前列腺肉瘤19例报告并文献复习

Report of 19 cases of prostate sarcoma and literature review

目的探讨前列腺肉瘤的临床特点和诊治方法。方法1995年10月至2010年9月我院19例前列腺肉瘤患者，年龄16—48岁，平均36岁。均因排尿困难入院，其中6例伴膀胱刺激征，3例伴肉眼血尿，3例伴尿中排出血块，3例伴排便困难，2例伴尿路感染，1例伴腰痛。直肠指检16例前列腺体积明显增大，其中6例向直肠内突出明显；余3例触及质硬结节且有触痛。B超检查均提示有前列腺肿物，内部组织回声不均匀。12例盆腔MRI检查提示前列腺不规则软组织密度影，边缘不清，前列腺内部呈高低混杂信号，肿物向外突出。其中7例前列腺明显增大与膀胱分界不清，6例与直肠分界不清，1例提示为囊实性改变。10例盆腔CT检查提示前列腺不规则肿物，其中5例肿物侵犯双侧精囊，2例肿物侵犯单侧精囊，3例伴淋巴结肿大。2例胸部X线片提示肺内有广泛转移。结果6例行全膀胱前列腺切除术，8例行前列腺根治性切除术，1例行双输尿管皮肤造瘘和直肠造瘘术，1例行膀胱造瘘术，1例行前列腺穿刺活检后化疗，2例仅行前列腺穿刺活检。病理诊断：平滑肌肉瘤6例，横纹肌肉瘤6例，恶性间叶瘤2例，叶状囊肉瘤1例，粒细胞肉瘤1例，分叶状肉瘤1例，恶性纤维组织肉瘤1例，低分化肌源性肉瘤1例。免疫组化检查19例波形蛋白均阳性表达，平滑肌肌动蛋白和肌红蛋白分别在平滑肌肉瘤和横纹肌肉瘤中阳性表达，而结蛋白在上述两种肉瘤中有不同程度的表达。CD117和S-100仅分别在1例含未分化成分和1例含脂肪肉瘤的恶性间叶瘤中弱阳性表达，粒细胞肉瘤中抗过氧化物酶阳性表达，分叶状肉瘤中p63阳性表达，恶性纤维组织细胞瘤中CD34阳性表达。14例术后给予化疗，其中6例联合放疗。19例均获随访，随访时间4—30个月，平均13个月。4例患者未复发，术后存活24～30个月，余15例均在确诊后18个月内死亡。结论前列腺肉瘤是临床罕见的恶性肿瘤，恶性程度较高，临床表现缺乏特异性，确诊依靠病理活检和免疫组化，早期诊断和根治性手术切除对提高生存率有一定的积极意义。

Objective To investigate the clinical features, diagnosis and treatment of prostate sarcoma. Methods 19 cases of prostate sarcoma had been treated in our hospital from Oct 1995 to Sep 2010, aged from 16 to 48 years （mean, 36 years）. They were hospitalized due to different degrees of difficulty in urination, 6 cases with bladder irritation, 3 cases with gross hematuria, 3 cases with blood clots in urination, 3 cases with difficult defecation, 2 cases with urinary tract infection, and 1 case with low back pain. Digital rectal examination showed prostate volume increased significantly in 16 cases, of which 6 cases prominent into bladder, and 3 cases touched hard nodules and tenderness. B ultrasound examination showed prostate neoplasms with internal echo uneven. Pelvic MRI in 12 cases indicated irregular soft tissue density in prostate with unclear edge, a high-low mixed signal inside, and mass protruding outward, among which 7 cases with significantly enlarged prostates and unclear bladder boundaries, 6 cases with unclear boundaries with rectum, and 1 case with solid and cystic change. Pelvic CT in 10 cases showed irregular prostate tumors, among which 5 cases with tumor violation of bilateral seminal vesicle, 2 cases with tumor invasion of unilateral seminal vesicle, and 3 cases with lymph node enlargement. Chest X-ray in 2 cases showed widespread metastatic lung. Results Different treatments were used according to different conditions： 6 eases with full cystoprostateetomy, 8 eases with radical prostatectomy, 1 case with skin routine ureter fistulization and rectal fistulization, 1 case with bladder fistulization, 1 case with chemotherapy after prostate biopsy, and 2 cases with prostate biopsy alone. Pathological diagnosis showed 6 cases of leiomyosarcoma, 6 eases of rhabdomyosarcoma, 2 eases of malignant mesenehymal tumor, 1 ease of phyllodes sarcoma, 1 ease of granuloeytic sarcoma, 1 ease of phyllodes sarcoma, 1 case of malignant fibrous tissue sarcoma, and 1 case of poorly differentiated muscle induced sarcoma. Immunohistoehemical examination in 19 eases of paraffin specimens stained with SP showed vimentin was positive, smooth muscle actin and myoglobin in leiomyosar- coma and rhabdomyosarcoma were positive respectively, and desmin in two kinds of afore-mentioned sarcoma expressed in different level. CD117 and S-100 were weakly positive in 1 patient with undifferentiated component and 1 patient with liposarcoma of the malignant mesenehymoma. Peroxidase was positive in granuloeytic sarcoma, p63 was positive in lobulated sarcoma, and CD34 was positive in malignant fibrous histioeytoma. 14 patients were given chemotherapy, among which 6 cases were combined with radiotherapy. 19 cases were fol- lowed up for 4 -30 months （mean 13 months）. 4 patients survived for 24 -30 months without recurrence, while 15 cases died within 18 months after diagnosis. Conclusions The prostate sarcoma is a clinically rare malignant tumor with high degree of malignancy and unspecific clinical manifestations. The diagnosis relies on biopsy and immunohistochemistry, and early diagnosis and radical surgical excision are helpful for increasing survival rate.