A case of primary spindle cell variant of embryonal rhabdomyosarcoma of the prostate.
A case of primary spindle cell variant of embryonal rhabdomyosarcoma of the prostate.
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发表时间：
2014-07
影响因子：
1.4
通讯作者：
Miki Asahina;Tsuyoshi Saito;A. Arakawa;Y. Suehara;T. Takagi;S. Hisasue;Kazuo Kaneko;S. Horie;T. Yao
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作者：
Miki Asahina;Tsuyoshi Saito;A. Arakawa;Y. Suehara;T. Takagi;S. Hisasue;Kazuo Kaneko;S. Horie;T. Yao
我们治疗了一例罕见的梭形细胞变异的胚胎性横纹肌肉瘤（RMS）的前列腺的病人转介到我们医院的肉眼血尿。计算机断层扫描和磁共振成像显示一个4厘米直径的肿块与局灶性囊性变化。经尿道前列腺电切术（TUR）是诊断和治疗完全性尿潴留。显微镜下，TUR标本几乎包括梭形肿瘤细胞的束状增殖，导致梭形细胞肉瘤的诊断。随后的全子宫切除术显示除了梭形细胞增殖外，还存在横纹肌母细胞。在该肿瘤中未检测到MyoD1 p.L122R突变。肿瘤局部复发，术后不久发现多处转移灶。患者接受化疗和放疗，但在首次出现后10个月死亡。虽然MyoD1突变被报道定义为胚胎性RMS的临床侵袭性子集，但胚胎性RMS的梭形细胞变体显示出极不良的临床结局，与MyoD1突变无关。
We treated a rare case of spindle cell variant of embryonal rhabdomyosarcoma (RMS) of the prostate of a patient referred to our hospital for gross hematuria. Computed tomography and magnetic resonance imaging revealed a 4-cm-diameter mass with focal cystic change. Transurethral resection (TUR) of the prostate was performed to diagnosis and treat for complete urinary retention. Microscopically, the TUR specimen almost comprised a fascicular proliferation of spindle-shaped tumor cells, leading to the diagnosis of spindle cell sarcoma. The consequent total prostatectomy revealed the presence of rhabdomyoblasts in addition to the spindle cell proliferation. A MyoD1 p.L122R mutation was not detected in this tumor. The tumor recurred locally, with multiple metastatic lesions found soon after surgery. The patient received chemotherapy and radiation therapy but died 10 months after initial presentation. Although MyoD1 mutation is reported to define a clinically aggressive subset of embryonal RMS, spindle cell variant of embryonal RMS shows extremely adverse clinical outcomes irrespective of MyoD1 mutation.