病理分析显示间质多形性，有大量非典型梭形细胞。检测到具有丰富嗜酸性细胞质的横纹肌母细胞。梭形细胞波形蛋白、INI1和β-连环蛋白呈阳性，横纹肌母细胞MyoD1、肌细胞生成素和INI1呈阳性。梭形细胞和上皮细胞P53偶尔呈阳性。
前列腺间质肉瘤肿瘤β-连环蛋白免疫反应阳性，提示Wnt/β-连环蛋白通路在这类肿瘤中起作用。
